POEMS syndrome: Cicatricial alopecia as an unusual cutaneous manifestationassociated with an underlying plasmacytoma

POEMS syndrome is a rare condition with cutaneous manifestations commonly i ncluding angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphad enopathy, erythematous thickening of The skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring a lopecia and sparse follicular pustules at: the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monocl onal gammopathy, subclinical thyreopathy, and an osteolytic bone lesion of the skull. Histologically, a plasmacytoma with lambda cell restriction was found. The overlying skin showed marked fibrosis, with loss of hair follicl es, and a plasma cell infiltrate of polyclonal origin. The cervical lymph n odes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There wa s no evidence of further plamacytomas. After excision of the plasmacytoma a nd postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further follic ulitis or hair loss on the rest of the scalp. This case illustrates the rea ctive character of POEMS syndrome as a paraneoplastic syndrome in myeloma p atients.