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PubMed1999

Dermatomyositis. Diagnosis and evaluation of dermatomyositis, polymyositis, and inclusion-body myositis.

In diseases of an unknown etiology, such as the idiopathic inflammatory myopathies, we must tackle first of all the question of classification and the degree of disease activity before we can institute treatment. The majority of idiopathic inflammatory myopathies are diagnosed clinically and confirmed by biopsy. The presently applicable methods of diagnosis and evaluation of idiopathic inflammatory myopathy have certain limitations, and hence it is necessary to apply new methods of rating the disease activities. Magnetic resonance imaging (MRI) and 99m-technetium muscle-scanning are the latest noninvasive methods for evaluation of disease activities with myositis. Future laboratory methods to determine the numerous myositis-specific autoantibodies will probably enable identification of subsets of these diseases.

Autoantibodies↗
PubMed2004

The management of dermatomyositis: current treatment options.

Dermatomyositis is traditionally classified as one of the idiopathic inflammatory myopathies. The traditional approach to the management of patients with dermatomyositis focuses predominantly on end points related to the systemic manifestations of this disorder, especially proximal muscle weakness resulting from myositis. However, the primary and secondary skin changes that are characteristic of dermatomyositis can, in themselves, produce significant morbidity and disability. This article presents a dermatological perspective on the management of dermatomyositis. As dermatological management approaches can vary between countries, an effort has been made to present a consensus dermatological view concerning this subject. A US dermatologist is most likely to see patients having dermatomyositis skin lesions in the following four clinical settings: soon after the onset of dermatomyositis skin disease activity before the appearance of clinically-evident muscle disease (i.e., 'pre-myopathic' dermatomyositis); during the course of clinically-amyopathic dermatomyositis; during co-management of classical dermatomyositis with other physicians; and for recrudescent skin disease activity in classical dermatomyositis patients after their symptomatic muscle inflammation has been fully suppressed by systematic immunosuppressive/immunomodulatory treatment (i.e., 'postmyopathic' dermatomyositis). Both topical and systemic therapies for dermatomyositis skin lesions encountered in these various settings will be discussed. In addition to specific approaches to the treatment of dermatomyositis skin lesions, broader management issues that dermatologists must be aware of when caring for dermatomyositis patients are included in this discussion (e.g., patient education, risk of associated systemic disease, such as myositis and interstitial lung disease, risk for occult malignancy, and prognosis counselling).

Dermatomyositis↗
PubMed1995

Nasopharyngeal carcinoma with dermatomyositis. Analysis of 12 cases.

OBJECTIVES: To evaluate the incidence rate of nasopharyngeal carcinoma with dermatomyositis, the influence of dermatomyositis on clinical course, and complications and survival of patients with nasopharyngeal carcinoma after radiotherapy. DESIGN: A retrospective study of 12 patients with nasopharyngeal carcinoma associated with dermatomyositis, with a maximum follow-up of 228 months. SETTING: Academic tertiary referral center. PATIENTS: There were 6441 new patients with nasopharyngeal carcinoma who were seen at the National Taiwan University Hospital, Taipei, during the period from 1970 through 1993. Twelve patients were found to have dermatomyositis. MAIN OUTCOME MEASURE: Clinical manifestations of nasopharyngeal carcinoma with dermatomyositis. RESULTS: Twenty-seven (26%) of 104 patients with dermatomyositis had an associated malignancy. Twelve of these patients were diagnosed as having nasopharyngeal carcinoma. The typical skin manifestation of dermatomyositis was found in all 12 patients. Myopathy occurred in 10 patients. Three patients died of recurrence of nasopharyngeal carcinoma, one died of a second malignancy, one died of upper gastrointestinal bleeding, and one became unavailable for follow-up. The other six patients have had disease-free survival, with a mean follow-up period of 100.5 months (range, 5 to 228 months). The 1-year survival rate was 83.8%, and the 5-year survival rate was 69.4%. CONCLUSIONS: In Taiwan, dermatomyositis is associated with an increased incidence rate of nasopharyngeal carcinoma. A search for nasopharyngeal carcinoma in patients with dermatomyositis should be performed in areas prevalent for nasopharyngeal carcinoma. If present, nasopharyngeal carcinoma can precede, occur concurrently with, or follow the diagnosis of dermatomyositis. Treatment of nasopharyngeal carcinoma may affect myositic or cutaneous disease. The prognosis and survival rates of nasopharyngeal carcinoma remained unaffected by dermatomyositis. No complications were noted owing to the radiotherapy used to treat dermatomyositis.

Adult↗
PubMed1994

[Vascular manifestations of dermatomyositis and polymyositis. Clinical, capillaroscopic and histological aspects].

Polymyositis is characterized by a T-cell-mediated and MHC-I-restricted cytotoxic process, whereas dermatomyositis is a primitively vascular disease with microangiopathy mediated by the complement C5b-9 membranolytic attack complex. We have tried to estimate the frequency of vascular abnormalities in polymyositis as defined by Bohan and Peter. We have retrospectively studied 17 patients with dermatomyositis and 15 patients with polymyositis. Vascular abnormalities have been defined by clinical, capillaroscopic and histologic (muscle biopsy and minor salivary glands biopsy) features. After clinical features, 5/17 dermatomyositis had a Raynaud's phenomenon, against 6/15 polymyositis. Digital necrosis has been observed for 2/17 dermatomyositis and 2/15 polymyositis. In capillaroscopy, 14/17 dermatomyositis had a microangiopathy with or no enlarged capillary loops, against 7/15 polymyositis. None of these polymyositis had enlarged capillary loops. The muscle biopsy showed a predominantly perivascular or perimysial inflammatory infiltrate (vascular process) for 10/16 dermatomyositis against 4/13 polymyositis; a perifascicular atrophy for 3/16 dermatomyositis against 2/13 polymyositis. The histological study of minor salivary glands, showed vascular lesions for 2/11 dermatomyositis and for 1/8 polymyositis. Finally, Bohan and Peter's classification is now inadequate to distinguish between dermatomyositis and polymyositis. Indeed, some dermatomyositis sine dermatitis, may exist and be recognized by their vascular features. To distinguish between dermatomyositis and polymyositis is important, to evaluate the risk of cancer which is more frequent in dermatomyositis.

Adult↗
PubMed2002

Breast carcinoma in patients with dermatomyositis: a retrospective analysis of eight cases.

BACKGROUND: The association between dermatomyositis and breast carcinoma has not been fully evaluated yet. The aim of the study was to clarify characteristics of the association between these two diseases. METHODS: The medical records of 128 patients with dermatomyositis or polymyositis from 1990 to 1998 were retrospectively reviewed. Eight patients (6.3%) were identified with dermatomyositis which was associated with an underlying breast carcinoma. Clinical features, laboratory data, electromyograms, muscle biopsies, and the prognoses of these patients were assessed. RESULTS: The mean age of the 8 patients with breast carcinoma and dermatomyositis (62.1 +/- 6.7 years) was larger than that of patients with breast carcinoma without dermatomyositis (48.5 +/- 11.8 years). Dermatomyositis preceded breast carcinoma in 2 patients, was concurrent with breast carcinoma in 5 patients, and followed breast carcinoma in 1 patient. Of these 8 patients, 4 had TNM classification stage IV (M1) breast cancer, 1 had IIIA (T2N2M0) breast cancer, and 3 had IIB (T2N1M0) breast cancer. The follow-up period ranged from 2 to 79 (median, 21) months. Five patients died of recurrence or distant metastasis, and the remaining 3 patients had disease-free survival, with a follow-up period ranging from 11 to 28 (median, 20) months. Four of 5 patients had parallel improvement in dermatomyositis after surgical treatment of breast carcinoma. CONCLUSION: In Taiwan, dermatomyositis is associated with an increased incidence of breast carcinoma. The mean age of the 8 patients with breast carcinoma and dermatomyositis was significantly larger than that of patients with breast carcinoma without dermatomyositis. If present, breast carcinoma can precede, occur concurrently with, or follow the diagnosis of dermatomyositis. Surgical treatment of breast carcinoma may alleviate the course of dermatomyositis and eliminate the need for steroids in some cases.

Adult↗