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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)