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Presse Medicale 2006-Mar

Multiple cutaneous angiomas and Poems syndrome.

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Brigitte Granel
Jacques Serratrice
Christine de Roux-Serratrice
Nicoleta Ene
Patrick Disdier
Pierre-Jean Weiller

Keywords

Abstract

BACKGROUND

Poems syndrome is characterized by polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin lesions.

METHODS

We describe here a case in which the eruption of diffuse cutaneous angiomas in a woman with a history of bone plasmocytoma and progressive polyneuropathy helped physicians to diagnose Poems syndrome.

CONCLUSIONS

Other manifestations of Poems syndrome in this patient included endocrine dysfunction (hypothyroidism, adrenal insufficiency, and hypogonadism), sclerotic bone lesions of the femoral shaft, ribs, and vertebral body, monoclonal gammopathy, and anasarca. Steroid treatment led to dramatic improvement of polyserositis and a generally good outcome, despite persistence of the cutaneous lesions. This case points out this unusual cutaneous manifestation associated with Poems syndrome.

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