Editor,—Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency defined by recurrent infections and markedly elevated serum immunoglobulin E (IgE) concentration. Because the primary defect in HIES is unknown, there is no specific treatment. In few cases, continuous treatment with trimethoprim sulfamethoxazole, recombinant interferon gamma or cyclosporin A have been used to reduce the serum IgE levels (up to 20%, 55%, and 95%, respectively).1 ,2 However, adverse effects with such long term treatments have been described in children with HIES.2

We report the case of a 12 year old Caucasian girl with HIES and severe bronchiectasis treated for 3 years with oral cefadroxil, a first generation cephalosporin known for its potential direct effect on IgE production.3 ,4 Our patient, with daily productive cough and wheezing, suffered from six to eight bronchitis or severe pneumonias per year (Candida albicans,Haemophilus influenzae). Blood tests showed increased IgE levels (8650 IU/ml). Skin prick tests were positive to dust mites and Alternaria extracts. Pulmonary function tests showed a severe bronchial obstruction (fig 1). Bronchiectasis involved all lobes except the right middle lobe. She was treated unsuccessfully with daily chest physiotherapy, antibiotics when needed, cetirizine, inhaled budesonide, and salmeterol. Cefadroxil (Oracefal, Bristol-Myers Squibb Laboratories) was introduced at an arbitrary dose of 500 mg per day. Serum IgE quickly decreased and became stable at around 500 IU/ml (94% decrease) after 2.5 years of treatment (fig 1). Moreover, our patient became symptom free with a nearly normalised pulmonary function test. No incident or side effect was reported and our patient currently continues to receive cefadroxil.

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Figure 1

Serum immunoglobulin E (IgE) levels and forced expiratory volume in one second (FEV1) values of a child with hyperimmunoglobulin E syndrome and severe bronchiectasis treated with cefadroxil (500 mg/day) for 3 years.

We are the first to report such a clinical and respiratory functional improvement with cefadroxil in a paediatric patient with bronchiectasis caused by HIES. Further clinical trials are required, however moderate doses of cefadroxil should be recommended in severe HIES before using more toxic drugs.