Journal article
Histopathologic characteristics of scleromyxedema: A study of a series of 34 cases.
- Rongioletti F 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.
- Merlo G Section of Dermatology, San Martino University Hospital, Genoa, Italy.
- Carli C Institute of Pathology, San Martino University Hospital, Genoa, Italy.
- Cribier B Université Louis Pasteur, Hôpitaux Universitaires, Strasbourg, France.
- Metze D Department of Dermatology, University Hospital, Münster, Germany.
- Calonje E John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom.
- Kempf W Kempf and Pfaltz, Histological Diagnostics, and Dept. of Dermatology, University Hospital Zürich, Zürich, Switzerland.
- Stefanato CM John's Institute of Dermatology, St Thomas' Hospital, London, United Kingdom.
- Marinho E Service de Dermatopathologie, Paris, France.
- Kanitakis J Department of Dermatology, Ed. Herriot Hospital Group, Lyon, France.
- 2016-02-22
Published in:
- Journal of the American Academy of Dermatology. - 2016
fibromucinous disorder
histopathology
immunohistochemistry
interstitial granulomatous dermatitis
lichen myxedematosus
scleromyxedema
Adult
Aged
Aged, 80 and over
Antigens, CD
Antigens, CD20
Antigens, Differentiation, Myelomonocytic
CD3 Complex
CD4 Antigens
CD4-Positive T-Lymphocytes
CD8 Antigens
CD8-Positive T-Lymphocytes
Cytoprotection
Factor XIIIa
Female
Fibroblasts
Fibrosis
Histiocytes
Humans
Immunohistochemistry
Male
Middle Aged
Mucins
Retrospective Studies
Scleromyxedema
Skin
English
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.
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.
- Language
-
- English
- Open access status
- closed
- Identifiers
-
- DOI 10.1016/j.jaad.2015.12.021
- PMID 26897387
- Persistent URL
- https://sonar.ch/global/documents/35828
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