Xanthogranuloma adultes D76.3

Very rare, benign "granulomatous" disease of the non-Langerhans cell histiocytosis type occurring on skin and mucous membranes with coarse yellowish-reddish papules, plaques and nodules (for classification see xanthogranulomas).

Face (periorbital region), neck, forearms; rarely in the genital region. Eye involvement with conjunctivitis, keratitis, scleritis or uveitis is possible.

Solitary or oligolesional skin-colored, yellow or yellow-red, asymptomatic papules or nodules with a smooth surface. No spontaneous regression; no association with systemic disease.

Epidermis flattened, no epidermotropism. Dense infiltrates in papillary and reticular dermis consisting of histiocytes, foam cells, and giant Touton-type cells. Focal lymphohistiocytic infiltrates and neutrophilic and eosinophilic granulocytes. Frequent (30-40% of cases) infiltration of deeper tissues such as fascia and muscle.

Immunohistology: Stabilin-1(MS-1 HMWP)-positive and CD163-positive histiocytes; S100- and CD1a negative.

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