title: POEMS in childhood.
abstract: A 17-year-old boy had a 3-year history of diabetes mellitus, malabsorption syndrome, and skin changes consisting of induration, hyperpigmentation, and hypertrichosis on the anterior aspect of both thighs, lower abdomen, and scrotum. Physical examination found hypogonadism, hepatomegaly, gynecomastia, growth retardation, and ankle edema. There was no neuropathy or plasma cell dyscrasia. However, the characteristic skin changes and the combination of symptoms suggest polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes syndrome. This is a rare multisystemic disorder of obscure pathogenesis and no conspicuous heredity. Overproduction of vascular endothelial growth factor is thought to cause microangiopathy, neovascularization, and accelerated vasopermeability causing the multiorgan deterioration. Cyclophosphamid cytostatic therapy seems beneficial.
authors.affiliation: Department of Dermatology and Venereology, Faculty of Medicine, Medical University, Sofia, Bulgaria.
authors.forename: Sonya
authors.initials: S
authors.lastname: Marina
authors.forename: Valentina
authors.initials: V
authors.lastname: Broshtilova
mesh_terms: D000293:Adolescent; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D016878:POEMS Syndrome / Q000175:diagnosis* / Q000188:drug therapy
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide