Initial management of cutaneous dermatomyositis
- Ruth Ann Vleugels, MD, MPH
Ruth Ann Vleugels, MD, MPH
- Associate Professor in Dermatology
- Harvard Medical School
Classic dermatomyositis (DM) is an idiopathic inflammatory myopathy that most commonly presents with progressive, symmetric proximal muscle weakness and a group of characteristic cutaneous findings. The cutaneous manifestations of DM may also develop in the absence of detectable muscle disease, and can persist after the successful treatment of DM-associated myositis.
Patients with DM often present with intensely pruritic areas of confluent, violaceous erythema on the scalp, face, upper trunk, and upper extremities. Pruritus can be disabling.
Skin lesions of DM are often resistant to photoprotection and topical therapies alone, necessitating the initiation of antimalarial drugs and/or methotrexate. Patients who fail to respond to these interventions may require more aggressive immunosuppressive or immunomodulatory therapies.
The initial management of the cutaneous manifestations of DM will be discussed here. The treatment of refractory DM, as well as the clinical manifestations, diagnosis, and management of the noncutaneous manifestations of DM and juvenile DM are reviewed elsewhere. (See "Management of refractory cutaneous dermatomyositis" and "Clinical manifestations of dermatomyositis and polymyositis in adults" and "Diagnosis and differential diagnosis of dermatomyositis and polymyositis in adults" and "Initial treatment of dermatomyositis and polymyositis in adults" and "Treatment of recurrent and resistant dermatomyositis and polymyositis in adults" and "Juvenile dermatomyositis and polymyositis: Epidemiology, pathogenesis, and clinical manifestations" and "Juvenile dermatomyositis and polymyositis: Treatment, complications, and prognosis".)
OVERVIEW OF CLINICAL FEATURES
Skin changes associated with dermatomyositis (DM) include pathognomonic findings such as Gottron's papules (violaceous papules overlying interphalangeal and metacarpophalangeal joints) and Gottron's sign (macular violaceous erythema overlying other joints, such as the elbows or knees) (picture 1A-D). Violaceous erythema involving the periorbital skin (heliotrope eruption), scalp, V of the neck, shoulders, extensor surfaces of the upper extremities, upper chest, and upper back are additional characteristic findings in DM (picture 2A-D). Scale may or may not be present, but is typically prominent on the scalp, where it may be accompanied by diffuse alopecia (picture 2E).
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- OVERVIEW OF CLINICAL FEATURES
- Impact on quality of life
- Disease course
- CLASSIFICATION OF CUTANEOUS DM
- Classic DM
- Amyopathic DM
- Hypomyopathic DM
- Postmyopathic DM
- PATIENT EVALUATION
- Indications for biopsy
- Additional tests
- Approach to treatment
- Interventions for pruritus
- Topical therapy
- - Topical corticosteroids
- - Topical calcineurin inhibitors
- - Other
- Systemic therapy
- - Approach to systemic therapy
- - First-line systemic therapy
- - Efficacy and administration
- - Adverse effects
- - Second-line systemic therapy
- - Efficacy and administration
- - Adverse effects
- RECURRENT AND RESISTANT DISEASE
- CALCINOSIS CUTIS
- SUMMARY AND RECOMMENDATIONS