[Multifocal Langerhans cell granulomatosis. Report of a clinical case].
Langerhans' cell granulomatosis is a rare disease whose incidence on adults is valued about one case out of 3.000.000. The multifocal form is rarer than the single eosinophilic granuloma of the bone. It mainly affects males during childhood and it is usually localized in the long bones, hands, feet, and in the skull. We present the case of a young woman affected by multifocal Langerhans cell granulomatosis with bones and skin involved. Unusual in the presented clinical case is the age in which the disease come out. There is the highest incidence of multifocal lesions during childhood, while the monofocal kind is more typical of the years of adult age. Bone localization in the sternum is also quite unusual. In fact it is impossible to find reports of it in recent literature. As the patient refused to undergo chemotherapy she was treated with radiant therapy as for as bone localization concerned and with mecloretamin for local administration on skin lesion. Eleven months after the diagnosis we have not noticed the appearance of new lesions.
Corti
F
F
USL 3--Versilia U.O. Medicina--Ospedale Viareggio-Camaiore, Lucca.
Valicenti
A
A
Bertolucci
D
D
Bruno
J
J
Gustinucci
R
R
D000293:Adolescent; D001847:Bone Diseases / Q000209:etiology*; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications*; D006801:Humans; D012871:Skin Diseases / Q000209:etiology*
Granulomatosi a cellule di Langerhans multifocale. Presentazione di un caso clinico.