ABSTRACT
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.
Subject(s)
Female , Humans , Adolescent , Dermatomyositis/complications , Immunoglobulin E/blood , Job Syndrome/immunology , Job Syndrome/diagnosis , Job Syndrome/complications , Staphylococcal Infections/immunology , Staphylococcal Infections/complications , Staphylococcus aureus/immunologySubject(s)
Humans , Neutropenia/physiopathology , Phagocyte Bactericidal Dysfunction/etiology , Leukocyte-Adhesion Deficiency Syndrome/physiopathology , Chemotaxis/physiology , Neutropenia/classification , Neutropenia/etiology , Phagocyte Bactericidal Dysfunction/diagnosis , Chediak-Higashi Syndrome/surgery , Chediak-Higashi Syndrome/diagnosis , Chediak-Higashi Syndrome/drug therapy , Leukocyte-Adhesion Deficiency Syndrome/diagnosis , Job Syndrome/physiopathology , Job Syndrome/immunologyABSTRACT
El síndrome de hiper IgE (SHIE) es una rara entidad que se caracteriza por presentar infecciones cutáneas y respiratorias, sobre todo neumonías a estafilococo, con posterior formación de neumatoceles, disturbios en el metabolismo óseo y una IgE sumamente elevada. En el presente artículo se trata de actualizar el tema en sus aspectos fisiopatológicos, inmunológicos y nuevas conductas de tratamiento