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Multisystem Langerhans? cell histiocytosis (Hand-Sch�ller-Christian disease) in an adult: a case report and review of the literature

2003; Springer Science+Business Media; Volume: 261; Issue: 6 Linguagem: Inglês

10.1007/s00405-003-0690-z

1434-4726

Paolo Scolozzi, Tommaso Lombardi, P Monnier, Bertrand Jaques,

Genital Health and Disease

Langerhans’ cell histiocytosis (LCH) is a rare and enigmatic clonal disorder that affects mainly children. It is characterized by single or multiple granulomatous mass lesions composed of cells with the Langerhans’ cell phenotype. Clinical presentation and behavior are heterogeneous and can range from a solitary lytic bone lesion (i.e., eosinophilic granuloma) with a favorable course to a fatal disseminated leukaemia-like form, with a wide spectrum of intermediate clinical presentations between these two extremes. Although LCH typically involves the bone, lesions can be found in almost all organs. We are reporting the case of a multisystem LCH in a 47-year-old patient who presented with a panhypopituitarism and diabetes insipidus, and who, 5 years later, developed mandibular, mastoid and femoral lesions. The final diagnosis of LCH was made on mandibular biopsy.