Treatment of Recalcitrant Dermatomyositis with Efalizumab

Abstract

Dermatomyositis (DM) is an auto-immune disease that primarily affects muscles and skin. Muscle weakness appears in the shoulders and hips in particular. Skin symp-toms include a diffuse violaceous erythema and oedema, predominantly of the upper face including the orbita, and violaceous-red papules of the finger (Gottron papules). The pathogenesis of DM is unclear, but recent studies suggest a central role of auto-aggressive lymphocytes and type I interferons (1–4), as has been proposed earlier for cutaneous lupus erythematosus (5–7). Corticosteroids are the established first-line treatment for DM. However, their use is limited because of side-effects. Unfortunately, recalcitrant courses exist that respond poorly to corticosteroids and to other immuno-modulatory and immunosuppressive drugs (8). CASe RepORTAn 82-year-old woman presented with peri-orbital viola-ceous-red oedema and erythema extending to the chest with proximal muscle weakness (Fig. 1a). She suffered from dysphagia and 6 kg weight loss during the last 4 months. Histology of lesional skin biopsy, taken from the chest, revealed an interface dermatitis associated with sparse lymphocytic infiltration in a perivascular distribution and mucin depositions. electromyographic investigations confirmed a pathological reaction pattern in the biceps brachii muscle, indicating myositis. Muscle biopsy was denied. She had slightly elevated antinuclear antibodies (1:80, granular pattern), but blood count, liver enzymes, and serum creatinine kinase were within normal limits. No malignancy could be found.Initial treatment with 60 mg prednisolone per day did not improve the clinical picture. Additional intravenous administration of 15 mg methotrexate per week for 8 weeks also had no effect.This prompted us to initiate a subcutaneous treatment with efalizumab (Raptiva