QJM: An International Journal of Medicine, 2018, 195 doi: 10.1093/qjmed/hcx207 Advance Access Publication Date: 27 October 2017 Clinical picture CLINICAL PICTURE A 68-year-old man presented to our clinic with a diffuse, pruritic Interstitial pulmonary fibrosis represents a major cause of mor- and burning rash that has been progressing over the past 4 weeks. tality among individuals with non-paraneoplastic DM. Medical history was positive for arterial hypertension only. The Generally, high clinical response rates can be achieved with patient also reported persistent fatigue. Physical examination systemic glucocorticoids. Patients with severe DM may be showed a generalized violaceous erythema with scattered erosions treated successfully with other immunosuppressive and immu- (Figure 1a) and dusky red papules over the extensor aspects of nomodulatory therapies such as methotrexate, cyclosporine, metacarpophalangeal joints (Figure 1b). The clinical diagnosis of cyclophosphamide, rituximab or intravenous immunoglobulin. dermatomyositis (DM) was confirmed by a skin biopsy. Computed Our case report underscores the importance of screening for tomography of the thorax revealed a tumour mass in the right malignancy in adult-onset DM and aims to increase the clinical upper pulmonary lobe, whereas bronchoalveolar lavage cytology awareness of this disorder as a paraneoplastic phenomenon. showed malignant cells with features of adenocarcinoma. Photographs and text from: J.M. Placke, M. Moelleken, K.A. Salva The DM is a rare inflammatory disease with a bimodal age- and J. Dissemond, Department of Dermatology, Venerology and at-onset pattern peaking at 10–15 years and 45–55 years. Allergology, University Hospital Essen, Hufelandstrasse 55, Although the pathophysiology of this entity remains poorly 45122 Essen, Germany. email: email@example.com understood, DM is recognized as a multisystem autoimmune disorder triggered by drugs or infectious agents. Furthermore, the occurrence of DM in adults as a manifestation of underlying malignancy is well documented. Characteristic cutaneous fea- Conflict of interest: None declared. tures include erythematous to violaceous papules over the extensor surfaces of metacarpophalangeal and/or interphalan- References geal joints known as Gottron’s papules, a violaceous periorbital erythema often accompanied by oedema, referred to as helio- 1.Aussy A, Boyer O, Cordel N. Dermatomyositis and immune- mediated necrotizing myopathies: a window on autoimmun- trope rash, and a diffuse violaceous erythema. The most com- mon systemic symptom is proximal muscle weakness, which ity and cancer. Front Immunol 2017; 8:992. affects the ability to perform daily living activities such as comb- 2.Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. ing hair or climbing stairs. Especially patients with juvenile DM Lancet 2003; 362:971–82. frequently develop calcinosis cutis primarily at sites of repeated 3.Dalakas MC. Pathogenesis and therapies of immune-mediated trauma, e.g. elbows and knees. Furthermore, overlap syndromes myopathies. Autoimmun Rev 2012; 11:203–6. with features of DM and lupus erythematodes, systemic sclerosis 4.Zahr ZA, Baer AN. Malignancy in myositis. Curr Rheumatol Rep and other connective tissue diseases have been described. 2011; 13:208–15. Figure 1. Cutaneous manifestations of DM: diffuse violaceous erythema (a) and Gottron’s papules (b). V C The Author 2017. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For Permissions, please email: firstname.lastname@example.org Downloaded from https://academic.oup.com/qjmed/article-abstract/111/3/195/4568569 by Ed 'DeepDyve' Gillespie user on 16 March 2018
QJM: An International Journal of Medicine – Oxford University Press
Published: Mar 1, 2018
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