Hyperimmunoglobulin E syndrome.

How to Cite

Petrova E., Semkova K., Miteva L. 2011. Hyperimmunoglobulin E syndrome. Eur. J. Pediat. Dermatol. 21 (2):86-91.


Petrova E. Semkova K. Miteva L.
pp. 86-91


Hyperimmunoglobulin E syndrome (HIES) is a rare immunodeficiency presenting with a triad of recurrent skin and pulmonary infections, elevated IgE and eczematous reaction. Two forms of HIES are currently recognized – autosomal-dominant and autosomal-recessive –. Treatment is symptomatic and the main therapeutic targets for HIES patients are prevention and management of infections. Systemic antistaphylococcal antibiotics are the mainstay of treatment. We report a case of HIES seen and followed up at our clinic and review the current literature. In our patient systemic acitretin and multiple courses of antibiotics led to a remarkable improvement and a remission period that has so far been maintained.


Hyperimmunoglobulin E syndrome, Recurrent skin infections, Eczema, STAT3, Acitretin