Urticaria Pigmentosa All

7/27/2019 Urticaria Pigmentosa All

1/24

Urticaria Pigmentosa

I. Definition:

Urticaria pigmentosa is an uncommon rash that usually affects the neck, arms,

legs and trunk of children and young adults. The rash consists of reddish-brown

spots that turn into hives when they are rubbed hard or scratched. Sometimes

the spots will blister.

II. Causes:

The spots in urticaria pigmentosa contain a large number of mast cells

(cells are the bodies building blocks). Mast cells are immune cells (cells

that fight infection) that live in the skin. Mast cells make a substance

called histamine. Histamine causes hives, itching, and flushing.

We do not known why people with urticaria pigmentosa have abnormal

collections of mast cells in the skin.

III. Prognosis:

Most children who develop urticaria pigmentosa before the age of five

will have resolution of the condition by adolescence or early adulthood.

When urticaria pigmentosa starts after the age of five, the abnormal

collections of mast cells will sometimes involve the internal organs and

the disease does not always go away. These patients need blood tests and

other studies.

IV.Treatment:

There is no satisfactory treatment for urticaria pigmentosa.

Patients with urticaria pigmentosa should avoid aspirin, codeine, opiates,

procaine, alcohol. polymyxin B, hot baths, and vigorous rubbing after

bathing and showering. These can release histamine which can cause

itching, flushing and hives.


2/24

Antihistamines such as Benadryl can help control itching, hives, and

flushing.

Match ALL words

Match ANY word

Your DermAtlas query returned at least 10 Images

Click on the IMAGE to see an enlargement.

The number of images has been limited to 10 per page.

Next Result Set

2001-05,

DermAtlas

ImageName:

urticaria_pigmentosa_1_051204 File Type:jpg

Diagnosis:URTICARIA PIGMENTOSA /URTICARIA PIGMENTOSA,DIFFUSE INFILTRATIVE

Category:cutaneous sign ofsystemic disease /hyperpigmentation

Body Site:total body / chestabdomen

Age: 5 years

Contributor: Bernard Cohen, MD

Description:diffuse confluent hyperpigmented plaques with some overlyingurticarial plaques

Comments:

This generally healthy 5-year-old boy with diffuse urticariapigmentosa complained of increasing abdominal pain. He had nodiarrhea, no headaches, but intermittent pruritus. Endocsopywas scheduled to evaluate the gastrointestinal tract for mast cellinvolvement.

RelatedImages:

All related Images urticaria_pigmentosa_4_051204urticaria_pigmentosa_3_051204urticaria_pigmentosa_2_051204
http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?OutputSet=2&BO=AND&Diagnosis=27http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=40http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=10http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=11http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1233756015http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=-2094088060&Rel2=1233756015&Rel3=-733675546&Rel4=1807451692&TotalRelImg=4http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-2094088060http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1233756015http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-733675546http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1807451692http://dermatlas.med.jhmi.edu/derm/index.cfmhttp://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?OutputSet=2&BO=AND&Diagnosis=27http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=40http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=10http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=11http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=-2094088060&Rel2=1233756015&Rel3=-733675546&Rel4=1807451692&TotalRelImg=4http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-2094088060http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1233756015http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-733675546


3/24

urticaria_pigmentosa_1_051204

2001-05,DermAtlas

ImageName:


Diagnosis:URTICARIA PIGMENTOSA /URTICARIA PIGMENTOSA,

DIFFUSE INFILTRATIVE

Category:cutaneous sign ofsystemic disease /

hyperpigmentation

Body Site:total body / chestabdomen / face

Age: 5 years



Comments:

This generally healthy 5-year-old boy with diffuse urticariapigmentosa complained of increasing abdominal pain. He had nodiarrhea, no headaches, but intermittent pruritus. Endocsopywas scheduled to evaluate the gastrointestinal tract for mast cell

involvement.RelatedImages:


2001-05,DermAtlas

ImageName:




Body Site: total body / back Age: 5 years



Comments:

This generally healthy 5-year-old boy with diffuse urticariapigmentosa complained of increasing abdominal pain. He had nodiarrhea, no headaches, but intermittent pruritus. Endocsopywas scheduled to evaluate the gastrointestinal tract for mast cellinvolvement.

RelatedImages:


2001-05,DermAtlas

ImageName:




Body Site: total body / back Age: 5 years


Description: diffuse confluent hyperpigmented plaques with some overlying
http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1807451692http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=40http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=10http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=11http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=2http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1233756015http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=-2094088060&Rel2=1233756015&Rel3=1807451692&TotalRelImg=3http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-2094088060http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1233756015http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1807451692http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=40http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=12http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-733675546http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=-2094088060&Rel2=-733675546&Rel3=1807451692&TotalRelImg=3http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-2094088060http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-733675546http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1807451692http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=40http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=12http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-2094088060http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1233756015http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-733675546http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1807451692http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=40http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=10http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=11http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=2http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=-2094088060&Rel2=1233756015&Rel3=1807451692&TotalRelImg=3http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-2094088060http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1233756015http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1807451692http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=40http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=12http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=-2094088060&Rel2=-733675546&Rel3=1807451692&TotalRelImg=3http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-2094088060http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-733675546http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1807451692http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=427957693http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=40http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=12http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1


4/24

urticarial plaques

Comments:

This generally healthy 5-year-old boy with diffuse urticariapigmentosa complained of increasing abdominal pain. He had nodiarrhea, no headaches, but intermittent pruritus. Endocsopywas scheduled to evaluate the gastrointestinal tract for mast cell

involvement.

RelatedImages:


2001-05,DermAtlas

ImageName:


Diagnosis:URTICARIA PIGMENTOSA /HYPERPIGMENTATION

Category:

lumps & bumps(plaques, nodules,tumors) /hyperpigmentation

Body Site: foot Age: 32 years


Description:multiple acral symmetric 2-3 mm golden brown macules andslightly elevated papules

Comments:

This healthy 32-year-old woman had a 3 year history of brownmacules and papules disseminated on her arms and legs withsparing of her trunk and face. A skin biopsy revealed anincreased number of mast cells around dermal blood vessels.

RelatedImages:

All related Images urticaria_pigmentosa_3_050523urticaria_pigmentosa_2_050523

2001-05,

DermAtlas

Image

Name: urticaria_pigmentosa_2_050523 File Type:jpg

Diagnosis:URTICARIA PIGMENTOSA /HYPERPIGMENTATION

Category:

lumps & bumps(plaques, nodules,tumors) /hyperpigmentation



Description:multiple acral symmetric 2-3 mm golden brown macules andslightly elevated papules

Comments:

This healthy 32-year-old woman had a 3 year history of brown

macules and papules disseminated on her arms and legs withsparing of her trunk and face. A skin biopsy revealed anincreased number of mast cells around dermal blood vessels.

RelatedImages:


ImageName:


Diagnosis: URTICARIA PIGMENTOSA / Category: lumps & bumps
http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-733675546http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=1233756015&Rel2=-733675546&Rel3=1807451692&TotalRelImg=3http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1233756015http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-733675546http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1807451692http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=472646841http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=16http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=661665390&Rel2=-320538303&TotalRelImg=2http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=661665390&Rel2=-320538303&TotalRelImg=2http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=661665390http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-320538303http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=472646841http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=16http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=661665390&Rel2=2137361986&TotalRelImg=2http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=661665390&Rel2=2137361986&TotalRelImg=2http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=661665390http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=2137361986http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=661665390http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-320538303http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=2137361986http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=1233756015&Rel2=-733675546&Rel3=1807451692&TotalRelImg=3http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1233756015http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-733675546http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1807451692http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=472646841http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=16http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=661665390&Rel2=-320538303&TotalRelImg=2http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=661665390http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-320538303http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=472646841http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=16http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=661665390&Rel2=2137361986&TotalRelImg=2http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=661665390http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=2137361986http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4


5/24

2001-05,DermAtlas

HYPERPIGMENTATION(plaques, nodules,tumors) /hyperpigmentation



Description: multiple acral symmetric 2-3 mm golden brown macules andslightly elevated papules

Comments:

This healthy 32-year-old woman had a 3 year history of brownmacules and papules disseminated on her arms and legs withsparing of her trunk and face. A skin biopsy revealed anincreased number of mast cells around dermal blood vessels.

RelatedImages:


2001-05,DermAtlas

ImageName:


Diagnosis: URTICARIA PIGMENTOSA Category:

lumps & bumps(plaques, nodules,tumors) /hyperpigmentation/cutaneous sign ofsystemic disease

Body Site: chest Age: 16 months

Contributor: Paul Honig, MD

Description: bronze 1-2 cm round to oval leathery plaques with fuzzy borders

Comments: This 16-month-old boy had a brown papule on his left thigh at

birth. In the early months of his life, the patient developed newbrown and tan papules on his chest, back, and occipital scalp.

Although his growth and development were normal, some of thepapules occasionally blistered after rubbing or irritation and hehad a few brief episodes of flushing. Urticaria pigmentosa is onemanifestation of cutaneous mastocytosis. This condition ischaracterized by persistent pruritic pigmented skin lesions thaturiticate with mechanical or chemical irritation. This finding isknown as Dariers sign. The condition is associated withmutations in the c-KIT protooncogene, which codes for the stemcell growth factor receptor (also known as the mast cell growth

factor receptor). Urticaria pigmentosa can occur from birth tomiddle age with most cases occurring within the first 6 months oflife. Cutaneous lesions consist of macules, papules, nodules,plaques, vesicle or bullae. Pruritus may be severe, and occursas a result of the massive release of histamine from mast celldegranulation. Triggers for degranulation include alcohol,opiates, aspirin, quinine, scopolamine, gallamine, reserpine,amphotericin B, polymyxin, and tubocarine. On histology, a
http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=472646841http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=16http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=-320538303&Rel2=2137361986&TotalRelImg=2http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=-320538303&Rel2=2137361986&TotalRelImg=2http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-320538303http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=2137361986http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=10http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=2051274298http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-9075779http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=472646841http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=16http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1http://dermatlas.med.jhmi.edu/derm/result_by_link_all.cfm?Rel1=-320538303&Rel2=2137361986&TotalRelImg=2http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-320538303http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=2137361986http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=10http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=2051274298


6/24

dense dermal collection of mast cells is diagnostic ofmastocytosis. Severe systemic disease may involve the lymphnodes, GI tract, bones, heart, blood, liver and spleen. Theprognosis for isolated cutaneous involvement, such as inurticaria pigmentosa, is generally good. Most cases clear

spontaenously or improve significantly over time. Symptomaticrelief can be obtained with systemic anti-histamines. PUVA isfrequently efficacious. Diarrhea as a result of urticariapigmentosa can be controlled with oral cromolyn sodium.

Avoidance of physical and chemical percipitants is of paramountimportance for control of this condition. References: 1. Azana JMet al. Urticaria pigmentosa. Pediatr Dermatol. 1994;11:102. 2.Godt, O, et al. Short- and long-term effectiveness of oral andbath PUVA therapy in urticaria pigmentosa and systemicmastocytosis. Dermatology 1997, 195: 35. 3. Henz BM et al.SCF and c-kit in mastocytosis. J Invest Derm. 1998;110:186. 4.

Topar G et al. Urticaria pigmentosa. Am J Clin Pathology1998;109:279. 5. Longley J et al. The mast cell and mast celldisease. J Am Acad Dermatol 1995;32:545. 6. Tharp MD. Mastcell disease and its diagnosis. J Invest Derm. 1995;104:885. 7.Tharp, MD. Understanding mast cells and mastocytosis. J InvestDerm 1997, 108:698.

RelatedImages:


2001-05,DermAtlas

ImageName:


Diagnosis:URTICARIA PIGMENTOSA /DARIER SIGN

Category:

lumps & bumps

(plaques, nodules,tumors) /hyperpigmentation/cutaneous sign ofsystemic disease

Body Site: trunk / back Age: 16 months

Contributor: Paul Honig, MD

Description:bronze 1-2 cm round to oval leathery plaques with fuzzy bordersand one plaque with surrounding wheal

Comments: This 16-month-old boy had a brown papule on his left thigh atbirth. In the early months of his life, the patient developed newbrown and tan papules on his chest, back, and occipital scalp.

Although his growth and development were normal, some of thepapules occasionally blistered after rubbing or irritation and hehad a few brief episodes of flushing. Urticaria pigmentosa is onemanifestation of cutaneous mastocytosis. This condition ischaracterized by persistent pruritic pigmented skin lesions that
http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-960925960http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-960925960http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=991706671http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=35http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=12http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=2051274298http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-960925960http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-960925960http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=991706671http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=5http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=1184808500http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=35http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=12http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=2051274298


7/24

uriticate with mechanical or chemical irritation. This finding isknown as Dariers sign. The condition is associated withmutations in the c-KIT protooncogene, which codes for the stemcell growth factor receptor (also known as the mast cell growthfactor receptor). Urticaria pigmentosa can occur from birth to

middle age with most cases occurring within the first 6 months oflife. Cutaneous lesions consist of macules, papules, nodules,plaques, vesicle or bullae. Pruritus may be severe, and occursas a result of the massive release of histamine from mast celldegranulation. Triggers for degranulation include alcohol,opiates, aspirin, quinine, scopolamine, gallamine, reserpine,amphotericin B, polymyxin, and tubocarine. On histology, adense dermal collection of mast cells is diagnostic ofmastocytosis. Severe systemic disease may involve the lymphnodes, GI tract, bones, heart, blood, liver and spleen. Theprognosis for isolated cutaneous involvement, such as in

urticaria pigmentosa, is generally good. Most cases clearspontaenously or improve significantly over time. Symptomaticrelief can be obtained with systemic anti-histamines. PUVA isfrequently efficacious. Diarrhea as a result of urticariapigmentosa can be controlled with oral cromolyn sodium.

Avoidance of physical and chemical percipitants is of paramountimportance for control of this condition. References: 1. Azana JMet al. Urticaria pigmentosa. Pediatr Dermatol. 1994;11:102. 2.Godt, O, et al. Short- and long-term effectiveness of oral andbath PUVA therapy in urticaria pigmentosa and systemicmastocytosis. Dermatology 1997, 195: 35. 3. Henz BM et al.

SCF and c-kit in mastocytosis. J Invest Derm. 1998;110:186. 4.Topar G et al. Urticaria pigmentosa. Am J Clin Pathology1998;109:279. 5. Longley J et al. The mast cell and mast celldisease. J Am Acad Dermatol 1995;32:545. 6. Tharp MD. Mastcell disease and its diagnosis. J Invest Derm. 1995;104:885. 7.Tharp, MD. Understanding mast cells and mastocytosis. J InvestDerm 1997, 108:698.

RelatedImages:


2001-05,DermAtlas

ImageName:


Diagnosis:URTICARIA PIGMENTOSA /MASTOCYTOSIS

Category:lumps & bumps(plaques, nodules,tumors)

Body Site:total body / foreheadshoulder

Age: 4 months

Contributor: Mark Simonian, MD

Description: multiple golden brown leathery papules
http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-9075779http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-9075779http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=168http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=40http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=37http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=51http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1504787488http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=1085704419http://dermatlas.med.jhmi.edu/derm/IndexDisplay.cfm?ImageID=-9075779http://dermatlas.med.jhmi.edu/derm/http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27http://dermatlas.med.jhmi.edu/derm/result.cfm?Diagnosis=168http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?Category=4http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=40http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=37http://dermatlas.med.jhmi.edu/derm/result.cfm?BodySite=51http://dermatlas.med.jhmi.edu/derm/result.cfm?Author=1504787488


8/24

Comments:

This healthy 4-month-old boy developed asymptomatic scatteredgolden brown leathery papules on the scalp, shoulder, ankle,and leg. The presence of a Darier sign (hive formation withrubbing) was diagnostic

Next Result Set

Match ALL words

Match ANY word

DermAtlas, Johns Hopkins University; 2000-2005Bernard A. Cohen, MD, Christoph U. Lehmann, MD

Return to the DermAtlas Home Page

Link directly to this page: http://DermAtlas.med.jhmi.edu/derm/result.cfm?Diagnosis=27

Urticaria pigmentosa

Definition:

A disease that produces skin lesions, intense itching, and hive formation at the site of thelesion upon rubbing.

Alternative Names:Systemic mastocytosis; Mastocytosis; Mastocytoma

Causes, incidence, and risk factors:Urticaria pigmentosa is caused by excessive numbers of inflammatory cells (mast cells) inthe skin. It is most often seen in children, but occurs in adults as well.

Rubbing of a lesion produces a rapid wheal (a hive-like bump). Younger children maydevelop a fluid-filled blisterover a lesion if it is traumatized.

A large histamine release may causeflushing,headache,diarrhea, a rapid heartbeat(tachycardia), and even fainting. This is uncommon with urticaria pigmentosa, but may beseen in other forms of mastocytosis.

Urticaria pigmentosa is one of several forms of mastocytosis. Other forms include thesolitary mastocytoma (a single lesion), and systemic mastocytosis (involvement in organsother than the skin).

Overview Symptoms Treatment Prevention
http://dermatlas.med.jhmi.edu/derm/result.cfm?OutputSet=2&BO=AND&Diagnosis=27mailto:[email protected],[email protected]?Subject=DermAtlas:%20NO%20clinical%20questions,%20please&body=DermAtlas:%20NO%20clinical%20questions,%20pleasemailto:[email protected],[email protected]?Subject=DermAtlas:%20NO%20clinical%20questions,%20please&body=DermAtlas:%20NO%20clinical%20questions,%20pleasehttp://dermatlas.med.jhmi.edu/derm/http://www.umm.edu/ency/article/003220.htmhttp://www.umm.edu/ency/article/003217.htmhttp://www.umm.edu/ency/article/003939.htmhttp://www.umm.edu/ency/article/003241.htmhttp://www.umm.edu/ency/article/003241.htmhttp://www.umm.edu/ency/article/003024.htmhttp://www.umm.edu/ency/article/003024.htmhttp://www.umm.edu/ency/article/003024.htmhttp://www.umm.edu/ency/article/003126.htmhttp://www.umm.edu/ency/article/003081.htmhttp://www.umm.edu/ency/article/003077.htmhttp://www.umm.edu/ency/article/003092.htmhttp://www.umm.edu/ency/article/003092.htmhttp://www.umm.edu/ency/article/001466.htmhttp://www.umm.edu/ency/article/001466sym.htmhttp://www.umm.edu/ency/article/001466trt.htmhttp://www.umm.edu/ency/article/001466prv.htmhttp://dermatlas.med.jhmi.edu/derm/result.cfm?OutputSet=2&BO=AND&Diagnosis=27mailto:[email protected],[email protected]?Subject=DermAtlas:%20NO%20clinical%20questions,%20please&body=DermAtlas:%20NO%20clinical%20questions,%20pleasemailto:[email protected],[email protected]?Subject=DermAtlas:%20NO%20clinical%20questions,%20please&body=DermAtlas:%20NO%20clinical%20questions,%20pleasehttp://dermatlas.med.jhmi.edu/derm/http://www.umm.edu/ency/article/001466.htmhttp://www.umm.edu/ency/article/001466sym.htmhttp://www.umm.edu/ency/article/001466trt.htmhttp://www.umm.edu/ency/article/001466prv.htmhttp://www.umm.edu/ency/article/003220.htmhttp://www.umm.edu/ency/article/003217.htmhttp://www.umm.edu/ency/article/003939.htmhttp://www.umm.edu/ency/article/003241.htmhttp://www.umm.edu/ency/article/003024.htmhttp://www.umm.edu/ency/article/003126.htmhttp://www.umm.edu/ency/article/003081.htmhttp://www.umm.edu/ency/article/003077.htmhttp://www.umm.edu/ency/article/003092.htm


9/24

Dermatographism -close-up

Dermatographism onthe arm

Urticaria pigmentosa inthe armpit

Mastocytosis, diffusecutaneous

Urticaria pigmentosaon the chest

Urticaria pigmentosa -close-up

Dermatographism onthe back

Review Date: 8/7/2001

Reviewed By: Michael Lehrer, M.D., Department of Dermatology, University of Pennsylvania Medical Center, Philadelphia, PA.Review provided by VeriMed Healthcare Network.

Authoritative facts about the skin from the New Zealand Dermatological Society Incorporated.

HomeFor patientsFor doctorsFind a dermatologistAboutStoreContactSite map

Search:go!

Home|Colour


Urticaria pigmentosa is the name given to a type of mastocytosis, in which there are brownpatches on the skin due to abnormal collections of mast cells.
http://www.umm.edu/imagepages/2017.htmhttp://www.umm.edu/imagepages/2017.htmhttp://www.umm.edu/imagepages/2018.htmhttp://www.umm.edu/imagepages/2018.htmhttp://www.umm.edu/imagepages/2037.htmhttp://www.umm.edu/imagepages/2037.htmhttp://www.umm.edu/imagepages/2438.htmhttp://www.umm.edu/imagepages/2438.htmhttp://www.umm.edu/imagepages/2484.htmhttp://www.umm.edu/imagepages/2484.htmhttp://www.umm.edu/imagepages/2485.htmhttp://www.umm.edu/imagepages/2485.htmhttp://www.umm.edu/imagepages/2780.htmhttp://www.umm.edu/imagepages/2780.htmhttp://dermnetnz.org/disclaimer.htmlhttp://dermnetnz.org/nzds.htmlhttp://dermnetnz.org/patient-information.htmlhttp://dermnetnz.org/patient-information.htmlhttp://dermnetnz.org/doctors/http://dermnetnz.org/doctors/http://dermnetnz.org/directory/http://dermnetnz.org/about.htmlhttp://dermnetnz.org/about.htmlhttp://dermnetnz.org/store.htmlhttp://dermnetnz.org/contact.htmlhttp://dermnetnz.org/contact.htmlhttp://dermnetnz.org/sitemap.htmlhttp://dermnetnz.org/colour/http://dermnetnz.org/colour/http://dermnetnz.org/colour/http://www.umm.edu/imagepages/2780.htmhttp://www.umm.edu/imagepages/2485.htmhttp://www.umm.edu/imagepages/2484.htmhttp://www.umm.edu/imagepages/2438.htmhttp://www.umm.edu/imagepages/2037.htmhttp://www.umm.edu/imagepages/2018.htmhttp://www.umm.edu/imagepages/2017.htmhttp://www.umm.edu/imagepages/2017.htmhttp://www.umm.edu/imagepages/2017.htmhttp://www.umm.edu/imagepages/2018.htmhttp://www.umm.edu/imagepages/2018.htmhttp://www.umm.edu/imagepages/2037.htmhttp://www.umm.edu/imagepages/2037.htmhttp://www.umm.edu/imagepages/2438.htmhttp://www.umm.edu/imagepages/2438.htmhttp://www.umm.edu/imagepages/2484.htmhttp://www.umm.edu/imagepages/2484.htmhttp://www.umm.edu/imagepages/2485.htmhttp://www.umm.edu/imagepages/2485.htmhttp://www.umm.edu/imagepages/2780.htmhttp://www.umm.edu/imagepages/2780.htmhttp://dermnetnz.org/disclaimer.htmlhttp://dermnetnz.org/nzds.htmlhttp://dermnetnz.org/patient-information.htmlhttp://dermnetnz.org/doctors/http://dermnetnz.org/directory/http://dermnetnz.org/about.htmlhttp://dermnetnz.org/store.htmlhttp://dermnetnz.org/contact.htmlhttp://dermnetnz.org/sitemap.htmlhttp://dermnetnz.org/colour/


10/24

Mast cells are normally widely distributed over the skin. They contain granules that containhistamine and other chemicals. When the mast cell is disturbed, these chemicals are releasedinto the surrounding skin. The chemicals make the blood vessels leaky, resulting in localiseditching, swelling and redness.

Pigmented spots are seen on the backof a man with extensive urticariapigmentosis of recent onset

Clinical features

Urticaria pigmentosa most often affects infants, with the first patches appearing at a fewmonths of age. They are often confused with insect bites at first, but persist and graduallyincrease in number for several months or years. They can appear on any part of the bodyincluding the scalp, face, trunk and limbs.

In urticaria pigmentosa, one can demonstrate the presence of mast cells by rubbing one of the

brown patches. Within a few minutes, the rubbed area becomes reddened, swollen and itchy.This is known as Dariers sign, and confirms the presence of mastocytosis.

Urticaria pigmentosa in an infantmay resemble persistent insect bites

In young children, it is common for the patches to blister when rubbed. If many patches areactivated at the same time the infant may become irritable but is uncommon for severesymptoms to arise.


11/24

Over the next few years the urticaria pigmentosa becomes less irritable and eventually thepatches fade away. By the teenage years, most patches will have gone.

Sometimes urticaria pigmentosa develops for the first time in an adult. Few or many lesionsappear and can be unsightly as well as itchy. Unfortunately, in adults urticaria pigmentosatends to persist long term. It is also more likely to be associated with internal symptoms.

Mast cells can accumulate in other organs as well as the skin (systemic mastocytosis). If thisoccurs, it may cause fever, weight loss, diarrhoea and abdominal pain. Rarely, systemicmastocytosis is due to malignancy and results in a form of leukaemia. Affected sites may bebone, liver, spleen, lymph nodes or the gastrointestinal tract.

Telangiectasia eruptiva macularis perstans (TEMP) is a rare form of mastocytosis in whichdiffuse red patches occur associated with overlying telangiectasia (dilated capillaries).

Precautions

Exercise or heat can aggravate symptoms. A severe reaction can result in flushing andfaintness.

Certain medications can cause mast cell degranulation and should be avoided if there is

extensive urticaria pigmentosa. These include:

Aspirin (salicylates)

Codeine and morphine (narcotics)

Alcohol

Anticholinergics

Tests

The appearance of urticaria pigmentosa is generally so characteristic that no specific tests arenecessary. However, occasionally a skin biopsy is needed to confirm the diagnosis. If there areany symptoms suggesting internal involvement, the following tests may be helpful:

X-rays of skull and long bones may show areas of bone thinning, or a diffuse ground

glass appearance

Liver function blood tests but the results may be normal even with extensive liver

involvement

Urinary histamine levels or 1,4-methylimidazole acetic acid may be elevated

Treatment

Urticaria pigmentosa is not serious, and does not require any treatment in most cases.However the following can be helpful.

Oral antihistamines

Mast cell stabilisers

Disodium cromoglycate orally may be helpful in some cases
http://dermnetnz.org/treatments/antihistamines.htmlhttp://dermnetnz.org/treatments/antihistamines.html


12/24

Low-dose aspirin

Paradoxically, this can help some patients, but must be taken cautiously as aspirin canalso worsen symptoms

Topical steroids

Potent steroid creams applied for several months under occlusion can reduce itchingand unsightliness, but the patches tend to recur within a few months. Topical steroids

are only suitable for limited areas. Photochemotherapy.(PUVA)

This form of ultraviolet radiation is the most effective treatment for adults withurticaria pigmentosa. Two or three treatments each week are required for severalmonths. PUVA lessens the itch and improves the appearance. The urticaria pigmentosais likely to recur within six to twelve months but the treatment can be repeated.

Interferon An expensive treatment appropriate only for the most severely affected

individuals

Related information

Other web sites:

Mastocytosis fact sheet - from National Institute of Allergy & Infectious Diseases

Mastocytosis - from emedicine dermatology, the online textbook

DermNet does not provide an on-line consultation service.If you have any concerns with your skin or its treatment, see a dermatologist for advice.

Created 1997. Last updated 29 May 2005. 2005 NZDS. Disclaimer.

Illustrations

Dermatographis

m - close-up

Dermatographis

m on the arm

Urticaria

pigmentosa in thearmpit

Mastocytosis

, diffusecutaneous

Urticaria

pigmentosa on thechest

Urticaria

pigmentosa - close-up

Dermatographism on the back
http://dermnetnz.org/treatments/topical-steroids.htmlhttp://dermnetnz.org/procedures/puva.htmlhttp://www.niaid.nih.gov/factsheets/masto.htmhttp://emedicine.com/derm/topic258.htmhttp://dermnetnz.org/directory/http://dermnetnz.org/nzds.htmlhttp://dermnetnz.org/disclaimer.htmlhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2017.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2017.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2018.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2018.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2037.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2037.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2037.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2037.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2438.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2438.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2438.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2484.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2484.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2484.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2484.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2485.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2485.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2485.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2485.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2780.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2780.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2780.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2485.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2484.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2438.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2037.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2018.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2017.htmhttp://dermnetnz.org/treatments/topical-steroids.htmlhttp://dermnetnz.org/procedures/puva.htmlhttp://www.niaid.nih.gov/factsheets/masto.htmhttp://emedicine.com/derm/topic258.htmhttp://dermnetnz.org/directory/http://dermnetnz.org/nzds.htmlhttp://dermnetnz.org/disclaimer.htmlhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2017.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2017.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2018.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2018.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2037.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2037.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2037.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2438.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2438.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2438.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2484.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2484.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2484.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2485.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2485.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2485.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2780.htmhttp://www.nlm.nih.gov/medlineplus/ency/imagepages/2780.htm


13/24

Alternative names Return to top

Mastocytosis; Mastocytoma

Definition Return to top

Urticaria pigmentosa is a disease that produces skin lesionsand intenseitching. If the lesions are rubbed,

hives may form on the site.

Causes, incidence, and risk factors Return to top

Urticaria pigmentosa is one of several forms of mastocytosis, which is caused by excessive numbers of

inflammatory cells (mast cells) in the skin. Other forms include solitary mastocytoma (a single lesion) and

systemic mastocytosis (involvement in organs other than the skin).

Urticaria pigmentosa is most often seen in children, but it can occur in adults as well.

Rubbing of a lesion produces a rapid wheal (a hive-like bump). Younger children may develop a fluid-

filledblisterover a lesion if it is scratched.

A large histamine release from these extra mast cells may cause flushing, headache, diarrhea, a rapid

heartbeat (tachycardia), and evenfainting. This is uncommon with urticaria pigmentosa but may be seen in

other forms of mastocytosis.

Symptoms Return to top

Appearance of brownish lesions on skin

Welt or hive formation when lesions are rubbed or scratched

Blister formation over lesion when it is rubbed

Facial flushing

Severe form: Diarrhea

Fast heart rate

Headache

Fainting (syncope)

Signs and tests Return to top

Urine histamine -- shows elevated levels of histamine

Skin biopsy -- confirms the presence of increased number of mast cells

Treatment Return to top

Antihistamines may relieve some of the histamine-induced symptoms such as itching and flushing. Discuss

the choice of antihistamine with your child's health care provider. With more severe and unusual forms ofurticaria pigmentosa, systemic symptoms should be controlled with other medications.

Expectations (prognosis) Return to top
http://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/003220.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003220.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003217.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003217.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003217.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/003939.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003939.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003939.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003077.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003092.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003092.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003092.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/003241.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/003840.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/003220.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003217.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/003939.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003077.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003092.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/003241.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/003840.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/#top


14/24

Urticaria pigmentosa goes away by puberty in about half of the affected children. Symptoms usually

decrease in the remaining patients as they grow into adulthood.

In adults, urticaria pigmentosa may progress to the more serious systemic mastocytosis.

Complications Return to top

Discomfort from itching, and possible self-consciousness about the "spots," are the primary complications.

Other problems such as diarrhea and fainting are rare.

Certain medications may trigger flares of urticaria pigmentosa. Discuss these with your doctor.

Bee stingsmay also cause severe allergic reactions in some patients with urticaria pigmentosa. Your doctor

may advise you to carry an EpiPen to be used in case of a bee sting.

Calling your health care provider Return to top

Call for an appointment with your health care provider if your child has symptoms suggesting urticaria

pigmentosa.

Update Date: 10/29/2004Updated by: Jonathan Kantor, M.D., Department of Dermatology, University of Pennsylvania MedicalCenter, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission(www.urac.org). URAC'saccreditation programis the first of its kind, requiring compliance with 53 standardsof quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve thisimportant distinction for online health information and services. Learn more about A.D.A.M.'s editorialprocess. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to theprinciples of the Health on the Net Foundation (www.hon.ch).

The information provided should not be used during any medical emergency or for the diagnosis ortreatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment

of any and all medical conditions. Call 911 for all medical emergencies. Adam makes no representation orwarranty regarding the accuracy, reliability, completeness, currentness, or timeliness of the content, textor graphics. Links to other sites are provided for information only -- they do not constitute endorsements ofthose other sites. Copyright 2005, A.D.A.M., Inc. Any duplication or distribution of the informationcontained herein is strictly prohibited.

Home | Health Topics | Drug Information | Encyclopedia | Dictionary |News | Directories | Other Resources

Copyright | Privacy | Accessibility | QualityGuidelines

U.S. National Library of Medicine, 8600Rockville Pike, Bethesda, MD 20894National Institutes of Health | Department ofHealth & Human Services

Page last updated: 09November 2005
http://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/002847.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/002847.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://medlineplus.gov/http://www.nlm.nih.gov/medlineplus/healthtopics.htmlhttp://www.nlm.nih.gov/medlineplus/druginformation.htmlhttp://www.nlm.nih.gov/medlineplus/encyclopedia.htmlhttp://www.nlm.nih.gov/medlineplus/mplusdictionary.htmlhttp://www.nlm.nih.gov/medlineplus/newsbydate.htmlhttp://www.nlm.nih.gov/medlineplus/directories.htmlhttp://www.nlm.nih.gov/medlineplus/otherresources.htmlhttp://www.nlm.nih.gov/medlineplus/copyright.htmlhttp://www.nlm.nih.gov/medlineplus/privacy.htmlhttp://www.nlm.nih.gov/medlineplus/accessibility.htmlhttp://www.nlm.nih.gov/medlineplus/criteria.htmlhttp://www.nlm.nih.gov/medlineplus/criteria.htmlhttp://www.nlm.nih.gov/cgi/medlineplus/leavemedplus.pl?theURL=http%3A%2F%2Fwww.nlm.nih.govhttp://www.nlm.nih.gov/cgi/medlineplus/leavemedplus.pl?theURL=http%3A%2F%2Fwww.nih.govhttp://www.nlm.nih.gov/cgi/medlineplus/leavemedplus.pl?theURL=http%3A%2F%2Fwww.hhs.govhttp://www.nlm.nih.gov/cgi/medlineplus/leavemedplus.pl?theURL=http%3A%2F%2Fwww.hhs.govhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://www.nlm.nih.gov/medlineplus/ency/article/002847.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/#tophttp://medlineplus.gov/http://www.nlm.nih.gov/medlineplus/healthtopics.htmlhttp://www.nlm.nih.gov/medlineplus/druginformation.htmlhttp://www.nlm.nih.gov/medlineplus/encyclopedia.htmlhttp://www.nlm.nih.gov/medlineplus/mplusdictionary.htmlhttp://www.nlm.nih.gov/medlineplus/newsbydate.htmlhttp://www.nlm.nih.gov/medlineplus/directories.htmlhttp://www.nlm.nih.gov/medlineplus/otherresources.htmlhttp://www.nlm.nih.gov/medlineplus/copyright.htmlhttp://www.nlm.nih.gov/medlineplus/privacy.htmlhttp://www.nlm.nih.gov/medlineplus/accessibility.htmlhttp://www.nlm.nih.gov/medlineplus/criteria.htmlhttp://www.nlm.nih.gov/medlineplus/criteria.htmlhttp://www.nlm.nih.gov/cgi/medlineplus/leavemedplus.pl?theURL=http%3A%2F%2Fwww.nlm.nih.govhttp://www.nlm.nih.gov/cgi/medlineplus/leavemedplus.pl?theURL=http%3A%2F%2Fwww.nih.govhttp://www.nlm.nih.gov/cgi/medlineplus/leavemedplus.pl?theURL=http%3A%2F%2Fwww.hhs.govhttp://www.nlm.nih.gov/cgi/medlineplus/leavemedplus.pl?theURL=http%3A%2F%2Fwww.hhs.gov


15/24

National Organization for Rare Disorders, Inc.

Urticaria Pigmentosa

Important

It is possible that the main title of the report UrticariaPigmentosa is not the name you expected. Please check the

synonyms listing to find the alternate name(s) anddisorder

subdivision(s) covered by this report.

Synonyms

Localized Infantile Mastocytosis

Mastocytosis, Infantile

Nettleship's, E. Disease Type I

Urticaria, Perstans Hemorrhagica

Xanthelasmoidea

Disorder Subdivisions

None

General Discussion

Urticaria pigmentosa is a rare skin disorder that is a

localized (cutaneous) form of mastocytosis. Some

clinicians suggest that urticaria pigmentosa is the childhoodform of mastocytosis. Mast cells are specialized cells of

connective tissue that release substances such as histamine

(a chemical important in the inflammatory process) and

heparin (an anti-clotting agent) when the bodys alarm

mechanism is set off. When mast cells cluster and multiplyexcessively (proliferate), histamine and heparin are released

into the skin (mastocytosis). The characteristic skin lesionsof urticaria pigmentosa appear in these areas. Urticaria

pigmentosa is generally benign and is usually self-limited.

The exact cause of the disease is not known, although somecases may be inherited.

New Search

Go

| Help |Search Menu |

Topic Contents

Synonyms

Disorder Subdivisions

General Discussion

Resources

For a Complete Report
http://www.bchealthguide.org/kbase/nord/#nord192-synonymshttp://www.bchealthguide.org/kbase/nord/#nord192-disorder-subdivisionshttp://www.bchealthguide.org/kbase/nord/#nord192-disorder-subdivisionshttp://www.bchealthguide.org/kbase/nord/#nord192-disorder-subdivisionshttp://www.bchealthguide.org/kbase/support/support-help-html/sec1.htmhttp://www.bchealthguide.org/kbase/default.htmhttp://www.bchealthguide.org/kbase/default.htmhttp://www.bchealthguide.org/kbase/nord/#nord192-synonymshttp://www.bchealthguide.org/kbase/nord/#nord192-disorder-subdivisionshttp://www.bchealthguide.org/kbase/nord/#nord192-general-discussionhttp://www.bchealthguide.org/kbase/nord/#nord192-resourceshttp://www.bchealthguide.org/kbase/nord/#nord192-CompleteReporthttp://www.bchealthguide.org/kbase/support/support-abouthw-html/sec1.htmhttp://www.bchealthguide.org/kbase/nord/#nord192-synonymshttp://www.bchealthguide.org/kbase/nord/#nord192-disorder-subdivisionshttp://www.bchealthguide.org/kbase/nord/#nord192-disorder-subdivisionshttp://www.bchealthguide.org/kbase/support/support-help-html/sec1.htmhttp://www.bchealthguide.org/kbase/default.htmhttp://www.bchealthguide.org/kbase/nord/#nord192-synonymshttp://www.bchealthguide.org/kbase/nord/#nord192-disorder-subdivisionshttp://www.bchealthguide.org/kbase/nord/#nord192-general-discussionhttp://www.bchealthguide.org/kbase/nord/#nord192-resourceshttp://www.bchealthguide.org/kbase/nord/#nord192-CompleteReport


16/24

.

Resources

NIH/National Institute of Allergy and Infectious

Diseases

9000 Rockville Pike

Building 31A

Bethesda, MD 20892

Tel: (301)496-5717Fax: (301)402-0120

Internet: http://www.niaid.nih.gov/

American Academy of Allergy Asthma and

Immunology

611 East Wells StreetMilwaukee, WI 53202

Tel: (414)272-6071

Fax: (414)276-3349

Tel: (800)822-2762Email: [email protected]

Internet: http://www.aaaai.org

For a Complete Report

This is an abstract of a report from the National

Organization for Rare Disorders, Inc. (NORD). A copy

of the complete report can be obtained for a small fee byvisiting the NORD website. The complete report contains

additional information including symptoms, causes,

affected population, related disorders, standard andinvestigational treatments (if available), and references

from medical literature. For a full-text version of this topic,

see http://www.rarediseases.org/search/rdblist.html

The information provided in this report isnot intended for diagnostic purposes. It is

provided for informational purposes only.

NORD recommends that affectedindividuals seek the advice or counsel of

their own personal physicians.

It is possible that the title of this topic is not

the name you selected. Please check the


17/24

Synonyms listing to find the alternatename(s) and Disorder Subdivision(s)

covered by this report.

This disease entry is based upon medical

information available through the date at theend of the topic. Since NORD's resources

are limited, it is not possible to keep every

entry in the Rare Disease Databasecompletely current and accurate. Please

check with the agencies listed in the

Resources section for the most currentinformation about this disorder.

For additional information and assistance

about rare disorders, please contact the

National Organization for Rare Disorders atP.O. Box 1968, Danbury, CT 06813-1968;

phone (203) 744-0100; web site

www.rarediseases.org or [email protected]

Last Updated: 3/18/2003

Copyright 1992, 1994, 2003 National

Organization for Rare Disorders, Inc.

Top of Page

Printer-Friendly

This information is not intended to replace the advice of a doctor.Healthwise disclaims any liability for the decisions you make based on this

information. For more information, click here. How this information wasdeveloped.
http://www.bchealthguide.org/kbase/nord/#_tophttp://print.healthwise.net/WebPrint/WebPrintMgr.aspx?c=moh&s=en-caQ3_05&h=nord192-Headerhttp://www.bchealthguide.org/kbase/support/support-abouthw-html/sec9.htmhttp://www.bchealthguide.org/kbase/support/support-abouthw-html/sec1.htmhttp://www.bchealthguide.org/kbase/support/support-abouthw-html/sec1.htmhttp://www.bchealthguide.org/kbase/support/support-abouthw-html/sec1.htmhttp://print.healthwise.net/WebPrint/WebPrintMgr.aspx?c=moh&s=en-caQ3_05&h=nord192-Headerhttp://www.bchealthguide.org/kbase/nord/#_tophttp://www.bchealthguide.org/kbase/nord/#_tophttp://print.healthwise.net/WebPrint/WebPrintMgr.aspx?c=moh&s=en-caQ3_05&h=nord192-Headerhttp://www.bchealthguide.org/kbase/support/support-abouthw-html/sec9.htmhttp://www.bchealthguide.org/kbase/support/support-abouthw-html/sec1.htmhttp://www.bchealthguide.org/kbase/support/support-abouthw-html/sec1.htm


18/24

The BC HealthGuide Program thanks our endorsers:

Dermatographism- close-up

Dermatographismon the arm

Urticariapigmentosa in the

armpit

Ma

c

Injury Disease Nutrition Po

Symptoms Surgery Test Spec

A B C D E F G H I J K L M N O P Q R S T U V W
http://www.shands.org/health/imagepages/2017.htmhttp://www.shands.org/health/imagepages/2017.htmhttp://www.shands.org/health/imagepages/2018.htmhttp://www.shands.org/health/imagepages/2018.htmhttp://www.shands.org/health/imagepages/2037.htmhttp://www.shands.org/health/imagepages/2037.htmhttp://www.shands.org/health/imagepages/2037.htmhttp://www.shands.org/health/imagepages/2438.htmhttp://www.shands.org/health/imagepages/2438.htmhttp://www.shands.org/health/information/acciidxa.htm%09http://www.shands.org/health/information/diseidxa.htm%09http://www.shands.org/health/information/nutridxa.htm%09http://www.shands.org/health/information/poisidxa.htm%09http://www.shands.org/health/information/sympidxa.htm%09http://www.shands.org/health/information/surgidxa.htm%09http://www.shands.org/health/information/testidxa.htm%09http://www.shands.org/health/information/miscidxa.htm%09http://www.shands.org/health/information/diseidxa.htmhttp://www.shands.org/health/information/diseidxb.htmhttp://www.shands.org/health/information/diseidxb.htmhttp://www.shands.org/health/information/diseidxc.htmhttp://www.shands.org/health/information/diseidxd.htmhttp://www.shands.org/health/information/diseidxd.htmhttp://www.shands.org/health/information/diseidxe.htmhttp://www.shands.org/health/information/diseidxf.htmhttp://www.shands.org/health/information/diseidxg.htmhttp://www.shands.org/health/information/diseidxh.htmhttp://www.shands.org/health/information/diseidxi.htmhttp://www.shands.org/health/information/diseidxj.htmhttp://www.shands.org/health/information/diseidxj.htmhttp://www.shands.org/health/information/diseidxk.htmhttp://www.shands.org/health/information/diseidxl.htmhttp://www.shands.org/health/information/diseidxl.htmhttp://www.shands.org/health/information/diseidxm.htmhttp://www.shands.org/health/information/diseidxn.htmhttp://www.shands.org/health/information/diseidxo.htmhttp://www.shands.org/health/information/diseidxo.htmhttp://www.shands.org/health/information/diseidxp.htmhttp://www.shands.org/health/information/diseidxq.htmhttp://www.shands.org/health/information/diseidxr.htmhttp://www.shands.org/health/information/diseidxs.htmhttp://www.shands.org/health/information/diseidxt.htmhttp://www.shands.org/health/information/diseidxu.htmhttp://www.shands.org/health/information/diseidxv.htmhttp://www.shands.org/health/information/diseidxw.htmhttp://www.shands.org/health/imagepages/2484.htmhttp://www.shands.org/health/imagepages/2438.htmhttp://www.shands.org/health/imagepages/2037.htmhttp://www.shands.org/health/imagepages/2018.htmhttp://www.shands.org/health/imagepages/2017.htmhttp://www.shands.org/health/http://www.shands.org/default.asphttp://www.bcpharmacists.org/http://www.crnbc.ca/http://www.bccfp.bc.ca/http://www.bcma.org/http://www.bchealthguide.org/kbaltindex.asphttp://www.bchealthguide.org/aboutprogram.stmhttp://www.bchealthguide.org/terms.stmhttp://www.shands.org/health/information/acciidxa.htm%09http://www.shands.org/health/information/diseidxa.htm%09http://www.shands.org/health/information/nutridxa.htm%09http://www.shands.org/health/information/poisidxa.htm%09http://www.shands.org/health/information/sympidxa.htm%09http://www.shands.org/health/information/surgidxa.htm%09http://www.shands.org/health/information/testidxa.htm%09http://www.shands.org/health/information/miscidxa.htm%09http://www.shands.org/health/information/diseidxa.htmhttp://www.shands.org/health/information/diseidxb.htmhttp://www.shands.org/health/information/diseidxc.htmhttp://www.shands.org/health/information/diseidxd.htmhttp://www.shands.org/health/information/diseidxe.htmhttp://www.shands.org/health/information/diseidxf.htmhttp://www.shands.org/health/information/diseidxg.htmhttp://www.shands.org/health/information/diseidxh.htmhttp://www.shands.org/health/information/diseidxi.htmhttp://www.shands.org/health/information/diseidxj.htmhttp://www.shands.org/health/information/diseidxk.htmhttp://www.shands.org/health/information/diseidxl.htmhttp://www.shands.org/health/information/diseidxm.htmhttp://www.shands.org/health/information/diseidxn.htmhttp://www.shands.org/health/information/diseidxo.htmhttp://www.shands.org/health/information/diseidxp.htmhttp://www.shands.org/health/information/diseidxq.htmhttp://www.shands.org/health/information/diseidxr.htmhttp://www.shands.org/health/information/diseidxs.htmhttp://www.shands.org/health/information/diseidxt.htmhttp://www.shands.org/health/information/diseidxu.htmhttp://www.shands.org/health/information/diseidxv.htmhttp://www.shands.org/health/information/diseidxw.htmhttp://www.shands.org/health/imagepages/2017.htmhttp://www.shands.org/health/imagepages/2017.htmhttp://www.shands.org/health/imagepages/2018.htmhttp://www.shands.org/health/imagepages/2018.htmhttp://www.shands.org/health/imagepages/2037.htmhttp://www.shands.org/health/imagepages/2037.htmhttp://www.shands.org/health/imagepages/2037.htmhttp://www.shands.org/health/imagepages/2438.htmhttp://www.shands.org/health/imagepages/2438.htmhttp://www.shands.org/health/imagepages/2438.htm


19/24

Urticariapigmentosa on the

chest

Urticariapigmentosa -

close-up

Dermatographismon the back


Alternative Names:Mastocytosis; Mastocytoma

Treatment:

Antihistamines may relieve some of the histamine-induced symptoms such asflushing. Discuss the choice of antihistamine with your child's health care provmore severe and unusual forms of urticaria pigmentosa, systemic symptoms be controlled with other medications.

Expectations (prognosis):

Urticaria pigmentosa goes away by puberty in about half of the affected childrSymptoms usually decrease in the remaining patients as they grow into adult

In adults, urticaria pigmentosa may progress to the more serious systemic ma

Complications:

Discomfort from itching, and possible self-consciousness about the "spots," aprimary complications. Other problems such as diarrheaandfainting are rare

Certain medications may trigger flares of urticaria pigmentosa. Discuss thesedoctor.

Bee stings may also cause severe allergic reactions in some patients with urt

pigmentosa. Your doctor may advise you to carry an EpiPen to be used in cassting.

Calling your health care provider:

Call for an appointment with your health care provider if your child has symptosuggesting urticaria pigmentosa.

Overview Symptoms Treatment Prev
http://www.shands.org/health/imagepages/2484.htmhttp://www.shands.org/health/imagepages/2484.htmhttp://www.shands.org/health/imagepages/2484.htmhttp://www.shands.org/health/imagepages/2485.htmhttp://www.shands.org/health/imagepages/2485.htmhttp://www.shands.org/health/imagepages/2485.htmhttp://www.shands.org/health/imagepages/2780.htmhttp://www.shands.org/health/imagepages/2780.htmhttp://www.shands.org/health/information/003241.htmhttp://www.shands.org/health/information/003217.htmhttp://www.shands.org/health/information/003126.htmhttp://www.shands.org/health/information/003126.htmhttp://www.shands.org/health/information/003092.htmhttp://www.shands.org/health/information/003092.htmhttp://www.shands.org/health/information/002847.htmhttp://www.shands.org/health/information/001466.htmhttp://www.shands.org/health/information/001466sym.htmhttp://www.shands.org/health/information/001466trt.htmhttp://www.shands.org/health/information/001466prv.htmhttp://www.shands.org/health/imagepages/2780.htmhttp://www.shands.org/health/imagepages/2485.htmhttp://www.shands.org/health/imagepages/2484.htmhttp://www.shands.org/health/imagepages/2484.htmhttp://www.shands.org/health/imagepages/2484.htmhttp://www.shands.org/health/imagepages/2485.htmhttp://www.shands.org/health/imagepages/2485.htmhttp://www.shands.org/health/imagepages/2485.htmhttp://www.shands.org/health/imagepages/2780.htmhttp://www.shands.org/health/imagepages/2780.htmhttp://www.shands.org/health/information/001466.htmhttp://www.shands.org/health/information/001466sym.htmhttp://www.shands.org/health/information/001466trt.htmhttp://www.shands.org/health/information/001466prv.htmhttp://www.shands.org/health/information/003241.htmhttp://www.shands.org/health/information/003217.htmhttp://www.shands.org/health/information/003126.htmhttp://www.shands.org/health/information/003092.htmhttp://www.shands.org/health/information/002847.htm


20/24

A.D.A.M., Inc. is accredited by URAC, also known as the American AccreditationCommission (www.urac.org). URAC'saccreditation programis the first of its kindcompliance with 53 standards of quality and accountability, verif ied by independA.D.A.M. is among the first to achieve this important distinction for online health services. Learn more about A.D.A.M.'seditorial reviewers. A.D.A.M. is also a fouHi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on thFoundation (www.hon.ch).

The information provided herein should not be used during any medical emerthe diagnosis or treatment of any medical condition. A licensed physician shoconsulted for diagnosis and treatment of any and all medical conditions. Call 9medical emergencies. Links to other sites are provided for information only -- constitute endorsements of those other sites. Copyright 2004 A.D.A.M., Inc. Aduplication or distribution of the information contained herein is strictly prohibi

home | doctors' offices | hospitals | jobs at shands | e-newsroom | about shandsuf health science center | contact us | copyright | terms & privacy | FAQ

Health Illustrated Encyclopedia

Injury ReferenceDisease Reference

Surgery ReferenceSpecial Topic ReferencePoison ReferenceNutrition ReferenceSymptoms ReferenceTest Reference

health newslettersPregnancy Health Center

Surgeries, Procedures, and Tests

enciclopedia de salud ilustradahealth in a heartbeat

search

Lifespan's A - Z Health Information

Library

Injury Disease Nutrition Poison Symptoms Surgery Test Special Topic

A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

Overview Symptoms Treatment Prevention
http://www.shands.org/default.asphttp://www.shands.org/offices/http://www.shands.org/hospitals/http://www.shands.org/hospitals/http://www.shands.org/jobs/http://www.shands.org/jobs/http://www.shands.org/news/http://www.shands.org/news/http://www.shands.org/about/http://www.shands.org/about/http://www.health.ufl.edu/http://www.health.ufl.edu/http://www.shands.org/contactus/http://www.shands.org/copyright.htmhttp://www.shands.org/copyright.htmhttp://www.shands.org/terms.htmhttp://www.shands.org/public/faq/http://www.shands.org/health/informationhttp://www.shands.org/health/information/acciidxa.htmhttp://www.shands.org/health/information/diseidxa.htmhttp://www.shands.org/health/information/surgidxa.htmhttp://www.shands.org/health/information/miscidxa.htmhttp://www.shands.org/health/information/poisidxa.htmhttp://www.shands.org/health/information/nutridxa.htmhttp://www.shands.org/health/information/sympidxa.htmhttp://www.shands.org/health/information/testidx1.htmhttp://www.shands.org/health/newsletterhttp://www.shands.org/health/Pregnancy/http://www.shands.org/health/surgeries/100000.htmlhttp://www.shands.org/health/spanishhttp://www.shands.org/health/heartbeathttp://www.lifespan.org/ADAM/English/HIE/acciidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/acciidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/nutridxa.htmhttp://www.lifespan.org/ADAM/English/HIE/nutridxa.htmhttp://www.lifespan.org/ADAM/English/HIE/poisidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/sympidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/sympidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/surgidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/testidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/testidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/miscidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/miscidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxb.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxb.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxc.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxc.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxd.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxd.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxe.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxf.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxg.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxg.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxh.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxh.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxi.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxj.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxk.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxl.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxm.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxn.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxo.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxo.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxp.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxq.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxr.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxs.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxs.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxt.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxt.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxu.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxv.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxv.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxw.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxx.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxx.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxy.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxz.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxz.htmhttp://www.lifespan.org/ADAM/English/HIE/001466.htmhttp://www.lifespan.org/ADAM/English/HIE/001466.htmhttp://www.lifespan.org/ADAM/English/HIE/001466sym.htmhttp://www.lifespan.org/ADAM/English/HIE/001466trt.htmhttp://www.lifespan.org/ADAM/English/HIE/001466prv.htmhttp://www.lifespan.org/ADAM/English/HIE/001466prv.htmhttp://www.adam.com/http://www.shands.org/default.asphttp://www.shands.org/offices/http://www.shands.org/hospitals/http://www.shands.org/jobs/http://www.shands.org/news/http://www.shands.org/about/http://www.health.ufl.edu/http://www.shands.org/contactus/http://www.shands.org/copyright.htmhttp://www.shands.org/terms.htmhttp://www.shands.org/public/faq/http://www.shands.org/health/informationhttp://www.shands.org/health/information/acciidxa.htmhttp://www.shands.org/health/information/diseidxa.htmhttp://www.shands.org/health/information/surgidxa.htmhttp://www.shands.org/health/information/miscidxa.htmhttp://www.shands.org/health/information/poisidxa.htmhttp://www.shands.org/health/information/nutridxa.htmhttp://www.shands.org/health/information/sympidxa.htmhttp://www.shands.org/health/information/testidx1.htmhttp://www.shands.org/health/newsletterhttp://www.shands.org/health/Pregnancy/http://www.shands.org/health/surgeries/100000.htmlhttp://www.shands.org/health/spanishhttp://www.shands.org/health/heartbeathttp://www.lifespan.org/ADAM/English/HIE/acciidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/nutridxa.htmhttp://www.lifespan.org/ADAM/English/HIE/poisidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/sympidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/surgidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/testidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/miscidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxa.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxb.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxc.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxd.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxe.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxf.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxg.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxh.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxi.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxj.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxk.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxl.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxm.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxn.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxo.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxp.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxq.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxr.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxs.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxt.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxu.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxv.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxw.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxx.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxy.htmhttp://www.lifespan.org/ADAM/English/HIE/diseidxz.htmhttp://www.lifespan.org/ADAM/English/HIE/001466.htmhttp://www.lifespan.org/ADAM/English/HIE/001466sym.htmhttp://www.lifespan.org/ADAM/English/HIE/001466trt.htmhttp://www.lifespan.org/ADAM/English/HIE/001466prv.htm


21/24

Careers at LifespanVolunteer your timeOther ways to give


Alternative Names

Mastocytosis; Mastocytoma

Treatment

Antihistamines may relieve some of the histamine-induced symptoms such as itchingand flushing. Discuss the choice of antihistamine with your child's health careprovider. With more severe and unusual forms of urticaria pigmentosa, systemicsymptoms should be controlled with other medications.

Outlook (Prognosis)

Urticaria pigmentosa goes away by puberty in about half of the

affected children. Symptoms usually decrease in the remaining

patients as they grow into adulthood.

In adults, urticaria pigmentosa may progress to the more serioussystemic mastocytosis.

Possible Complications

Discomfort from itching, and possible self-consciousness about the

"spots," are the primary complications. Other problems such as

diarrhea and fainting are rare.

Certain medications may trigger flares of urticaria pigmentosa.

Discuss these with your doctor.

Bee stings may also cause severe allergic reactions in some patients

with urticaria pigmentosa. Your doctor may advise you to carry anEpiPen to be used in case of a bee sting.

When to Contact a Medical Professional

Call for an appointment with your health care provider if your child has symptomssuggesting urticaria pigmentosa.


Dermatographismon the arm

Urticariapigmentosa in the

armpit

Mastocytosis,diffuse

cutaneous
http://www.lifespan.org/ADAM/English/HIE/002847.htmhttp://www.lifespan.org/ADAM/English/Images/2017.htmhttp://www.lifespan.org/ADAM/English/Images/2017.htmhttp://www.lifespan.org/ADAM/English/Images/2017.htmhttp://www.lifespan.org/ADAM/English/Images/2018.htmhttp://www.lifespan.org/ADAM/English/Images/2018.htmhttp://www.lifespan.org/ADAM/English/Images/2037.htmhttp://www.lifespan.org/ADAM/English/Images/2037.htmhttp://www.lifespan.org/ADAM/English/Images/2037.htmhttp://www.lifespan.org/ADAM/English/Images/2438.htmhttp://www.lifespan.org/ADAM/English/Images/2438.htmhttp://www.lifespan.org/ADAM/English/Images/2438.htmhttp://www.lifespan.org/ADAM/English/Images/2438.htmhttp://www.lifespan.org/ADAM/English/Images/2438.htmhttp://www.lifespan.org/ADAM/English/Images/2037.htmhttp://www.lifespan.org/ADAM/English/Images/2018.htmhttp://www.lifespan.org/ADAM/English/Images/2017.htmhttp://www.lifespan.org/ADAM/English/HIE/002847.htmhttp://www.lifespan.org/ADAM/English/Images/2017.htmhttp://www.lifespan.org/ADAM/English/Images/2017.htmhttp://www.lifespan.org/ADAM/English/Images/2018.htmhttp://www.lifespan.org/ADAM/English/Images/2018.htmhttp://www.lifespan.org/ADAM/English/Images/2037.htmhttp://www.lifespan.org/ADAM/English/Images/2037.htmhttp://www.lifespan.org/ADAM/English/Images/2037.htmhttp://www.lifespan.org/ADAM/English/Images/2438.htmhttp://www.lifespan.org/ADAM/English/Images/2438.htmhttp://www.lifespan.org/ADAM/English/Images/2438.htm


22/24

Urticariapigmentosa on the

chest

Urticariapigmentosa -

close-up

Dermatographismon the back

Review Date: 10/29/2004Reviewed By: Jonathan Kantor, M.D., Department of Dermatology, University ofPennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMedHealthcare Network.

A.D.A.M., Inc. is accredited by URAC, also known as the AmericanAccreditation HealthCare Commission (www.urac.org). URAC'saccreditation

program is the first of its kind, requiring compliance with 53 standards of qualityand accountability, verified by independent audit. A.D.A.M. is among the first toachieve this important distinction for online health information and services.Learn more about A.D.A.M.'seditorial process. A.D.A.M. is also a founding

member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of theHealth on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A

licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to

other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2005A.D.A.M., Inc. Any

duplication or distribution of the information contained herein is strictly prohibited.

Home Search News Find a Physician CareersServices Better Health Get Involved

About Us Feedback Site Map

Copyright 2004, Lifespan Web site design by Lifespan

By using this website, you accept theterms of ouruser agreement. Please read it.


Injury Disease Nutrition Poison

Symptoms Surgery Test Special Topic

A B C D E F G H I J K L MN O P Q R S T U V W X Y Z
http://www.lifespan.org/ADAM/English/Images/2484.htmhttp://www.lifespan.org/ADAM/English/Images/2484.htmhttp://www.lifespan.org/ADAM/English/Images/2484.htmhttp://www.lifespan.org/ADAM/English/Images/2485.htmhttp://www.lifespan.org/ADAM/English/Images/2485.htmhttp://www.lifespan.org/ADAM/English/Images/2485.htmhttp://www.lifespan.org/ADAM/English/Images/2485.htmhttp://www.lifespan.org/ADAM/English/Images/2780.htmhttp://www.lifespan.org/ADAM/English/Images/2780.htmhttp://www.lifespan.org/http://www.lifespan.org/http://www.lifespan.org/Search.htmlhttp://www.lifespan.org/news/http://www.lifespan.org/news/http://www.lifespan.org/Services/FindaDoc/http://www.lifespan.org/Services/FindaDoc/http://www.lifespan.org/Services/hr/http://www.lifespan.org/Services/services.htmlhttp://www.lifespan.org/Services/services.htmlhttp://www.lifespan.org/staywell/http://www.lifespan.org/staywell/http://www.lifespan.org/GetInvolved/http://www.lifespan.org/about/http://www.lifespan.org/feedback.htmlhttp://www.lifespan.org/sitemap.htmlhttp://www.lifespan.org/agreement.htmlhttp://www.lifespan.org/agreement.htmlhttp://www.humed.com/humc_ency/ency/images/2017.htmhttp://www.humed.com/humc_ency/ency/images/2017.htmhttp://www.humed.com/humc_ency/ency/article/acciidxa.htm%09http://www.humed.com/humc_ency/ency/article/diseidxa.htm%09http://www.humed.com/humc_ency/ency/article/nutridxa.htm%09http://www.humed.com/humc_ency/ency/article/poisidxa.htm%09http://www.humed.com/humc_ency/ency/article/sympidxa.htm%09http://www.humed.com/humc_ency/ency/article/surgidxa.htm%09http://www.humed.com/humc_ency/ency/article/testidxa.htm%09http://www.humed.com/humc_ency/ency/article/miscidxa.htm%09http://www.humed.com/humc_ency/ency/article/miscidxa.htm%09http://www.humed.com/humc_ency/ency/article/diseidxa.htmhttp://www.humed.com/humc_ency/ency/article/diseidxb.htmhttp://www.humed.com/humc_ency/ency/article/diseidxc.htmhttp://www.humed.com/humc_ency/ency/article/diseidxc.htmhttp://www.humed.com/humc_ency/ency/article/diseidxd.htmhttp://www.humed.com/humc_ency/ency/article/diseidxd.htmhttp://www.humed.com/humc_ency/ency/article/diseidxe.htmhttp://www.humed.com/humc_ency/ency/article/diseidxf.htmhttp://www.humed.com/humc_ency/ency/article/diseidxg.htmhttp://www.humed.com/humc_ency/ency/article/diseidxh.htmhttp://www.humed.com/humc_ency/ency/article/diseidxh.htmhttp://www.humed.com/humc_ency/ency/article/diseidxi.htmhttp://www.humed.com/humc_ency/ency/article/diseidxj.htmhttp://www.humed.com/humc_ency/ency/article/diseidxk.htmhttp://www.humed.com/humc_ency/ency/article/diseidxl.htmhttp://www.humed.com/humc_ency/ency/article/diseidxm.htmhttp://www.humed.com/humc_ency/ency/article/diseidxn.htmhttp://www.humed.com/humc_ency/ency/article/diseidxo.htmhttp://www.humed.com/humc_ency/ency/article/diseidxp.htmhttp://www.humed.com/humc_ency/ency/article/diseidxq.htmhttp://www.humed.com/humc_ency/ency/article/diseidxr.htmhttp://www.humed.com/humc_ency/ency/article/diseidxs.htmhttp://www.humed.com/humc_ency/ency/article/diseidxt.htmhttp://www.humed.com/humc_ency/ency/article/diseidxu.ht

Documents

Urticaria Pigmentosa All