Juvenile Dermatomyositis Sine Myositis

This 8-year-old girl has had swollen, red eyelids for the past 24 hours. She denies pain or pruritus. She has had no eye drainage, fever, or other systemic signs of illness. She reports some mild fatigue for the past few months but denies myalgias or decreased muscle strength. Her medical history includes type 1 diabetes mellitus diagnosed at 3 years of age. The child is afebrile on examination. She has full range of motion of the eyes, no scleral injection, and normal pupillary reflexes. Muscle strength is normal in all extremities; no abnormalities of the hands or fingers are apparent.
 

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Answer: Heliotropic rash consistent with juvenile dermatomyositis sine myositis.

Juvenile dermatomyositis is a rare autoimmune vasculitis in children. Although the pathogenesis is still unclear, a genetic susceptibility to acquiring the disease among first-degree relatives and an association with other autoimmune diseases have been noted. Affected children often present with symmetrical proximal muscle weakness, weight loss, fever, fatigue, and anorexia, or they may present with one or both of the classic rashes of the disease.¹ The heliotropic rash-the presenting symptom in this child-is a violaceous flat rash of the eyelids, sometimes with malar involvement, that is often accompanied by periorbital edema. The other characteristic rash, Gottron sign, is an erythematous eruption over the dorsum of the knuckles.

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Dermatomyositis sine myositis is a term used to describe patients who present with the dermatological features of dermatomyositis but an absence of associated muscle findings. It is not uncommon for children to present with cutaneous findings before the onset of muscle involvement. Sometimes the presentation of the dermatological features of the disease can precede the muscle features by months to years.²

Complications of dermatomyositis include contractures, GI vasculopathy, and calcinosis. Treatment includes immunosuppression and physical therapy. For patients with the heliotropic rash, which is photosensitive, the judicious use of sunscreen is advised.³