背景:坏疽性脓皮病,痤疮,化脓性汗腺炎(PASH)综合征是一种罕见的疾病,其特征是所有三种皮肤病的临床特征。PASH综合征的管理是困难的,在治疗指南上没有达成共识。由于PASH综合征会增加发病率并对生活质量产生不利影响,需要更好地描述有效的治疗方法。
方法:进行了一项回顾性队列研究,以确定2015年至2021年在俄亥俄州立大学韦克斯纳医学中心治疗的所有坏疽性脓皮病(PG)患者。PG诊断通过PARACELSUS评分确认。随后的图表审查确定了8例合并化脓性汗腺炎(HS)和痤疮的患者,这些患者在临床上被诊断为PASH综合征。
结果:根据我们机构的临床表现,有8例患者被临床诊断为PASH综合征。七名患者在就诊前未能接受某种类型的药物治疗,包括外用皮质类固醇,口服皮质类固醇,口服抗生素,和生物制品。一名患者还在外部机构尝试了手术引流。6名患者接受了生物制剂的有效治疗,通常与其他疗法相结合。一名患者在诊断和治疗潜在的血液系统恶性肿瘤后,皮肤病变得到改善。
结论:生物制剂联合糖皮质激素和/或抗生素的医疗管理在大多数患者的管理中是有效的。在难治性病例中,应优先诊断和治疗基础疾病。如果检查结果是否定的,可以考虑手术治疗。需要对更多患者进行进一步调查,以制定PASH综合征的管理指南。
BACKGROUND: Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) syndrome is a rare condition characterized by clinical features of all three dermatologic conditions. The management of PASH syndrome is difficult, with no consensus on treatment guidelines. Since PASH syndrome can increase morbidity and adversely impact quality of life, better characterization of effective therapies is needed.
METHODS: A retrospective cohort study was conducted to identify all patients with pyoderma gangrenosum (PG) treated at The Ohio State University Wexner Medical Center between 2015 and 2021. PG diagnosis was confirmed via PARACELSUS score. Subsequent chart review identified eight patients with concomitant hidradenitis suppurativa (HS) and acne who were clinically diagnosed with PASH syndrome.
RESULTS: Eight patients were clinically diagnosed with PASH syndrome based on their clinical presentation at our institution. Seven patients had failed some type of medical therapy prior to presentation, including topical corticosteroids, oral corticosteroids, oral antibiotics, and biologics. One patient had also tried surgical drainage at an outside institution. Six patients were effectively treated with biologics, usually in combination with other therapies. One patient experienced improvement of her skin lesions after diagnosis and treatment of her underlying hematologic malignancy.
CONCLUSIONS: Medical management with biologics in combination with corticosteroids and/or antibiotics was effective in the management of most patients. Diagnosis and treatment of an underlying condition should be prioritized in refractory cases. If workup is negative, surgical management may be considered. Further investigation with a greater number of patients is required to develop management guidelines for PASH syndrome.