POEMS syndrome with idiopathic flushing mimicking carcinoid syndrome.
POEMS syndrome, a rare multisystem disease, is a variant of osteosclerotic myeloma and is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins, and skin changes. Presented herein is a case of POEMS syndrome with flushing. The flushing was intermittent, involving the face and upper third of the trunk, and was associated with hypotension and bronchospasm. Final diagnosis was made by biopsy examination of an axillary lymph node, which showed angiofollicular hyperplasia that stained strongly and selectively for lambda light chains. The patient had most of the typical features of POEMS syndrome but was unique in that her most striking finding was carcinoid-like flushing. The flushing improved with steroid therapy, as did some of the other clinical features of her disease. This case suggests that idiopathic flushing can be added to the skin changes observed in POEMS syndrome.
Myers
B M
BM
Department of Gastroenterology, Mayo Clinic and Foundation, Rochester, Minnesota 55905.
Miralles
G D
GD
Taylor
C A
CA
Gastineau
D A
DA
Pisani
R J
RJ
Talley
N J
NJ
D001706:Biopsy; D005871:Castleman Disease / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D005483:Flushing / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D011115:Polyneuropathies / Q000150:complications / Q000175:diagnosis* / Q000473:pathology