Palisaded Granulomatous Dermatoses

Palisaded Granulomatous

Dermatoses

• GA

• Annular Elastolytic Giant Cell Granuloma

and Actinic Granuloma

• Granuloma Multiforme

• Interstitial Granulomatous Drug Rxn

• Necrobiotic Xanthogranuloma

Missinglink.ucsf.edu

GA

• Localized

• Generalized

• Patch-type or Macular

• Subcutaneous

• Perforating

• In HIV disease

• And Malignant Neoplasms

Matching • *Women 5th and 6th decade

• Young boys

• Children and young to middle aged

• Hands, feet, elbows and ankles

• Upper medial thighs

• Distal extremities or scalp

• *Papule dorsal hands with central keratotic core

• Assoc sun exposure

• Assoc autoimmune thyroiditis

• Assoc trauma

• Pseudorheumatoid nodule

1. Localized

2. Generalized

3. Patch-type or Macular

4. Subcutaneous

5. Perforating

6. In HIV disease

7. And Malignant Neoplasms

Missinglink.ucsf.edu

Missinglink.ucsf.edu

Treatment

• What are treatments for localized GA?

– Do nothing

– IL TAC

– Topical steroids or tacrolimus

• What are treatments for generalized GA?

– Tetracycline 500mg bid + nicotinamide 500mg tid

– Tetracycline 500mg bid +PUVA

– Dapsone, pentoxifylline, potassium iodide SSKI,

cyclosporin, UVA1, defibrotide, tranilast, antimalarials

Generalized granuloma annulare treated with

short-term administration of etretinate.

JAm Acad Dermatol. 2006 May;54(5

Suppl):S245-7.

Asano Y, Saito A, Idezuki T, Igarashi A.

Skinandaging.com

Matching • Central Africa

• Blindness

• *Sun damaged skin

• Meischer’s

• Leiker

• O’Brien

• IgG paraproteinemia

• assoc temporal arteritis

• Assoc CCB

• Deep dermal perivascular and interstitial infiltrate of neutr, eos, histiocytes and giant cells surrounding degenerated collagen

• atrophic yellow thin plaque forehead

• *interstitial giant cells & macrophages with elastophagocytosis, decreased elastic tissue

1. Annular Elastolytic Giant

Cell Granuloma

2. Actinic Granuloma

3. Granuloma Multiforme

4. Interstitial

Granulomatous Drug Rxn

5. Necrobiotic

Xanthogranuloma

Dermatopathology interactive atlas

Som.tulane.edu

Photograph by Victoria Nguyen, MD (me!)

Som.tulane.edu

Dermatopathology interactive atlas

Dermpath interactive atlas

Dermpath interactive atlas

Dermpath interactive atlas

Sarcoidosis

• In the US, which race is sarcoidosis most prevalent in?

– African-Americans

• In African-Americans, compared to Caucasians, do you see more specific or nonspecific cutaneous lesions?

– Specific 50% compared

• What is the pediatric sarcoidosis early triad?

– Skin lesions

– Uveitis

– Arthritis

Matching • **African Americans

• ****Systemic disease

• nonspecific

• Hilar adenopathy

• Rhinophyma-like

• *Elevated ESR

• Elevated ACE

• *Bone cysts

• Infiltration of tattoos

• Chronic pulmonary fibrosis

1. Papular sarcoid

2. Annular sarcoidosis

3. Hypopigmented sarcoidosis

4. Lupus pernio

5. Ulcerative sarcoidosis

6. Subcutaneous sarcoidosis

7. Sarcoidosis in scars

8. Plaques

9. Erythrodermic sarcoidosis

10. Ichthyosiform sarcoidosis

11. Alopecia

12. Morpheaform sarcoidsois

13. Mucosal sarcoidosis

14. Erythema nodosum in sarcoidosis

15. Systemic sarcoidosis

More Matching • Good prognosis

• **Head and neck

• **Primarily Legs

• Miliary sarcoid

• Uveo-parotid fever/Heerfordt syndrome

• Darier-Roussy

• Mikulicz syndrome

• Lofgren syndrome

• Uhthoff phenomenon

1. Papular sarcoid

2. Annular sarcoidosis

3. Hypopigmented sarcoidosis

4. Lupus pernio

5. Ulcerative sarcoidosis

6. Subcutaneous sarcoidosis

7. Sarcoidosis in scars

8. Plaques

9. Erythrodermic sarcoidosis

10. Ichthyosiform sarcoidosis

11. Alopecia

12. Morpheaform sarcoidsois

13. Mucosal sarcoidosis

14. Erythema nodosum in sarcoidosis

15. Systemic sarcoidosis

Sarcoidosis

• Which cytokine polymorphisms are

associated with sarcoidosis?

– TNF-alpha

– IFN-alpha

– IL-12

Dermatopathology interactive atlas

Dermatopathology interactive atlas

Dermatopathology interactive atlas

Dermatopathology interactive atlas

Diagnosis Sarcoidosis

• How do you establish the dx of sarcoidosis?

– Demonstrate two organ systems:

– Cutaneous

– Pulmonary

– Ophthalmologic

– Salivary glands

Sarcoidosis and Immunologic

Abnormalities

• Which immunologic abnormalities are

associated with sarcoidosis?

– Hodgkin’s lymphoma

– IFN-alpha therapy

– Hep C

– HAART

– Hematopoetic stem cell transplantation

Which of the following is not a

treatment for cutaneous

sarcoidosis?

a. Systemic corticosteroids

b. IL kenalog

c. Doxycycline

d. Hydroxychloroquine

e. Methotrexate

f. Isotretinoin

g. Interferon

h. Thalidomide

i. Etanercept

Non-X Histiocytoses • Juvenile Xanthogranuloma

• Benign Cephalic Histiocytosis

• Generalized Eruptive Histiocytoma

• Xanthoma Disseminatum (Montgomery syndrome)

• Progressive Nodular Histiocytosis

• Papular Xanthoma

• Hereditary Progressive Mucinous Histiocytosis in Women

• Reticulohistiocytosis

• Intermediate Cell Histiocytosis

• Sea-Blue Histiocytosis

Dermatlas.med.jhmi.edu

Which of the following is not true of

JXG?

a. Extracutaneous sites include lung, liver, and testis

b. Extracutaneous JXG is uncommon, presenting in 5% of multiple cutaneous cases

c. Children with JXG should be examined for stigmata for NF-1 and if found the pediatrician should be alerted of possible JCML

d. JXG is a proliferation of dermal dendrocytes, in contrast to LCH which is a proliferation of langerhans cells

e. Children with multiple JXG under 3 yrs need ocular screening

Dermpath interactive atlas

Dermpath interactive atlas

Dermpath interactive atlas

CD 68

JXG

• In what age group is this found?

– 5-17% birth

– 40-70% 1st yr of life

– Adults

• What are the three forms?

– Small nodular (2-5mm)

– Large nodular (5-20mm)

– Giant (>20mm)

Dermpath interactive atlas

Which of the following

statements regarding multicentric

reticulohistiocytosis is false? a. Multisystem disease begins age 50

b. Hand and finger involvement is the most common with classic “coral bead” appearance

c. Nodules over joints can resemble rheumatoid nodules

d. Associated arthropathy is inflammatory, symmetrical polyarticular arthritis and can be confused with rheumatoid or psoriatic arthritis

e. Treatment should be geared to cutaneous lesions, not arthritis

f. Should screen for malignancy, TB and myocardial involvement

Dermpath interactive atlas

Dermpath interactive atlas

Matching: Other Non-X

Histiocytoses

• foamy scalloped macrophages

• S100 and CD1a positive

• Leonine facies

• *Visceral involvement

• Giemsa and May-Gruenwald positive

• Abundant mucin

• Benign Cephalic Histiocytosis

• Generalized Eruptive Histiocytoma

• Xanthoma Disseminatum (Montgomery syndrome)

• Progressive Nodular Histiocytosis

• Papular Xanthoma

• Hereditary Progressive Mucinous Histiocytosis in Women

• Intermediate Cell Histiocytosis

• Sea-Blue Histiocytosis

What distinguishes Non-X

histiocytoses from X-type

histiocytoses?

• Langerhans cells present in X-type

• Monocyte/macrophages infiltrates in non-X

type

X-type Histiocytoses

• Congenital Self-Healing

Reticulohistiocytosis (Hashimoto-Pritzker)

• Langerhans Cell Histiocytosis

(Histiocytosis X)

Dermpath interactive atlas

Dermpath interactive atlas

Dermpath interactive atlas

Dental.mu.edu

Dental.mu.edu

Radiology.vcu.edu

Which statement regarding LCH

is false? a. Localized LCH is more common in children and

multisystem in adults

b. Associated ALL and AML have occurred in LCH pts

c. Visceral involvement includes bone, LN, bone marrow, liver, and lungs

d. Endocrine dysfxn is in form of diabetes mellitus

e. In multisystem disease of childhood, vinblastine and corticosteroids are the standard

Dermpath interactive atlas

Dermpath interactive atlas

Dermpath interactive atlas CD1A

Dermpath interactive atlas

Alta Dermatology

Victoria Nguyen Wang, MD

27882 Forbes RD Suite 201

Laguna Nigel, CA 92677

(949) 364-9264