title: Histopathologic characteristics of scleromyxedema: A study of a series of 34 cases.
abstract: BACKGROUND
  Few histologic studies describe the histopathologic aspects of scleromyxedema.
  
  
  OBJECTIVE
  We sought to describe the histopathologic and immunohistochemical features of scleromyxedema in a large series of patients.
  
  
  METHODS
  We studied all the cases with scleromyxedema diagnosed between 2000 and 2014 at participating centers. Sections with hematoxylin-eosin and special stains were examined. Immunohistochemistry for CD3, CD4, CD8, CD20, CD68, and factor XIIIa was performed in 10 cases.
  
  
  RESULTS
  A total of 44 skin biopsy specimens from 34 patients were reviewed. Two different histopathologic patterns were observed: the classic microscopic triad (dermal mucin deposition, fibroblast proliferation, fibrosis) was identified in 34 specimens, whereas an interstitial granuloma annulare-like pattern was found in 10 specimens. A superficial perivascular infiltrate with T lymphocytes was found in all specimens whereas an interstitial proliferation of CD68(+) epithelioid cells was identified in the 10 specimens with an interstitial granuloma annulare-like pattern. Elastic fibers were largely lost, explaining the redundant folds of the disease.
  
  
  CONCLUSIONS
  This was a retrospective study.
  
  
  CONCLUSIONS
  Scleromyxedema shows 2 histopathologic patterns, including the classic type with the microscopic triad of mucin, fibroblast proliferation and fibrosis, and an interstitial granuloma annulare-like pattern. Recognition of these histologic presentations expands the spectrum of scleromyxedema and highlights the difficulty in diagnosing this disabling condition in the absence of a clinicopathological correlation.
authors.affiliation: Section of Dermatology, "Mario Aresu" Department of Medical Science, University Hospital of Cagliari, S.Giovanni di Dio, Cagliari, Italy; Institute of Pathology, San Martino University Hospital, Genoa, Italy; Section of Dermatology, San Martino University Hospital, Genoa, Italy. Electronic address: rongioletti@unica.it.
authors.forename: Franco
authors.initials: F
authors.lastname: Rongioletti
authors.affiliation: Section of Dermatology, San Martino University Hospital, Genoa, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: Merlo
authors.affiliation: Institute of Pathology, San Martino University Hospital, Genoa, Italy.
authors.forename: Carla
authors.initials: C
authors.lastname: Carli
authors.affiliation: Université Louis Pasteur, Hôpitaux Universitaires, Strasbourg, France.
authors.forename: Bernard
authors.initials: B
authors.lastname: Cribier
authors.affiliation: Department of Dermatology, University Hospital, Münster, Germany.
authors.forename: Dieter
authors.initials: D
authors.lastname: Metze
authors.affiliation: John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Calonje
authors.affiliation: Kempf and Pfaltz, Histological Diagnostics, and Dept. of Dermatology, University Hospital Zürich, Zürich, Switzerland.
authors.forename: Werner
authors.initials: W
authors.lastname: Kempf
authors.affiliation: John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom.
authors.forename: Catherine M
authors.initials: CM
authors.lastname: Stefanato
authors.affiliation: Service de Dermatopathologie, Paris, France.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Marinho
authors.affiliation: Department of Dermatology, Ed. Herriot Hospital Group, Lyon, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Kanitakis
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000032:analysis*; D018951:Antigens, CD20 / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D017252:CD3 Complex / Q000032:analysis; D015704:CD4 Antigens / Q000032:analysis; D015496:CD4-Positive T-Lymphocytes / Q000737:chemistry / Q000473:pathology; D016827:CD8 Antigens / Q000032:analysis; D018414:CD8-Positive T-Lymphocytes / Q000737:chemistry / Q000473:pathology; D019610:Cytoprotection; D026122:Factor XIIIa / Q000032:analysis*; D005260:Female; D005347:Fibroblasts / Q000473:pathology; D005355:Fibrosis; D006644:Histiocytes / Q000737:chemistry / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D009077:Mucins; D012189:Retrospective Studies; D053718:Scleromyxedema / Q000276:immunology / Q000473:pathology*; D012867:Skin / Q000737:chemistry / Q000473:pathology*; D000097382:CD68 Molecule
chemical_list: D015703:Antigens, CD; D018951:Antigens, CD20; D015214:Antigens, Differentiation, Myelomonocytic; D017252:CD3 Complex; D015704:CD4 Antigens; D016827:CD8 Antigens; D026122:Factor XIIIa; D009077:Mucins; C067980:CD68 antigen, human; D000097382:CD68 Molecule
keywords: fibromucinous disorder; histopathology; immunohistochemistry; interstitial granulomatous dermatitis; lichen myxedematosus; scleromyxedema