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Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
Indexed/Abstracted in: EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,014
Pozzi M. L., Coassini A., Ambonati M., Ravanelli G., Lodi A.
From the IV Department of Dermatology, Ospedale S. Paolo, University of Milan, Milan, Italy
The hyperimmunoglobulinemia E syndrome is a rare immunologic defect characterized by significantly increased levels of IgE (2000 UI/ml), eczematous-like lesions that mostly occur on the face, cutaneous and systemic infections. The aethiopathogenetic process is a controversial matter. A 20-year-old male was affected since five years by recurrent nodular-cystic lesions with colliquative evolution on face, buttocks and thighs, associated with recurrent infections involving lungs and respiratory tract. Laboratory data pointed out high levels of IgE (2144 UI/ml), a decrease in CD4 lymphocyte count, HIV antibodies testing was negative, neutrophil function was normal. The patient was submitted to a systemic antibiotic therapy, obtaining only a temporary improvement in subcutaneous abscess. Then, a therapy with Isotretinoin per os was started associated with a topical antibiotic obtaining a moderate improvement. An association between hyper-IgE syndrome and defective neutrophil granulocyte chemotaxis has been reported, and related to an impaired production of immunological mediators. In our patient a defective neutrophil function was never found. We could assume that in vitro it is not possible to have a full evidence of these cells defects because the difficulties in reproducing patient’s conditions and in vivo complex interactions between humoral and cellular immunity.