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KMID : 0338419990140010095
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The Korean Journal of Internal Medicine
1999 Volume.14 No. 1 p.95 ~ p.98
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Hyperimmunoglobulin E-Recurrent Infection Syndrome In A Patient With Juvenile Dermatomyositis
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Jun Ki Min/Jun Ki Min
Mi La Cho/Seok Chan Kim/Youn Soo Lee†/Sang Heon Lee/Sung Hwan Park/Yeon Sik Hong/Chul Soo Cho/Ho Youn Kim
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Abstract
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A 13-year-old girl presented with multiple skin abscesses. She was diagnosed as having juvenile dermatomyositis (DM) at the age of 7 years. She had suffered from recurrent skin infections, atypical pruritic dermatitis and pneumonia since the age of 8 years. Bacteriologic and fungal cultures for skin abscesses and oral mucosa were positive S. aureus and C. albicans, respectively. Chemotactic defect in peripheral blood neutrophils was observed. The level of serum IgE was markedly elevated, and anti-S.aureus specific IgE was found. A diagnosis of hyperimmunoglobulin E-recurrent infection syndrome (HIE) was made and she was successfully treated with surgical drainage and antibiotics. To our knowledge, this is the first case report of HIE in a patient with juvenile dermatomyositis.
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KEYWORD
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Juvenile dermatomyositis, hyperimmunoglobulin E-recurrent infection syndrome (HIE),
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