Plasma cell dyscrasia with polyneuropathy--POEMS syndrome presenting with vasculitic skin lesions and responding to combination chemotherapy.
We report a 61-year-old male patient who presented with severe sensorimotor neuropathy, leg edema and skin lesions with M-paraprotein and 50% plasma cells in the bone marrow. The POEMS (Crow-Fukase) syndrome was diagnosed and the skin lesions were compatible with vasculitis according to the histopathology. The patient was treated with aggressive combined chemotherapy, which induced improvement in both the clinical and laboratory parameters of his disease. To the best of our knowledge this is the first report of a vasculitic process underlying the skin changes in the POEMS syndrome. Our findings may shed light on the unknown pathogenesis of this syndrome and the successful results of treatment support the adoption of an aggressive therapeutic approach in symptomatic patients.
Sharabi
Y
Y
Department of Internal Medicine D, The Chaim Sheba Medical Center Tel Hashomer and Faculty of Medicine, Tel Aviv University, Israel.
Raanani
P
P
Shenkar
A
A
Thaler
M
M
Grossman
E
E
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology; D010265:Paraproteinemias / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D011115:Polyneuropathies / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D017445:Skin Diseases, Vascular / Q000175:diagnosis* / Q000473:pathology; D014657:Vasculitis / Q000209:etiology / Q000473:pathology