Physical examination reveals definite proximal muscle weakness, a periorbital heliotrope rash, and skin findings associated with the hands (shown here).
The most likely diagnosis isA) Lupus erythematosus
C) Sjögren’s disease
E) Polymyalgia rheumatica
The answer is D. (Dermatomyositis)
Discussion: Dermatomyositis presents with a heliotrope rash and the presence of Gottron’s papules. The rash is violaceous in color and involves the periorbital areas. Gottron’s papules are erythematous or violaceous papules or plaques that form over the bony prominences, particularly the metacarpophalangeal joints, the proximal and distal interphalangeal joints. The condition is also associated with a myopathy that involves the proximal muscles. The shoulders and pelvic
girdle are mainly affected in a symmetric pattern.
Symptoms include fatigue, weakness, inability to climb stairs, or weakness in rising from a
squatting or sitting position. Dysphagia is also seen. Approximately 20% of patients have an associated malignancy.
Laboratory tests include elevated serum creatine kinase or aldolase, or both. Electromyographic studies and a muscle biopsy can also provide additional diagnostic information.
The mainstays of treatment are systemic steroids. Other options include immunosuppressant agents and hydroxychloroquine. Those who are older and have severe myositis, dysphagia, associated malignancy, and a poor response to corticosteroids have a poorer prognosis.