Benign cephalic histiocytosis

Orpha code: 157997OMIM code:

Definition

A rare non-Langerhans cell histiocytosis characterized by multiple small yellowish-red or brown papules initially erupting predominantly in the head and neck region. The histopathological hallmark of these eventually self-healing lesions is a dermal proliferation of histiocytes with intracytoplasmic comma-shaped bodies, coated vesicles, and desmosome-like structures. Birbeck granules are absent. The disease typically occurs in young children.

Disease data
Classification

Disease

ORPHA code
157997
OMIM code
-
ICD10 code
D76.3
ICD11 code
EE81

No additional description.

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