SELECTED ARTICLES FROM THE RECENT LITERATURE 2005

10/25/05

Hyper-IgE syndromes

Summary
Background - An elevated serum IgE occurs commonly in allergic disorders. The hyper-IgE syndromes (HIES) are very uncommon disorders that must be considered in the differential diagnosis when the serum IgE is very high.

Findings - Grimbacher et al of the Univ of Freiburg in Germany reviewed the HIES, primary immuno-deficiencies characterized by a triad of: 1) recurrent staph absecces; 2) recurrent cyst-forming pneumonia, and 3) considerably increased serum IgE, (above 2000 IU/ml). Most cases are sporadic. However, multiple cases within a family displaying either autosominal dominant (AD) or autosomal recessive (AR) patterns have been described.

In most sporadic and AD cases the clinical triad described above is part of a multisystem disorder including abnormalities of the soft tissues, skeleton and dental architecture. In contrast, the AR-HIES cases have severe molluscum contagiosum and perhaps severe neurological complications. However, the AR-HIES cases lack skeletal or dental abnormalities and do not develop lung cysts.

The etiology of the HIES is still not definite with some evidence of a Th1/Th2 imbalance and involvement by chemokines.

Reference
Immunol Rev 2005;203:244-250

Editor's Comments
In usual atopics with predominantly respiratory manifestations, the serum IgE is often increased, but almost always <1000 IU/ml. However, in atopics with extensive atopic dermatitis (AD), markedly elevated serum IgE levels are sometimes present. In many AD patients, staph infection of the skin is often present, aggravating the skin problems. One must differentiate between staph complications of AD and the recurrent eczema and staph abscesses typical of HIES. Indeed, anti-staph antibiotic therapy is helpful in both AD and HIES. However, lung cysts, dental and skeletal abnormalities are not common in AD. Treatment with either interferon, IV Ig and low-dose cyclosporine have been reportedly beneficial in individual HIES patients but their role in therapy is not yet defined.

 

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