Dermatomyositis: A dermatology-based case series

doi:10.1016/S0190-9622(98)70496-7

Journal of the American Academy of Dermatology

Volume 38, Issue 3, March 1998, Pages 397-404

https://doi.org/10.1016/S0190-9622(98)70496-7 Get rights and content

Abstract

Background: Dermatomyositis is associated with significant morbidity and occasional mortality. Currently there is no consensus on treatment for patients with dermatomyositis. Objective: Our purpose was to review the clinical features and response to therapy of patients with dermatomyositis and compare these data with previous series of patients with dermatomyositis/polymyositis. Methods: Clinical characteristics of 65 patients seen during a 10-year period were reviewed retrospectively. Twenty-one of these patients were enrolled in a prospective, uncontrolled study of treatment with high-dose prednisone followed by slow tapering. Results: Clinical features were similar to those previously described; however, muscle strength at diagnosis was on average greater in patients in this series than in patients previously reported. Malignancy was present in 5 of 43 adult patients (12%), but was not found in patients with juvenile dermatomyositis. Another connective tissue disease was present in 19% of patients. Twelve patients had dermatomyositis sine myositis. Eighteen of 21 patients (85%) in the prednisone study group had resolution of myositis. Conclusion: Patients with dermatomyositis in this series had less active myositis at presentation, but were otherwise similar to patients with dermatomyositis/polymyositis previously reported. Treatment with high-dose daily prednisone followed by slow tapering was effective. (J Am Acad Dermatol 1998;38:397-404.)

Section snippets

PATIENTS AND METHODS

Patients during a 10-year period (March 1986 to March 1996) who met the clinicopathologic criteria for dermatomyositis, as defined by Bohan and Peter,¹⁵ were examined. A total of 53 patients with dermatomyositis, 43 adults and 10 children, were seen. Forty-three patients had definite dermatomyositis, seven had probable dermatomyositis, and three had possible dermatomyositis. In, addition, 11 adults and one child with skin findings consistent with dermatomyositis sine myositis were seen. Patient

Clinical characteristics

The 43 adult patients with dermatomyositis consisted of 6 men and 37 women, with a mean age at diagnosis of 51.7 years (range, 21 to 82 years) (Table I). The 10 patients with juvenile dermatomyositis included 6 boys and 4 girls; mean age at onset in this group was 7.6 years (range, 1.5 to 17 years). The dermatomyositis sine myositis group consisted of one man and 10 women with a mean age of 48.6 years (range, 26 to 70 years). The male patient with dermatomyositis sine myositis was 17 years old

DISCUSSION

The clinical features of our patients, including age at onset, frequency of malignancy, and association with other connective tissue diseases, were similar to those described in previous series.6, 7, 12, 17, 18, 19, 20, 21, 22 There was a higher than expected ratio of female to male patients (6.2:1). This difference may be the consequence of a relatively small study group or selection bias related to early consultation for a cutaneous eruption.

Another exception was less active myositis at

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There is an increased incidence of malignancy in patients with dermatomyositis. It is unknown if the risk differs between the subtypes of dermatomyositis.
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^☆: Supported by the Mark and Catherine Winkler Foundation.

^☆☆: Reprint requests: Joseph L. Jorizzo, MD, Department of Dermatology, Wake Forest University School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157-1071.

^★: 0190-9622/98/$5.00 + 0 16/1/87610

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Dermatomyositis: A dermatology-based case series☆,☆☆,★

Abstract

Section snippets

PATIENTS AND METHODS

Clinical characteristics

DISCUSSION

Clin Dermatol

Dis Month

Am J Med

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

Current concepts in the idiopathic inflammatory myopathies: polymyositis, dermatomyositis, and related disorders

Ann Intern Med

The relationship of complement-mediated microvasculopathy to the histologic features and clinical duration of disease in dermatomyositis

Arch Neurol

Membrane attack complex deposits in cutaneous lesions of dermatomyositis

Arch Dermatol

Polymyositis: Its presentation, morbidity, and mortality

Brain

A computer-assisted analysis of 153 patients with polymyositis and dermatomyositis

Medicine

Polymyositis and dermatomyositis: combined methotrexate and corticosteroid therapy

Ann Intern Med

Azathioprine with prednisone for polymyositis

Ann Intern Med