Dermatomyositis presenting with severe subcutaneous edema: Five additional cases and review of the literature

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Abstract

Objectives

Dermatomyositis (DM) constitutes a subset of idiopathic inflammatory myopathies clinically characterized by proximal muscle weakness and skin involvement. Some of the dermatologic manifestations are highly prevalent and characteristic, but others such as generalized or limb edema are truly rare. The aim of the present study was to describe five cases of edematous DM diagnosed at our institution and to perform a review of the literature, as well as identify clinical, laboratory, or pathological data associated with this manifestation.

Methods

We performed a retrospective clinical, laboratory, and pathological evaluation of five cases of this edematous presentation out of 86 DM cases diagnosed at our hospital from 2004 to 2013. Moreover, we undertook a medical literature search using inflammatory myopathy, dermatomyositis, and edema as key words, limited to articles published in MEDLINE, EMBASE, and LILACS database in English and Spanish from 1987 to 2013.

Results

A total of 19 patients were identified, five diagnosed at our hospital and 14 cases from the literature. Overall, the median time from disease onset to diagnosis was 2 months, and most of the patients (16/84 patients, 21%) required more aggressive therapy, including immunosuppressive agents and intravenous immunoglobulin (12/63 patients, 15%). Microinfarction was present 2.3 times more frequently in DM patients with edema compared with those without edema.

Conclusions

The presence of edema in DM is uncommon but seems to be a sign of severe disease, requiring early and aggressive treatment. Microischemia-producing microinfarction may play an important pathophysiological role and determine the degree of disease severity.

Introduction

Dermatomyositis (DM) is a systemic disease included within the group of idiopathic inflammatory myopathies (IIM) along with polymyositis (PM), sporadic inclusion body myositis (sIBM), and necrotizing autoimmune myopathy (MNA) [1]. It has an estimated incidence of about 8 cases/million population/year [2] and typically has a bimodal age distribution with juvenile and adult forms. Its association with cancer is well known, with an incidence 5–7 times greater than that of the general population [3].

The disease is believed to begin when putative antibodies directed against endothelial cells activate complement C3, which leads to the formation of the membranolytic attack complex (MAC), which is deposited on capillaries, inducing their necrosis and the consequent microischemia-producing microinfarction of muscle fibers [4]. This process is more exaggerated in the outer region of the muscle bundles, producing characteristic perifascicular atrophy. In clinical terms, DM is manifested with proximal muscle weakness accompanied or preceded by typical skin lesions, such as violaceous poikiloderma (hypopigmentation, hyperpigmentation, telangiectasias, and epidermal atrophy), on the back and the shoulders (shawl sign), on the anterior neck and the chest (V sign), around the eyes (heliotrope rash), the knuckles, the elbows and the knees (Gottron sign), and the face and the scalp. Patients may also present calcinosis, mechanic׳s hands, Gottron papules, and gastrointestinal and pulmonary involvement [5].

Generalized and/or limb edema in IIM in either DM or PM is very rare, although Wagner included this sign in the original description of the PM in 1887 [6]. Regarding DM, only 14 cases have been reported in adults to date [7], [8], [9], [10], [11], [12], [13], [14], [15], [16].

Diagnosis relies on the clinical, typical electromyography pattern, elevated muscle enzymes, and muscle biopsy according to the criteria by Bohan and Peter [17] and Dalakas and Hohlfeld [4]. Treatment is initiated with corticosteroids, but in most cases, complementary drugs are necessary either to control the disease or to allow a reduction in corticosteroid doses.

Here, we report five cases of edematous DM among a series of 86 cases from a database of the Muscle Research Group in the Hospital Clínic of Barcelona from 2004 to 2013 and a review of all cases reported to date in adults.

Section snippets

Case reports

Patient no. 1 was a 72-year-old woman with a history of colon cancer 3 years previously treated with hemicolectomy and chemotherapy. She presented with progressive proximal muscle weakness of 3 weeks of duration. Dysphagia, diarrhea, and malaise were also present. Physical examination showed non-pitting edema in the upper limbs and the trunk and proximal muscular weakness; other systems were normal. Myositis-specific autoantibodies as well as serum tumor markers were negative. Creatine kinase

Discussion

Edematous DM is a rare form of this disease and, to date, only 14 cases have been reported in the medical literature. In our experience, less than 6% of 86 DM patients presented with this variant.

The mechanisms underlying dermatomyositis with edema are still unclear. The observation of raised values of vascular endothelial growth factor (VEGF) in some cases of seronegative symmetrical synovitis with edema prompted some investigators to assess VEGF in a case of edematous DM with negative results

Conclusions

  • Subcutaneous edema is a rare manifestation in DM, usually presenting in patients with very severe and active disease.

  • Prompt initiation of aggressive treatment with immunosuppressive agents and IVIG associated with glucocorticoids may be helpful.

  • Patients with edematous DM present with a shorter time of evolution (2–8 weeks) in comparison with other classical DM cases. Microischemia-producing microinfarction may play an important pathophysiological role and determine the degree of disease

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