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generalized eruptive histiocytosis

Tuesday 16 December 2003

Definition : Generalized eruptive histiocytosis belongs to the cutaneous non-Langerhans cell histiocytoses and is a rare monocyte-macrophage proliferative disorder that usually follows a benign clinical course. It can be considered as a systemic multifocal juvenile xanthogranuloma.

Synopsis

- erythematous macules and slightly elevated papules widely distributed over the trunk, neck, face, and thighs
- diffuse histiocytic infiltrate in the skin

Ultrastructurally

- no Birbeck granules

Immunochemistry

- CD68+
- S-100+/-
- CD1a-
- MS-1 high molecular weight protein + (highly characteristic for cutaneous non-Langerhans cell histiocytoses)

Associations

- acute monocytic leukemia (14576638)