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. 2015 Apr-Jun;18(2):194-9.
doi: 10.4103/0972-2327.150603.

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

Sudhir Babu Karri  1 , Meena Anga Muthu Kannan  2 , Liza Rajashekhar  3 , Megha S Uppin  1 , Sundaram Challa  1
Affiliations

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

Sudhir Babu Karri et al. Ann Indian Acad Neurol. 2015 Apr-Jun.

Abstract

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.

Keywords: Dermatomyositis; perifascicular atrophy; perivascular inflammation.

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Conflict of interest statement

Conflict of Interest: None declared.

Figures

Figure 1
Figure 1
(a) Photomicrograph showing perifascicular atrophy (PFA) (H and E ×40) (b) PFA (ATPase pH 9.4 ×40)
Figure 2
Figure 2
(a) Photomicrograph showing perivascular/perimysial inflammation (H and E ×40), (b) Perivascular/perimysial inflammation (H and E ×100)
Figure 3
Figure 3
(a) Type 2 atrophy (ATPase pH 9.4 X100) (b) Fibrosis (Masson's Trichrome x100)
See this image and copyright information in PMC

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