title: Scleromyxedema.
abstract: Scleromyxedema is a rare cutaneous mucinous disease characterized by a generalized papular sclerodermoid eruption and systemic manifestations that can lead to significant morbidity and mortality. Although its etiology remains unknown, most theories focus on a pathogenic role by paraproteins; it must be noted, however, that nonparaprotein factors have been suggested to cause fibroblast proliferation and increased mucin production. Several treatment modalities including melphalan, cyclophosphamide, interferon alfa, and plasmapheresis have been suggested; however, further research is needed to prove treatment efficacy.
authors.affiliation: Division of Dermatology, Cooper University Hospital, Camden, NJ 08103, USA. doctorbeba@hotmail.com
authors.forename: Clara-Dina
authors.initials: CD
authors.lastname: Cokonis Georgakis
authors.forename: Gerald
authors.initials: G
authors.lastname: Falasca
authors.forename: Alexander
authors.initials: A
authors.lastname: Georgakis
authors.forename: Warren R
authors.initials: WR
authors.lastname: Heymann
mesh_terms: D018450:Disease Progression; D006801:Humans; D053718:Scleromyxedema / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology*; D012867:Skin / Q000276:immunology / Q000473:pathology*