Juvenile Dermatomyositis Presenting with Erythematous Swelling of the Upper Eyelid

ABSTRACT

Purpose@#To report a case of juvenile dermatomyositis that presented with erythematous swelling on the upper eyelid as the first clinical sign.Case summary: An 11-year-old female patient presented with erythematous swelling of both upper eye lids that persisted for 3 weeks prior to examination. She also had an erythematous rash over both cheeks but no pain or hyperemia in either eye. During anti-inflammatory eye drop treatment under suspicion of blepharitis, a reddish-purple heliotrope rash appeared around the eyelid margin. She also developed fever, arthralgia, multiple oral ulcers, an erythematous scaly rash behind the ear, and erythematous firm papules on the metacarpal (MCP) joint and around the elbow. On evaluation, aspartate aminotransferase was elevated to 184 IU/L, alanine aminotransferase to 352 IU/L, and alkaline phosphatase to 266 IU/L. We performed a skin biopsy. Based on histopathological examination, the eyelid lesion was diagnosed as dermatomyositis and the MCP joint lesion as Gottron’s papule. From the above findings, we diagnosed the patient with juvenile dermatomyositis based on the European League Against Rheumatism/American College of Rheumatology classification. The patient was treated with prednisolone. Three months after diagnosis, she died from rapidly progressive interstitial pneumonia, a complication of juvenile dermatomyositis. @*Conclusions@#Heliotrope rash should be considered in the differential diagnosis of patients presenting with eyelid swelling, for early diagnosis and better prognosis of juvenile dermatomyositis.