Congenital Solitary Histiocytoma: A Variant of Hashimoto-Pritzker Histiocytosis

Autor: C. Eschard, Anne Durlach, Philippe Bernard, C. Zunino-Goutorbe
Rok vydání: 2008
Předmět:
Zdroj: Dermatology. 216:118-124
ISSN: 1421-9832
1018-8665
DOI: 10.1159/000111508
Popis: Background: Self-healing solitary-lesion Hashimoto-Pritzker histiocytosis (HPH), a rare, congenital, purely cutaneous Langerhans histiocytosis (only 30 cases reported), carries a good prognosis. Objective: To describe the clinical and histopathological characteristics of solitary HPH. Methods: To conduct a retrospective, observational study on 8 affected newborns. Results: For these infants, with otherwise normal physical examinations, the unique nodule or papule (5–15-mm diameter) was congenital. Systematic routine histological examination of the lesions found dermal infiltrates constituted predominantly of histiocytes with lymphocytes and eosinophils. Protein S100 and CD1a immunolabelings, done for 7 patients, were positive. Electron microscopy (n = 4) observed Birbeck granules. No visceral involvement or recurrence has ever been observed after 2–12 years of follow-up. Conclusions: Because of its self-healing nature, congenital solitary HPH frequency has probably been underestimated. In the absence of systemic involvement, regular physical examination for at least 2 years seems a valid approach.
Databáze: OpenAIRE