Spira I, Simon M, Keller J
Hautarzt. 1986 Apr;37(4):222-5.
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.
无菌性嗜酸性脓疱病(SEP)于1970年由Ofuji等人首次描述为“嗜酸性脓疱性毛囊炎”,显然是一种新的疾病实体。大多数患者为日本人。迄今为止,仅报道了4例欧洲病例。然而,我们最近观察到一名46岁的希腊男性(欧洲第5例)具有SEP的典型临床特征:反复出现聚集性瘙痒性(毛囊性)丘疹脓疱(直径1 - 2毫米)。皮疹发生在四肢、面部和躯干上略微隆起的红斑区域。组织学检查显示表皮内脓疱含有嗜酸性粒细胞,真皮有中度浸润,在毛囊、皮脂腺和血管周围有单核细胞和嗜酸性粒细胞。除了特征性的临床和组织学特征外,我们的患者还表现出先前描述的所有典型实验室检查结果:脓疱细菌培养阴性、血液嗜酸性粒细胞增多、总IgE升高、回忆抗原皮内试验阴性。此外,外周血中抑制性/细胞毒性T细胞(OKT 8细胞)的比例显著降低,体外淋巴细胞有丝分裂刺激试验为阴性(淋巴细胞转化试验降低)。这些实验室数据类似于特应性疾病的免疫病理学发现。另一方面,无菌性嗜酸性脓疱主要发生在皮脂腺丰富皮肤区域的脂溢性皮炎似乎是SEP的另一个显著特征。然而,在SEP中观察到的类特应性免疫状态与脂溢性皮炎同时出现是否具有致病重要性这一问题仍未解决。除了皮质类固醇和砜类药物外,环氧化酶抑制剂可能会产生良好的治疗效果。在我们的病例中,吲哚美辛带来了快速且持久的治疗益处。