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