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Therapeutics in Dermatology
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Hypereosinophilic syndrome

5 June 2012, by STAUMONT-SALLÉ D. & DELAPORTE E.

1 - DEFINITION

Hypereosinophilic syndrome (HES) is a rare disorder affecting middle-aged men and defined by Chusid et al in 1975 by the following criteria [1]:

– eosinophil count greater than 1 500/mm3;

– persisting for at least 6 months;

– no known cause (exclusion of known causes of high eosinophil count, i.e. parasite infestation, allergic disorders and blood diseases);

– combined with visceral damage directly related to eosinophilic infiltration of the tissues.

HES is clinically and pathophysiologically extremely heterogeneous. However, it is important to distinguish two subgroups since each requires specific treatment [2]:

– lymphocytic variant (10 to 30 % of cases), whose clinical symptoms and signs are commonly restricted to the skin and are combined with elevated serum IgE levels and polyclonal hypergammaglobulinemia; this form often responds favourably to oral corticosteroids;

– myeloproliferative variant (20 to 30 % of cases), which has a less favourable prognosis owing to the risk of serious major organ complications – especially where the heart is concerned – and the development of corticosteroid resistance making recourse to other treatments, such as tyrosine kinase inhibitors, necessary. 

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