POEMS SYNDROME/PLASMA CELL DYSCRASIA WITH POLYNEUROPATHY
By Gary M. White, MD
POEMS syndrome stands for polyneuropathy, organomegaly (e.g. lymphadenopathy, splenomegaly, hepatomegaly), endocrinopathy (e.g. hypogonadism which may result in impotence, glucose intolerance, hypothyroidism), M protein and skin changes).
- There is a male predominance and an average age of onset in the forties.
- A progressive sensorimotor, peripheral polyneuropathy occurs in up to 100% of patients.
- The differentiation between POEMS syndrome and osteosclerotic myeloma with peripheral neuropathy appears to have no value. It has been suggested that the term plasma cell dyscrasia with polyneuropathy be used. In contrast to multiple myeloma which is characterized by lytic bone lesions and bone marrow plasmacytosis with more that 10% plasma cells, PCD with P characteristically has osteosclerotic bone lesions and a bone marrow with less that 5% plasma cells.
- Various vascular lesions may occur, including microvenular hemangiomas, cherry hemangiomas, multinucleated cell angiohistiocytomas and glomeruloid hemangiomas, but glomeruloid hemangiomas seem to be specific for POEMS syndrome.
The skin changes in POEMS syndrome are most often nonspecific and include diffuse hyperpigmentation, hypertrichosis, induration, hyperhidrosis, Raynaud's phenomenon, and clubbing. The glomeruloid hemangioma is the most specific skin sign and occurs in about 1/3 of patients. Clinically, they appear as multiple polypoid angiomas. Facial lipoatrophy may occur in up to 50% of patients.
One patient's skin changes improved greatly after UVA1 phototherapy [JAAD 2001;45;969].
Hyperpigmentation. Washington University Case of the Month
Acquired facial lipoatrophy. Arch Dermatol. 2010 Jun;146(6):615-23.
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