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Anais Brasileiros de Dermatologia

Print version ISSN 0365-0596

Abstract

JEUNON, Thiago et al. Glomeruloid hemangioma in POEMS syndrome: a report on two cases and a review of the literature. An. Bras. Dermatol. [online]. 2011, vol.86, n.6, pp. 1167-1173. ISSN 0365-0596.  http://dx.doi.org/10.1590/S0365-05962011000600016.

Glomeruloid hemangioma is characterized by coiled capillary vessels contained within enlarged vascular spaces displaying an architecture that resembles renal glomeruli. The condition is strongly associated with POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes). The present paper reports on two cases of glomeruloid hemangiomas associated with POEMS syndrome, and includes a review of the literature. Case one refers to a 63-year old female patient admitted to hospital with ascites, hepatosplenomegaly, walking difficulties and cutaneous hemangiomas. Histopathology revealed a diagnosis of glomeruloid hemangioma and served to guide the clinical work-up, which revealed sensorimotor polyneuropathy, a plasmacytoma in the L4 vertebra with tumor cells positive for kappa chain, and diabetes mellitus. These findings permitted a diagnosis of POEMS syndrome to be reached. The second case consisted of a 39-year old woman admitted to hospital with edema, ascites, pleural effusion, glomeruloid hemangiomas and lymphadenopathy (Castleman's disease). Additional findings included monoclonal IgG-lambda paraproteinemia, blastic lesions in the right iliac bone and L4 vertebra, and demyelinating sensorimotor neuropathy affecting all four limbs. The final diagnosis in this case was POEMS syndrome associated with Castleman's disease.

Keywords : Dermatology; Giant lymph node hyperplasia; Hemangioma; POEMS syndrome; Pathology.

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