Medical management and pharmacology update
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Dermatomyositis: a contemporary review for oral health care providers

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Dermatomyositis (DM) is an autoimmune muscle disorder characterized by skin rashes and progressive muscle weakness. This disease is a subset of the idiopathic inflammatory myopathies (IIM) and affects both children and adults. Increased incidence of malignancy has been observed in adult DM, making early diagnosis, treatment, and monitoring crucial. In the past, limitations of these diagnostic criteria and classifications often made it difficult to identify the disease in the early stages. However, in recent years a new understanding of the pathogenesis and clinical features of DM has developed, which enables clinicians to distinguish DM from other autoimmune disorders and other subsets of IIM. Many signs and symptoms of DM involve the orofacial regions. This paper reviews and updates the disease process, orofacial manifestations, and dental considerations for patients with DM. Understanding new knowledge of DM helps oral health care providers coordinate care for patients with this disease.

Section snippets

Epidemiology

DM shows a bimodal age distribution, with children under 18 years of age and adults in their late 40s to early 60s most affected.9 DM affects females more than males, and juvenile DM (JDM) is defined by onset before 18 years of age. Information about the incidence and prevalence of DM is limited because of the rarity of the disease and its inconsistent diagnostic criteria. Earlier studies estimated an incidence ranging from 2 to 10 new cases per million persons,4, 10 and a recent

Etiology and Pathogenesis

The exact cause of DM is unknown, but it is considered a complement-mediated microangiopathy6, 12, 13 in which the specific target of immunoreaction is intramuscular capillaries. It has been hypothesized that the activation of complement components is followed by deposition of the C5b–C9 membranolytic attack complex on intramuscular capillaries, which leads to perivascular inflammation, loss of capillaries, muscle ischemia, and perifascicular muscle fiber atrophy.6, 13 However, because neither

Clinical Features

A characteristic feature of DM is skin rash mainly on the face, which precedes, accompanies, or follows progressive proximal muscle weakness. The presence of skin change has been used by many clinicians to distinguish DM from polymyositis (PM).18 However, skin lesions can be transient, atypical, or completely missing (adermatopathic DM). Similarly, the characteristic proximal symmetric muscle weakness may not be noticeable until late in the course of the disease or even completely absent

Diagnosis

Diagnostic criteria and classification of DM have been evolving since the first diagnosed case in 1875.3 An early but influential classification and set of diagnostic criteria were proposed by Bohan and Peter in 1975 (Table I),36 based mainly on their clinical experience with IIM. Most recently, a classification based on more objective diagnostic criteria, prediction of disease course, and responsiveness to therapy has been proposed (Table II). DM without muscle involvement has been fully

Medical Management and Treatment

There is no cure for DM. The goal of treatment is to manage the symptoms, improve functions, and further prevent disability. Because of the complexity of the disease, a multidisciplinary team approach and patient education are particularly important.19, 46

Prognosis

Most patients respond to early treatment. However, when cardiac or pulmonary organs are involved, patients may be refractory to therapy. Other indicators of poor prognosis include delay in diagnosis, older age, fever, dysphagia, and the association with malignancy.19 The overall 5-year survival rate is approximately 80%.9, 53 However, it is only 69% in patients with dysphagia54 and 10% in patients with cancer.55 The most common causes of death are respiratory failure in association with

Orofacial Manifestations

Along with its characteristic facial and/or scalp skin rashes, as previously described in this article, a variety of oral manifestations have been reported,57, 58, 59 sometimes as initial manifestations of DM.60, 61 However, the prevalence of oral involvement in DM is unknown. Most available information comes from single case reports or small case series, and some early case reports did not clearly separate DM from PM. The case reports we summarize below represent the best knowledge available (

Dental Considerations

Oral health providers play an important role in the early detection of DM through careful observation of patients' signs and symptoms. Identifying oral and maxillofacial cancer and dysphagia is particularly important because these conditions affect the outcome of patients with DM. During the health history review, the following questions are recommended to assess dysphagia.75, 77

Does food get stuck in your throat?

Do you have to swallow repeatedly to get rid of food?

Do you cough during meals?

Do

Conclusions

Although DM is a rare disease, its association with cancer, dysphagia, and orofacial manifestations has great relevance for oral health care providers. It is important to recognize and understand this complex disease because providers may be able to identify initial signs and symptoms in patients who are as yet undiagnosed with DM. Coordination of care with physicians is essential for successful dental management and improvement of quality of life for DM patients. Oral health care providers are

Acknowledgment

This review is dedicated to Dr. S. Miles Standish, an oral pathologist who specialized in muscle disorders.

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