SeminarDermatomyositis
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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Cited by (351)
2020 guide for the diagnosis and treatment of interstitial lung disease associated with connective tissue disease
2021, Respiratory InvestigationCitation Excerpt :However, because anti-MDA5 antibodies are accompanied by a skin rash characteristic of “dermatomyositis” in most cases, it is necessary to discuss and accumulate cases to determine whether ILD cases without skin eruptions need to be screened. PM is a CTD that causes muscle weakness with symmetric proximal muscle predominance because of inflammation of the skeletal muscle, with typical cutaneous symptoms diagnosed as DM [36]. If there are only skin symptoms with no clinical myositis findings, it is defined as CADM [37].
A CASE OF CLINICALLY AMYOPATHIC DERMAMYOSITIS WITH INTERSTITIAL LUNG DISEASE SHOWING TWO DETERIORATIONS IN 4 YEARS
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