%0 Journal Article
%T Histopathologic characteristics of scleromyxedema: A study of a series of 34 cases.
%A Rongioletti F
%A Merlo G
%A Carli C
%A Cribier B
%A Metze D
%A Calonje E
%A Kempf W
%A Stefanato CM
%A Marinho E
%A Kanitakis J
%J J Am Acad Dermatol
%V 74
%N 6
%D Jun 2016
%M 26897387
%F 15.487
%R 10.1016/j.jaad.2015.12.021
%X <B>BACKGROUND: </B>Few histologic studies describe the histopathologic aspects of scleromyxedema.<BR><B>OBJECTIVE: </B>We sought to describe the histopathologic and immunohistochemical features of scleromyxedema in a large series of patients.<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>This was a retrospective study.<BR><B>CONCLUSIONS: </B>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.