POEMS syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma.
POEMS syndrome is a rare condition with cutaneous manifestations commonly including angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphadenopathy, erythematous thickening of the skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring alopecia and sparse follicular pustules at the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monoclonal 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 follicles, and a plasma cell infiltrate of polyclonal origin. The cervical lymph nodes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There was no evidence of further plamacytomas. After excision of the plasmacytoma and postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further folliculitis or hair loss on the rest of the scalp. This case illustrates the reactive character of POEMS syndrome as a paraneoplastic syndrome in myeloma patients.
D000328:Adult; D000505:Alopecia / Q000209:etiology / Q000473:pathology*; D005871:Castleman Disease / Q000209:etiology / Q000473:pathology; D002921:Cicatrix / Q000209:etiology / Q000473:pathology*; D006201:Hair Diseases / Q000473:pathology; D006801:Humans; D008297:Male; D009069:Movement Disorders / Q000209:etiology; D016878:POEMS Syndrome / Q000209:etiology / Q000473:pathology*; D010257:Paraneoplastic Syndromes / Q000209:etiology / Q000473:pathology*; D010954:Plasmacytoma / Q000150:complications / Q000473:pathology*; D012535:Scalp / Q000473:pathology*; D012878:Skin Neoplasms / Q000150:complications / Q000473:pathology*; D016138:Walking / Q000502:physiology