title: Scleromyxedema: clinical diagnosis and autopsy findings.
abstract: Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.
authors.affiliation: Universidade Estadual de Campinas (UNICAMP) - Campinas (SP), Brazil.
authors.forename: Ana Carolina Bulhões
authors.initials: AC
authors.lastname: Sala
authors.affiliation: Faculdade de Medicina de Jundiaí (FMJ) - Jundiaí (SP), Brazil.
authors.forename: Paulo Rowilson
authors.initials: PR
authors.lastname: Cunha
authors.affiliation: Faculdade de Medicina de Jundiaí (FMJ) - Jundiaí (SP), Brazil.
authors.forename: Clóvis Antônio Lopes
authors.initials: CA
authors.lastname: Pinto
authors.affiliation: Faculdade de Medicina de Jundiaí (FMJ) - Jundiaí (SP), Brazil.
authors.forename: Célia Antônia Xavier de Moraes
authors.initials: CA
authors.lastname: Alves
authors.affiliation: Private clinic - Vitória (ES), Brazil.
authors.forename: Ingrid Barreto
authors.initials: IB
authors.lastname: Paiva
authors.affiliation: Private clinic - São Luís (MA) - Brazil.
authors.forename: Ana Paula Vieira
authors.initials: AP
authors.lastname: Araujo
mesh_terms: D001706:Biopsy; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008875:Middle Aged; D009077:Mucins / Q000378:metabolism; D053718:Scleromyxedema / Q000473:pathology*; D012867:Skin / Q000473:pathology*
chemical_list: D009077:Mucins