3 Iconography
Access to the text (HTML) Access to the text (HTML)
PDF Access to the PDF text

Access to the full text of this article requires a subscription.
  • If you are a subscriber, please sign in 'My Account' at the top right of the screen.

  • If you want to subscribe to this journal, see our rates

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

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

Few histologic studies describe the histopathologic aspects of scleromyxedema.


We sought to describe the histopathologic and immunohistochemical features of scleromyxedema in a large series of patients.


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.


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.


This was a retrospective study.


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.

© 2015  American Academy of Dermatology, Inc.@@#104156@@
EM-CONSULTE.COM is registrered at the CNIL, déclaration n° 1286925.
As per the Law relating to information storage and personal integrity, you have the right to oppose (art 26 of that law), access (art 34 of that law) and rectify (art 36 of that law) your personal data. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.
Personal information regarding our website's visitors, including their identity, is confidential.
The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.
Article Outline
You can move this window by clicking on the headline