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Clinico pathological study of adult dermatomyositis: Importance of muscle histology in the diagnosis

AIMS: To study the histological features on muscle biopsy and correlate them with clinical features, other laboratory data in adult patients to make a diagnosis of dermatomyositis (DM), applying the European Neuromuscular center (ENMC) criteria. MATERIALS AND METHODS: Adult patients who fulfilled cl...

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Autores principales: Karri, Sudhir Babu, Kannan, Meena Anga Muthu, Rajashekhar, Liza, Uppin, Megha S., Challa, Sundaram
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medknow Publications & Media Pvt Ltd 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4445196/
https://www.ncbi.nlm.nih.gov/pubmed/26019418
http://dx.doi.org/10.4103/0972-2327.150603
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author Karri, Sudhir Babu
Kannan, Meena Anga Muthu
Rajashekhar, Liza
Uppin, Megha S.
Challa, Sundaram
author_facet Karri, Sudhir Babu
Kannan, Meena Anga Muthu
Rajashekhar, Liza
Uppin, Megha S.
Challa, Sundaram
author_sort Karri, Sudhir Babu
collection PubMed
description AIMS: To study the histological features on muscle biopsy and correlate them with clinical features, other laboratory data in adult patients to make a diagnosis of dermatomyositis (DM), applying the European Neuromuscular center (ENMC) criteria. MATERIALS AND METHODS: Adult patients who fulfilled clinical, laboratory, and muscle biopsy findings according to ENMC criteria for DM during the period 2010–2013 were included in the study. Cryostat sections of muscle biopsy were reviewed with emphasis on Perifascicular atrophy (PFA), perivascular/endomysial inflammation. Muscular dystrophies and metabolic myopathies were excluded by appropriate immunohistochemistry and special stains. RESULTS: The diagnosis of adult DM was made in 45 patients out of 170 clinically suspected idiopathic inflammatory myopathies. These included 33 definite, 4 probable, 7 possible sine dermatitis, and 1 amyopathic DM. All patients with definite DM had typical rash and proximal muscle weakness and muscle biopsy showed PFA with or without inflammation. Thirteen patients had quadriparesis, neck muscle weakness, dysphagia/dysphonia at presentation. Patients with probable DM had rash and showed perivascular/endomysial inflammation with no PFA. Possible DM sine dermatitis showed PFA with perivascular/endomysial infiltrates. One patient of amyopathic DM had typical heliotrope rash and characteristic skin biopsy. CONCLUSIONS: Histological features are important for the diagnosis of DM. Relying on PFA for diagnosis of definite DM underestimates the true frequency of DM.
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spelling pubmed-44451962015-05-27 Clinico pathological study of adult dermatomyositis: Importance of muscle histology in the diagnosis Karri, Sudhir Babu Kannan, Meena Anga Muthu Rajashekhar, Liza Uppin, Megha S. Challa, Sundaram Ann Indian Acad Neurol Original Article AIMS: To study the histological features on muscle biopsy and correlate them with clinical features, other laboratory data in adult patients to make a diagnosis of dermatomyositis (DM), applying the European Neuromuscular center (ENMC) criteria. MATERIALS AND METHODS: Adult patients who fulfilled clinical, laboratory, and muscle biopsy findings according to ENMC criteria for DM during the period 2010–2013 were included in the study. Cryostat sections of muscle biopsy were reviewed with emphasis on Perifascicular atrophy (PFA), perivascular/endomysial inflammation. Muscular dystrophies and metabolic myopathies were excluded by appropriate immunohistochemistry and special stains. RESULTS: The diagnosis of adult DM was made in 45 patients out of 170 clinically suspected idiopathic inflammatory myopathies. These included 33 definite, 4 probable, 7 possible sine dermatitis, and 1 amyopathic DM. All patients with definite DM had typical rash and proximal muscle weakness and muscle biopsy showed PFA with or without inflammation. Thirteen patients had quadriparesis, neck muscle weakness, dysphagia/dysphonia at presentation. Patients with probable DM had rash and showed perivascular/endomysial inflammation with no PFA. Possible DM sine dermatitis showed PFA with perivascular/endomysial infiltrates. One patient of amyopathic DM had typical heliotrope rash and characteristic skin biopsy. CONCLUSIONS: Histological features are important for the diagnosis of DM. Relying on PFA for diagnosis of definite DM underestimates the true frequency of DM. Medknow Publications & Media Pvt Ltd 2015 /pmc/articles/PMC4445196/ /pubmed/26019418 http://dx.doi.org/10.4103/0972-2327.150603 Text en Copyright: © Annals of Indian Academy of Neurology http://creativecommons.org/licenses/by-nc-sa/3.0 This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Article
Karri, Sudhir Babu
Kannan, Meena Anga Muthu
Rajashekhar, Liza
Uppin, Megha S.
Challa, Sundaram
Clinico pathological study of adult dermatomyositis: Importance of muscle histology in the diagnosis
title Clinico pathological study of adult dermatomyositis: Importance of muscle histology in the diagnosis
title_full Clinico pathological study of adult dermatomyositis: Importance of muscle histology in the diagnosis
title_fullStr Clinico pathological study of adult dermatomyositis: Importance of muscle histology in the diagnosis
title_full_unstemmed Clinico pathological study of adult dermatomyositis: Importance of muscle histology in the diagnosis
title_short Clinico pathological study of adult dermatomyositis: Importance of muscle histology in the diagnosis
title_sort clinico pathological study of adult dermatomyositis: importance of muscle histology in the diagnosis
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4445196/
https://www.ncbi.nlm.nih.gov/pubmed/26019418
http://dx.doi.org/10.4103/0972-2327.150603
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