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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...

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Autores principales: Radke, Josefine, Pehl, Debora, Aronica, Eleonora, Schonenberg-Meinema, Dieneke, Schneider, Udo, Heppner, Frank L., de Visser, Marianne, Goebel, Hans H., Stenzel, Werner
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2014
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.
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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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