Доброякісний гістіоцитоз головного мозку
ORPHA:157997· ICD-10 D76.3· Benign cephalic histiocytosis
Визначення(English summary)
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.
- Поширеність
- <1 / 1 000 000
- Успадкування
- Not applicable
- Вік початку
- Infancy