Saint-Georges Valentina, Peternel Sandra, Kaštelan Marija, Brajac Ines
Assist. Prof. Sandra Peternel, MD, PhD, Department of Dermatology and Venereology, Clinical Hospital Center Rijeka, Krešimirova 42, 51000 Rijeka, Croatia;
Acta Dermatovenerol Croat. 2018 Jun;26(2):173-178.
The clinical triad of pyoderma gangrenosum (PG), acne and suppurative hidradenitis (HS) has been described under the acronym PASH syndrome and is considered to represent a distinct entity in the group of autoinflammatory diseases. It is a fairly new, only recently recognized disorder with a limited number of reported cases and without defined treatment recommendations. We aimed to summarize currently available data on the use of tumor necrosis factor (TNF) antagonists in the management of PASH syndrome and report on our own experience with the use of adalimumab in a patient presenting with this specific constellation of clinical signs and symptoms. Among the 11 cases identified in the literature, infliximab and adalimumab were the most commonly used agents, both exhibiting favorable effects in the majority of, but not all, patients. This was particularly evident in terms of relatively rapid remission of PG whereas HS lesions seemed to be more resistant to treatment. In our patient, adalimumab monotherapy resulted in a remarkable and sustained remission, although significant improvement of HS lesions was observed only from week 16 of therapy onwards. In summary, TNF antagonists are a promising treatment for PASH; however, conclusions regarding the choice of a specific agent, optimal dosing or use in combination with other treatment modalities cannot yet be drawn.
坏疽性脓皮病（PG）、痤疮和化脓性汗腺炎（HS）的临床三联征已被简称为PASH综合征，并被认为是自身炎症性疾病中的一种独特病症。它是一种相当新的疾病，直到最近才被认识，报告的病例数量有限，且没有明确的治疗建议。我们旨在总结目前关于使用肿瘤坏死因子（TNF）拮抗剂治疗PASH综合征的可用数据，并报告我们使用阿达木单抗治疗一名具有这种特定临床症状和体征组合患者的经验。在文献中确定的11例病例中，英夫利昔单抗和阿达木单抗是最常用的药物，两者在大多数（但不是所有）患者中均显示出良好效果。这在PG相对较快缓解方面尤为明显，而HS病变似乎对治疗更具抗性。在我们的患者中，阿达木单抗单药治疗导致显著且持续的缓解，尽管仅在治疗第16周后才观察到HS病变有显著改善。总之，TNF拮抗剂是治疗PASH的一种有前景的方法；然而，关于选择特定药物、最佳剂量或与其他治疗方式联合使用，目前尚无法得出结论。
