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GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA

A Journal on Dermatology and Sexually Transmitted Diseases


Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
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Giornale Italiano di Dermatologia e Venereologia 2006 December;141(6):533-6

language: English

Xanthoma disseminatum with hypothalamus-pituitary infiltration and partial hypopituitarism

Cannavò S. P. 1, Curtò L. 1, Guarneri C. 1, Torre M. L. 2, Marini F. 2, Cannavò S. 2

1 Unit of Dermatology Department of Territorial Social Medicine University of Messina, Messina, Italy
2 Unit of Endocrinology Department of Medicine and Pharmacology University of Messina, Messina, Italy


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Xanthoma disseminatum (XD) is a rare benign form of non-Langherhans cell histiocytosis and represents a variant of normolipemic mucocutaneous xanthomatosis of unknown etiology. It is mainly reported in young to middle-aged men with a male/female ratio of approximately 2:1 and is characterized by widespread papular eruptions with preference for main body flexure and, in about 40% of cases, for mucous membranes of the mouth and the upper airways. Diabetes insipidus, attributed to xanthomatoid cells infiltration of the hypothalamic-pituitary region, develops in about 40-50% of the patients and can represent the first symptom of disease. The coexistence of anterior pituitary deficiencies with XD in adults has been described only in other 2 cases. We present a patient in whom diabetes insipidus and multiple hypopituitarism preceded the appearance of the hypothalamic infiltration as well as of the brown to red-yellowish papulonodular lesions. Histological, immunohistochemical and ultrastructural features led to the diagnosis of XD.

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