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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 1998 February;133(1):55-7

language: Italian

Familial multiple lipomatosis. A hereditary condition with its typical clinical diagnosis

Noto G., D'anna C., Brignola G.

Università degli Studi - Palermo, Clinica Dermatologica (Direttore: Prof. M. Aricò)


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A case of familial multiple lipomatosis (FML) is reported and differential diagnosis of multiple lipomatoses is discussed. A 32-year-old woman presented with a number of subcutaneous lipomas located on the forearms, trunk and abdomen, measuring from 1 to 6 cm in diameter. One of them, on the volar aspect of the arm, caused pain and paresthesias. Such as lesions increased in number during the past 10 years, sparing face, neck, scalp and lower limbs. Routine blood analysis were within the normal range. The father, one brother and one sister were also affected. A diagnosis of FML was made. This is a rare inherited disease, transmitted with autosomal dominant trait, starting during the third decade of life, with progressive appearance of multiple encapsulated lipomas involving forearms, trunk and thighs, usually sparing shoulders, head and neck. Differential diagnosis of FML includes Dercum’s disease and Multiple Symmetric Lipomatosis (MSL, Madelung’s disease), lipomas of Dercum’s disease, not inherited, are very painful. In MSL many coalescent, non-capsulated lipomas are localized around the neck and proximal upper extremities, with symmetric subcutaneous lipomatous deposits in a “horse collar” distribution, with very rare familial occurrence.

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