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The lymphoid follicle variant of dermatomyositis
OBJECTIVE: To investigate the clinical and morphologic spectrum of early adult–onset dermatomyositis (DM), an inflammatory disease that affects small vessels of the muscle and the skin. METHODS: Histologic evaluation of frozen muscle samples was employed to visualize the cellular organization of ect...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Lippincott Williams & Wilkins
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4202675/ https://www.ncbi.nlm.nih.gov/pubmed/25340071 http://dx.doi.org/10.1212/NXI.0000000000000019 |
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author | Radke, Josefine Pehl, Debora Aronica, Eleonora Schonenberg-Meinema, Dieneke Schneider, Udo Heppner, Frank L. de Visser, Marianne Goebel, Hans H. Stenzel, Werner |
author_facet | Radke, Josefine Pehl, Debora Aronica, Eleonora Schonenberg-Meinema, Dieneke Schneider, Udo Heppner, Frank L. de Visser, Marianne Goebel, Hans H. Stenzel, Werner |
author_sort | Radke, Josefine |
collection | PubMed |
description | OBJECTIVE: To investigate the clinical and morphologic spectrum of early adult–onset dermatomyositis (DM), an inflammatory disease that affects small vessels of the muscle and the skin. METHODS: Histologic evaluation of frozen muscle samples was employed to visualize the cellular organization of ectopic lymphoid structures in muscle biopsies obtained from 2 patients diagnosed with DM. Clinical presentation and morphologic features, as well as treatment and follow-up, were assessed and documented. Electron microscopy was used to confirm the light microscopic diagnosis of DM. Clonality analysis of B-cell populations using PCR was performed. RESULTS: Muscle biopsy of both patients fulfilled the morphologic European Neuromuscular Centre criteria of DM. Analyses of muscle biopsy samples revealed ectopic lymphoid follicle-like structures that showed a remarkable similarity to secondary lymphoid organs (SLOs) with distinct T- and B-cell compartmentalization. Our 2 patients exhibited an atypical and mild clinical presentation and responded favorably to therapy. CONCLUSIONS: The clinical and histopathologic features of DM can be atypical, and the presence of SLOs is not inevitably linked to an unfavorable prognosis. |
format | Online Article Text |
id | pubmed-4202675 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
spelling | pubmed-42026752014-10-22 The lymphoid follicle variant of dermatomyositis Radke, Josefine Pehl, Debora Aronica, Eleonora Schonenberg-Meinema, Dieneke Schneider, Udo Heppner, Frank L. de Visser, Marianne Goebel, Hans H. Stenzel, Werner Neurol Neuroimmunol Neuroinflamm Article OBJECTIVE: To investigate the clinical and morphologic spectrum of early adult–onset dermatomyositis (DM), an inflammatory disease that affects small vessels of the muscle and the skin. METHODS: Histologic evaluation of frozen muscle samples was employed to visualize the cellular organization of ectopic lymphoid structures in muscle biopsies obtained from 2 patients diagnosed with DM. Clinical presentation and morphologic features, as well as treatment and follow-up, were assessed and documented. Electron microscopy was used to confirm the light microscopic diagnosis of DM. Clonality analysis of B-cell populations using PCR was performed. RESULTS: Muscle biopsy of both patients fulfilled the morphologic European Neuromuscular Centre criteria of DM. Analyses of muscle biopsy samples revealed ectopic lymphoid follicle-like structures that showed a remarkable similarity to secondary lymphoid organs (SLOs) with distinct T- and B-cell compartmentalization. Our 2 patients exhibited an atypical and mild clinical presentation and responded favorably to therapy. CONCLUSIONS: The clinical and histopathologic features of DM can be atypical, and the presence of SLOs is not inevitably linked to an unfavorable prognosis. Lippincott Williams & Wilkins 2014-07-28 /pmc/articles/PMC4202675/ /pubmed/25340071 http://dx.doi.org/10.1212/NXI.0000000000000019 Text en © 2014 American Academy of Neurology This is an open access article distributed under the terms of the Creative Commons Attribution-Noncommercial No Derivative 3.0 License, which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially. |
spellingShingle | Article Radke, Josefine Pehl, Debora Aronica, Eleonora Schonenberg-Meinema, Dieneke Schneider, Udo Heppner, Frank L. de Visser, Marianne Goebel, Hans H. Stenzel, Werner The lymphoid follicle variant of dermatomyositis |
title | The lymphoid follicle variant of dermatomyositis |
title_full | The lymphoid follicle variant of dermatomyositis |
title_fullStr | The lymphoid follicle variant of dermatomyositis |
title_full_unstemmed | The lymphoid follicle variant of dermatomyositis |
title_short | The lymphoid follicle variant of dermatomyositis |
title_sort | lymphoid follicle variant of dermatomyositis |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4202675/ https://www.ncbi.nlm.nih.gov/pubmed/25340071 http://dx.doi.org/10.1212/NXI.0000000000000019 |
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