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CUTANEOUS MANIFESTATIONS IN POEMS SYNDROME: CASE REPORT AND REVIEW
Marinho, Flauberto Sousa et al. | Date Issued: 2015
Author
Marinho, Flauberto Sousa
Pirmez, Rodrigo
Nogueira, Renata
Cuzzi, Tullia
Sodré, Celso Tavares
Silva, Marcia
Affilliation
Federal University of Rio de Janeiro. University Hospital. Sector of Dermatology. Rio de Janeiro, RJ, Brazil / Federal University of Rio de Janeiro. School of Medicine. Post-Graduation Course. Rio de Janeiro, RJ, Brazil.
Federal University of Rio de Janeiro. University Hospital. Sector of Dermatology. Rio de Janeiro, RJ, Brazil / Federal University of Rio de Janeiro. School of Medicine. Post-Graduation Course. Rio de Janeiro, RJ, Brazil.
Federal University of Rio de Janeiro. University Hospital. Sector of Neurology. Rio de Janeiro, RJ, Brazil / Federal University of Rio de Janeiro. School of Medicine. Post-Graduation Course. Rio de Janeiro, RJ, Brazil.
Federal University of Rio de Janeiro. University Hospital. Sector of Pathology. Rio de Janeiro, RJ, Brazil / Federal University of Rio de Janeiro. School of Medicine. Post-Graduation Course. Rio de Janeiro, RJ, Brazil.
Federal University of Rio de Janeiro. University Hospital. Sector of Dermatology. Rio de Janeiro, RJ, Brazil / Federal University of Rio de Janeiro. School of Medicine. Post-Graduation Course. Rio de Janeiro, RJ, Brazil.
Federal University of Rio de Janeiro. University Hospital. Sector of Dermatology. Rio de Janeiro, RJ, Brazil / Federal University of Rio de Janeiro. School of Medicine. Post-Graduation Course. Rio de Janeiro, RJ, Brazil.
Abstract
The authors report a case of sensorimotor polyneuropathy, diffuse cutaneous hyperpigmentation, skin sclerodermiform thickening and papular lesions in the infraclavicular and abdominal region. Besides weight loss, there were diabetes mellitus and hypothyroidism. The alterations were consistent with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes) syndrome, which is a rare systemic disease with monoclonal proliferation of plasmacytes and slow progression. Cutaneous alterations are present in 68% of patients with diffuse cutaneous hyperpigmentation, plethora and acrocyanosis. Leukonychia, necrotizing vasculitis, hypertrichosis and cutaneous thickening of sclerodermiform type are also cited. The onset of multiple cutaneous angiomas in this syndrome has been observed in 24–44% of patients.
Keywords
POEMS syndrome
Plasmocytoma
Polyneuropathy
Endocrinopathy
Monoclonal gammopathy
Skin changes
 
Publisher
Karger Publishers Open Access
Citation
MARINHO, Flauberto Sousa et al. Cutaneous manifestations in POEMS syndrome: case report and review. Case Reports in Dermatology, v. 7, p. 61-69, 2015.
DOI
10.1159/000381302
Notes
Tullia Cuzzi. Fundação Oswaldo Cruz. Instituto Nacional de Infectologia Evandro Chagas. Documento produzido em parceria ou por autor vinculado à Fiocruz, mas não consta a informação no documento.