Sammendrag

• An idiopathic inflammatory myopathy characterised by the presence of hallmark cutaneous lesions (e.g., heliotrope rash, Gottron's papules).
• Skin lesions are frequently the initial presenting complaint, with muscle involvement developing later.
• Some patients have cutaneous involvement alone.
• May be associated with interstitial lung disease, cardiac involvement, oesophageal involvement, and underlying malignancy.
• Initial treatment of muscle disease is with high-dose oral corticosteroids (prednisone), followed by the addition of immunosuppressants and intravenous immunoglobulin in refractory cases.
• Skin manifestations are treated with topical antipruritics and topical corticosteroids. Antimalarials, topical tacrolimus, immunosuppressants, and intravenous immunoglobulin are generally used for resistant or severe skin disease. Photoprotection is advised in all patients.