POEMS Syndrome Revealed by Multiple Glomeruloid Angiomas

Abstract

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin disorders) is a rare multisystemic disease associated with plasma cell dyscrasia. A 68-year-old woman with chronic renal insufficiency and arterial hypertension included in her medical history was admitted to the hospital with confusion, somnolence and asthenia. She presented ascites, hepatosplenomegaly, leg oedema, distal dysesthesias, leuconychia and multiple nodular purple red angiomas on the trunk, upper limbs and fingers. Hypothyroidism was revealed in the laboratory investigations and monoclonal IgG peak in immunoelectrophoresis. Electromyography showed both demyelinisating and axonal degenerative neuropathy. The diagnosis of POEMS syndrome was based on the dermatopathological examination of a cutaneous angioma; histology revealed features of glomeruloid angioma, a specific marker of this syndrome.

Papillary haemangioma. A distinctive cutaneous haemangioma of the head and neck area containing eosinophilic hyaline globules

“…Glomeruloid haemangioma was first described by Chan et al. in two patients in 1990, 10 since when only single case reports have been published, 17–25 which indicates that glomeruloid haemangioma is a rare vascular lesion. It is strongly associated with, and considered specific for, POEMS syndrome and is often found in association with multicentric Castleman’s disease.…”

Glomeruloid hemangioma leading to a diagnosis of POEMS syndrome

“…Because glomeruloid hemangiomas may occur early in this disease, discovery of such lesions warrants careful evaluation and follow-up of these patients. 5,7,24 …”

Skin Manifestations and Vascular Endothelial Growth Factor Levels in POEMS Syndrome

“…On histopathological examination, the suggestive glomeruloid pattern was exclusively observed in biopsy specimens originating from 30% of tuberous hemangiomas. [31][32][33][34] The cherrylike angiomas showed only vascular hyperplasia, which was clinically evident. Biopsies should therefore be performed only on tuberous hemangiomas.…”