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Hyperimmunoglobulin E-Recurrent Infection Syndrome In A Patient With Juvenile Dermatomyositis

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...

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Detalles Bibliográficos
Autores principales: Min, Jun-Ki, Cho, Mi-La, Kim, Seok-Chan, Lee, Youn-Soo, Lee, Sang-Heon, Park, Sung-Hwan, Hong, Yeon-Sik, Cho, Chul-Soo, Kim, Ho-Youn
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Korean Association of Internal Medicine 1999
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4531902/
https://www.ncbi.nlm.nih.gov/pubmed/10063322
http://dx.doi.org/10.3904/kjim.1999.14.1.95
Descripción
Sumario: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.