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Giornale Italiano di Dermatologia e Venereologia 2001 February;136(1):45-7

Copyright © 2001 EDIZIONI MINERVA MEDICA

language: English

A case of hyperimmunoglobulinemia E syndrome

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


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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.

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