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Journal of the American Academy of Dermatology
Volume 74, n° 6
pages 1194-1200 (juin 2016)
Doi : 10.1016/j.jaad.2015.12.021
accepted : 10 December 2015
Dermatopathology

Histopathologic characteristics of scleromyxedema: A study of a series of 34 cases
 

Franco Rongioletti, MD a, b, c, , Giulia Merlo, MD c, Carla Carli, MD b, Bernard Cribier, MD d, Dieter Metze, MD e, Eduardo Calonje, MD f, Werner Kempf, MD g, Catherine M. Stefanato, MD, FRCPat f, Eduardo Marinho, MD h, Jean Kanitakis, MD i
a Section of Dermatology, “Mario Aresu” Department of Medical Science, University Hospital of Cagliari, S.Giovanni di Dio, Cagliari, Italy 
b Institute of Pathology, San Martino University Hospital, Genoa, Italy 
c Section of Dermatology, San Martino University Hospital, Genoa, Italy 
d Université Louis Pasteur, Hôpitaux Universitaires, Strasbourg, France 
e Department of Dermatology, University Hospital, Münster, Germany 
f John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom 
g Kempf and Pfaltz, Histological Diagnostics, and Dept. of Dermatology, University Hospital Zürich, Zürich, Switzerland 
h Service de Dermatopathologie, Paris, France 
i Department of Dermatology, Ed. Herriot Hospital Group, Lyon, France 

Reprint requests: Franco Rongioletti, MD, Section of Dermatology, “Mario Aresu” Department of Medical Science, University Hospital of Cagliari, S.Giovanni di Dio, via dell'Ospedale 54, 09124 Cagliari, Italy.Section of Dermatology, “Mario Aresu” Department of Medical ScienceUniversity Hospital of CagliariS.Giovanni di Diovia dell'Ospedale 54Cagliari09124Italy
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.

Limitations

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.

The full text of this article is available in PDF format.

Key words : fibromucinous disorder, histopathology, immunohistochemistry, interstitial granulomatous dermatitis, lichen myxedematosus, scleromyxedema



 Funding sources: None.
 Conflicts of interest: None declared.



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