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[Langerhans cell histiocytosis]
L Ben Slama1, B Ruhin, A Zoghbani
1Service de chirurgie maxillofaciale et stomatologie, hôpital Adulte de la Pitié-Salpêtrière, université Pierre-et-Marie-Curie Paris-6, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France. lbenslama@noos.fr
Abstract:
Langerhans cell histiocytosis (ex histiocytosis X) is usually present in children. It is a clonal proliferation of non-functional Langerhans's cells. Histological aspects are variable. The diagnosis is made in immunolabeling by anti-CD1a. Clinical presentations are variable, depending on their extension. Three syndromes are actually the same pathogenic process: eosinophilic granuloma (single or multiple osseous localizations), Hand-Schüller-Christian disease (chronic form with bone and visceral dissemination) and Abt-Letterer-Siwe disease (disseminated and acute malignant presentation).