{Reference Type}: Journal Article
{Title}: Histopathologic characteristics of scleromyxedema: A study of a series of 34 cases.
{Author}: Rongioletti F;Merlo G;Carli C;Cribier B;Metze D;Calonje E;Kempf W;Stefanato CM;Marinho E;Kanitakis J;
{Journal}: J Am Acad Dermatol
{Volume}: 74
{Issue}: 6
{Year}: Jun 2016
{Factor}: 15.487
{DOI}: 10.1016/j.jaad.2015.12.021
{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.