21 May 2010

Monocyte / Macrophage Disorders



Monocyte / Macrophage Disorders
Northeast Regional Medical Center/KCOM

Granuloma Annulare
* Localized
* Generalized
* Macular
* Deep
* Perforating
* In HIV
* In Lymphoma
* Common, Idiopathic, all races
* 50% patients IgM and C3 in vessels
* LCV changes sometimes seen
* Suggests Ab mediated vasculitis
* Common in HIV patients
* EBV sometimes found
* Occurs in resolved lesions Zoster

GA - Histology
Interstitial GA
* Upper dermis
* “Skip areas”
* Mucin
* Deep dermis, subQ
* No “skip” areas
* No mucin

Localized GA
* Young adults
* Acral
* Annular, scalloped
* White or pink flat topped papules spread peripherally
* 75% clear in 2 yrs
* 25% last 8 yrs
Diffuse GA
Subcutaneous GA
Perforating GA
GA in HIV disease
GA and Lymphoma
GA- Treatment
Annular Elastolytic Giant Cell Granuloma of Meischer/Actinic Granuloma of O’Brien
Photoexacerbated GA
Granuloma Mulitforme of Leiker
Sarcoidosis
* Multisystem Disease
* Lungs, lymph nodes, skin and eyes MC.
* 10x more frequent in blacks in US
* Women under age 40
* Irish, African, Afro-Caribbean.
* Presence inversely proportional to the incidence of TB and/or Leprosy.
* Etiology unknown
* HLA-A1 – Lofgren’s syndrome
* HLA-B13 – Chronic & Persistent form
* HLA-B8
* HLA-DR3
* Final common pathway is granuloma formation
“NAKED” GRANULOMAS
“NAKED” meanse a sparse rather than a dense infiltrate. Lymphocytes, macrophages & fibroblasts may occur
Sarcoid Skin Involvement
Sarcoid – like syphillis, mimics many other dz’s
Papular Sarcoid
Annular Sarcoidosis
Hypopigmented Sarcoid
Lupus
Pernio
Punched-Out Lytic lesions, Bone Cysts
Ulcerative Sarcoidosis
Lupus Pernio
Darier-Roussy Sarcoid
Scar Sarcoid
Erythrodermic Sarcoid
Ichthyosiform Sarcoid
Alopecia
Morpheaform Sarcoid
Mucosal Sarcoid
Erythema Nodosum in Sarcoid
Systemic Sarcoidosis
Heerfort’s Syndrome
Mikulicz’s Syndrome
CXR- Hilar Adenopathy
Sarcoidosis in Fingers
Candle-wax drippings – granulomatous uveitis
Sarcoid - Treatment
Non-X Histocytoses
* Juvenile Xanthogranuloma
* Benign Cephalic Histiocytosis
* Solitary/Multicentric Reticulohistiocytosis
* Generalized Eruptive Histiocytoma
* Necrobiotic Xanthogranuloma
* Xanthoma Disseminatum
* Papular Xanthoma
* Indeterminate Cell Histiocytosis
* Progressive Nodular Histiocytoma
* Hereditary Progressive Mucinous Histiocytosis
* Rosai-Dorfman Disease
* Sea-Blue Histiocytosis
Juvenile Xanthogranuloma (JXG)
JXG Histopathology
Reticulohistiocytosis
Reticulohistiocytic Granuloma
Multicentric Reticulohistiocytosis
“Coral Bead” Paronychia
Tx: Multicentric Reticulohisticytosis
Generalized Eruptive Histiocytoma
Necrobiotic Xanthogranuloma (NXG)
NXG and Malignancy
Xanthoma Disseminatum
XD - Pathology
Papular Xanthoma
Indeterminate Cell Histiocytosis
Progressive Nodular Histiocytosis
Hereditary Progressive Mucinous Histiocytosis in Women
Rosai-Dorfman Disease
Rosai-Dorfman Disease – LN Biopsy
RDD - Emperipolesis – Histiocytes engulf plasma cells and lymphocytes
RDD - Treatment
Sea-Blue Histiocytosis
Sea-Blue Histiocytosis – Bone Marrow
X-type Histiocytoses
Hashimoto-Pritzker
Hashimoto-Pritzker Before and After
H-P MANAGEMENT
Histiocytosis X
Histiocytosis X - TX

Monocyte / Macrophage Disorders.ppt

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