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POEMS syndrome revealed by multiple glomeruloid angiomas
Christel Scheers1, Athanassios Kolivras, Alexandra Corbisier
1Department of Dermatology, CHU Saint-Pierre and CHU Brugmann, Brussels, Belgium. christel_email@yahoo.com
Summary
POEMS syndrome, a rare multisystemic disorder, was diagnosed in a woman presenting with neuropathy and organomegaly. Glomeruloid angioma confirmed the diagnosis, highlighting its role in identifying this plasma cell disorder.
Area of Science:
- Hematology
- Neurology
- Dermatology
Background:
- POEMS syndrome is a rare multisystemic disorder linked to plasma cell dyscrasia.
- It presents with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.
Observation:
- A 68-year-old woman with hypertension and renal insufficiency presented with confusion, somnolence, asthenia, ascites, hepatosplenomegaly, edema, dysesthesias, leuconychia, and angiomas.
- Laboratory tests revealed hypothyroidism and a monoclonal IgG peak.
- Electromyography indicated a mixed demyelinating and axonal neuropathy.
Findings:
- The patient exhibited multiple clinical features consistent with POEMS syndrome.
- Dermatopathological examination of a cutaneous angioma revealed glomeruloid angioma, a specific diagnostic marker for POEMS syndrome.
Implications:
- This case underscores the importance of recognizing the diverse clinical manifestations of POEMS syndrome.
- Identifying specific markers like glomeruloid angioma is crucial for accurate diagnosis and management of this rare plasma cell disorder.