DERMATOLOGICAL MANIFESTATIONS OF IDIOPATHIC HYPEREOSINOPHILIC SYNDROME - AN UNUSUAL OBSERVATION WITH PROTRACTED COURSE AND LITERATURE-REVIEW

The idiopathic hypereosinophilic syndrome is defined by a protracted e osinophilia without defined etiology. Mucocutaneous signs seem to be f requent in this pathological entity but have not been to date the subj ect of a special study. The purpose of this study is to describe a new case of hypereosinophilic syndrome with unusual cutaneous features an d a protracted course. Furthermore, dermatological manifestations in t he reported observations of this syndrome are reviewed. The frequency of mucocutaneous signs in hypereosinophilic syndrome seem to be high b ut can not be assessed with accuracy. They often are the presenting si gns. The most usual manifestation is a diffuse of localized pruritic r ash with an eosinophilic-rich perivascular infiltrate. Attacks of angi oedema as described by Gleich could belong to this spectrum as suggest ed by our observation. The presence of mucocutaneous lesions does not seem to be involved in the general prognosis, rather linked to the sub set of the syndrome. Dermatological abnormalities, even non-specific o nes, must raise the question of the manifestations of an idiopathic hy pereosinophilic syndrome when associated with a protracted eosinophili a.