CASE REPORTS   

Giornale Italiano di Dermatologia e Venereologia 2006 December;141(6):533-6

Copyright © 2006 EDIZIONI MINERVA MEDICA

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

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.