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Benign cephalic histiocytosis

Disease 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.

ORPHA:157997

Classification level: Disorder
  • Synonym(s): -
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: -
  • ICD-10: D76.3
  • OMIM: -
  • UMLS: C0347403
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information

Article for general public

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