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).

Clinical

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.

Treatment

One patient's skin changes improved greatly after UVA1 phototherapy [JAAD 2001;45;969].

References

Hyperpigmentation. Washington University Case of the Month

Acquired facial lipoatrophy. Arch Dermatol. 2010 Jun;146(6):615-23.

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