Elsevier

The Lancet

Volume 355, Issue 9197, 1 January 2000, Pages 53-57
The Lancet

Seminar
Dermatomyositis

https://doi.org/10.1016/S0140-6736(99)05157-0Get rights and content

Summary

Dermatomyositis is one of the idiopathic inflammatory myopathies with characteristic cutaneous manifestations including the heliotrope rash, Gottron's papules, cuticular changes including periungual telangiectasia, a photodistributed erythema or poikiloderma, and a scaly alopecia. Dermatomyositis has been linked to cancer, particularly ovarian cancer. Cancer-associated disease is more commonly found in older patients, and when present, is associated with a poor prognosis. A childhood form of the disease exists and is frequently complicated by the development of calcinosis. Dermatomyositis is a systemic disorder and whereas the skin and muscles are the most commonly affected organs, patients may have arthralgias, arthritis, oesophageal disease, or cardiopulmonary dysfunction. Recently described serological abnormalities, known as myositis-specific antibodies, add credence to the notion that this disorder is distinct from all other collagen-vascular diseases, and may lead to important discoveries about the pathogenesis of the inflammatory myopathies, which are not currently of practical use in the clinic or office. Management of the patient with myositis usually includes systemic corticosteroids with or without an immunosuppressive agent. Cutaneous disease is more difficult to manage, but antimalarials, methotrexate, and intravenous immunoglobulin are effective in small, often open-label, studies.

Section snippets

Cutaneous manifestations

A heliotrope rash and Gottron's papules are characteristic and possibly pathognomonic cutaneous features of dermatomyositis. The heliotrope rash is a violaceous to dusky erythematous rash, with or without oedema, in a symmetrical distribution involving periorbital skin (figure 1). This sign can be slight and may appear only as a mild discolouration along the eyelid margin. A heliotrope rash is rarely seen in lupus erythematosus and scleroderma, thus its presence is highly suggestive of

Myositis and malignant disease

The link of dermatomyositis and polymyositis to malignant disease has been clarified.8 The reported rate of malignant disease in dermatomyositis has varied from 6–60%, with most large population-based cohort studies reporting a rate of 20–25%. Several Scandinavian studies have documented an increased rate of malignant disease associated with dermatomyositis over that observed in the general population.8, 18, 19 Although patients with polymyositis have a slight increase in rates of cancer, the

Childhood dermatomyositis

Dermatomyositis is more common than polymyositis in children and adolescents.22 A fulminant course might occur, but most commonly the onset is indolent and children are first thought to have viral infections or dermatitis. Delayed diagnosis is more common in non-white people. Childhood dermatomyositis is commonly characterised as a vasculitis, but the major difference to adult disease is the greater potential for calcinosis.

Drug-induced dermatomyositis

The cause of most cases of dermatomyositis is unknown, but, in a few patients, the cutaneous manifestations are caused by, or exacerbated by drugs. This effect has been best documented for hydroxyurea in which de-challenges and re-challenges have been done.23 However, quinidine, non-steroidal anti-inflammatory drugs,24 penicillamine, and 3-hydroxy-3-methylglutaryl coenzyme A-reductase inhibitors have also been linked to dermatomyositis.

Diagnosis and assessment of patients with dermatomyositis

The diagnosis of dermatomyositis is suspected in patients with clinically compatible cutaneous findings. Exclusion of other possible cutaneous conditions is aided by results of skin biopsy samples and the diagnosis of muscle involvement. In the absence of identifiable myopathy, the differentiation from cutaneous lupus erythematosus might be difficult. Muscle weakness may be caused by many other factors, including toxins, infections, metabolic abnormalities, and neurological disorders. However,

Therapy

Several general measures are helpful in treating patients with dermatomyositis. Bedrest is usually valuable in patients with progressive weakness, but must be combined with a range-of-motion exercise programme to prevent contractures. Patients who have evidence of dysphagia should have the head of their bed raised and should avoid eating meals immediately before they go to bed.

The mainstay therapy for dermatomyositis is systemic corticosteroids. Traditionally, 0·5–1·0 mg/kg bodyweight

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