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Chapter 47: Dermatomyositis

Jannett Nguyen; David Fiorentino

DERMATOMYOSITIS

Therapeutic Approach

Dermatomyositis (DM) is a systemic autoimmune inflammatory condition primarily affecting skin, muscle, and lungs. Patients with skin disease without objective evidence of muscle weakness are classified as clinically amyopathic. DM is associated with higher cancer risk and, as such, thorough evaluation for malignancy is important, especially during the first 3 years from disease onset. In addition, baseline pulmonary function tests are recommended, and should be repeated should new respiratory symptoms develop. Treatment is tailored according to organs involved, severity of disease, and medical comorbidities. In most cases, topical therapy is insufficient for control of cutaneous disease, and antimalarials are often considered first-line options for systemic treatment. Other systemic therapies used to treat other organ involvement can also be helpful for skin disease. This treatment table primarily focuses on the current evidence for managing skin manifestations of DM. For patients with muscle or lung involvement, collaboration with a rheumatologist, neurologist, and/or pulmonologist is recommended. See Table 47-1.

Table 47-1Dermatomyositis Treatment Table
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Table 47-1 Dermatomyositis Treatment Table
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MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Sunscreen Skin Photoprotective At least SPF 30; apply daily with reapplication q2-3h Skin irritation None III1
Corticosteroids Skin Anti-inflammatory Strength and vehicle of topical steroid depend on treatment area; apply daily to BID Skin atrophy, dyspigmentation, skin fragility None III1
Calcineurin inhibitors Skin Anti-inflammatory Pimecrolimus 1% cream, tacrolimus 0.1% ointment; apply daily to BID Skin irritation None III1-3
Systemic Therapy

Antimalarial agents: Hydroxychloroquine (HCQ), chloroquine (CQ), quinacrine

Can use quinacrine in conjunction with HCQ or CQ, but do not use HCQ and CQ together given risk of retinopathy

 Skin, joints Inhibits ultraviolet radiation induced reactions, anti-inflammatory HCQ 4-5 mg/kg/d CQ 2-3 mg/kg/d Quinacrine 100 mg daily GI upset, retinopathy (CQ > HCQ), cutaneous hypersensitivity reaction (HCQ), blue-gray skin discoloration (HCQ), yellow skin discoloration (quinacrine) For HCQ and CQ, baseline ophthalmology exam (then annually after 5 y). CBC-d and CMP q4-6mo III1,4

Systemic corticosteroids

Generally used for short-term treatment of flares, with concurrent initiation or continuation of steroid-sparing agent.

 Skin, muscle, lung, joints Anti-inflammatory Prednisone 0.5-1 mg/kg/d with taper for skin flares Higher equivalent doses of systemic corticosteroids may be used for severe muscle or lung disease, and may be given as pulse-dose or split-dose, with slow taper. Hyperglycemia, hypertension, hyperlipidemia, Cushingoid changes, cataracts, peptic ulcers, osteoporosis, myopathy, psychosis, infection

CBC-d, CMP, lipid panel q2-3mo; blood pressure checks

Baseline DEXA scan if long-term treatment is anticipated

 III,1,4#IB5
Dapsone Skin Anti-inflammatory, inhibits folate synthesis, inhibits neutrophil activity 25-150 mg daily Hemolytic anemia, methemoglobinemia, agranulocytosis, peripheral neuropathy, hypersensitivity reaction

Baseline: CBC-d, CMP, screen G6PD level

Monitor: CBC-d q1-2wk until 3 mo or with dose escalation, then CBC-d and CMP q3mo

 III1
Methotrexate Skin, muscle, joints Anti-proliferative, inhibits folate synthesis

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