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[Sterile eosinophilic pustulosis]
Abstract:
Sterile eosinophilic pustulosis (SEP) first described by Ofuji et al. in 1970 as "eosinophilic pustular folliculitis," is obviously a new entity. The majority of patients are Japanese. So far only four European cases have been reported. However, we have recently observed a 46-year-old Greek male (the fifth case in Europe) with the typical clinical features of SEP: repeated eruptions of aggregated pruritic (follicular) papulopustules (1-2 mm in diameter). The eruptions developed in slightly elevated erythematous patches localized on the extremities, face, and trunk. Histologic examination revealed intraepidermal pustules containing eosinophils and moderate dermal infiltrates with mononuclear cells and eosinophils around follicles, sebaceous glands, and vessels. In addition to the characteristic clinical and histological features, our patient showed all the typical laboratory findings previously described: negative bacterial cultures from the pustules, blood eosinophilia, increased total IgE, negative reactions to intradermal tests of recall agents. Furthermore, the rate of suppressor/cytotoxic T-cells (OKT 8-cells) in peripheral blood was significantly diminished, and mitogenic stimulation of lymphocytes in vitro was negative (decreased LTT). These laboratory data resemble the immunopathological findings in atopic diseases. On the other hand, seborrheic skin with sterile eosinophilic pustules predominantly occurring in skin areas rich in sebaceous glands seems to be another remarkable sign of SEP. The question of whether the co-incidence of atopylike immunological constellation and seborrheic skin observed in SEP has pathogenetic importance, however remains open. Apart from corticosteroids and sulfones, a favorable therapeutic effect may be obtained by inhibitors of cyclooxygenase. In our case indomethacin caused a quick and lasting therapeutical benefit.