Kromer Christian, Loewe Emilia, Schaarschmidt Marthe-Lisa, Pinter Andreas, Gerdes Sascha, Celis Daniel, Poortinga Sietske, Wilsmann-Theis Dagmar, Mössner Rotraut
Department of Dermatology, Venereology and Allergology, University Medical Center, Göttingen, Germany.
Department of Dermatology, University Medical Center Mannheim, Mannheim, Germany.
J Dermatol. 2020 Sep;47(9):989-997. doi: 10.1111/1346-8138.15466. Epub 2020 Jul 6.
Acrodermatitis continua of Hallopeau (ACH) is a rare chronic inflammatory skin disease. Treatment is extremely challenging and mostly based on empirics as there is only scarce evidence from case reports and few small case series. In this retrospective study, patients with ACH treated at five university medical centers were analyzed according to patient and disease characteristics and treatment experience. We identified 39 patients with ACH with a mean age of 54.4 years at onset, of whom 22 (56.4%) were female. A total of 115 systemic treatment courses were analyzed with methotrexate as the most common therapy (27.0%). Overall, effectiveness of systemic treatments was low (excellent response rate: 14.8%). Among non-biologics, excellent response was noted in 21.1% (4/19) of treatment courses with methotrexate, followed by acitretin (13.3%; 2/15). Among biologics, guselkumab (excellent response: 100%; 2/2), secukinumab (excellent response: 42.9%; 3/7) and adalimumab (excellent response: 20.0%; 2/10) were most efficacious. The median drug survival was 7.0 months and did not differ significantly between the subgroup of non-biologic and biologic therapies. To our knowledge, this is the largest case series in ACH investigating patient characteristics and treatment outcomes. Based on our treatment experience, we suggest a treatment algorithm starting with acitretin or methotrexate as first-line therapy, followed by biologics. Cyclosporin may be used for short-term control. However, none of the applied systemic therapies yielded satisfying efficacy in our cohort. In patients with primary non-response, switch of treatment should be evaluated timely on an individual basis, considering possible irreversible disease complications such as nail loss. More research with prospective design is needed to further evaluate traditional and also particularly newer antipsoriatic drugs in ACH.
哈洛佩奥连续性肢端皮炎（ACH）是一种罕见的慢性炎症性皮肤病。治疗极具挑战性，且大多基于经验，因为病例报告和少数小病例系列提供的证据非常有限。在这项回顾性研究中，对在五所大学医学中心接受治疗的ACH患者，根据患者和疾病特征以及治疗经验进行了分析。我们确定了39例ACH患者，发病时的平均年龄为54.4岁，其中22例（56.4%）为女性。共分析了115个系统治疗疗程，甲氨蝶呤是最常用的治疗方法（27.0%）。总体而言，系统治疗的有效性较低（极佳缓解率：14.8%）。在非生物制剂中，甲氨蝶呤治疗疗程的极佳缓解率为21.1%（4/19），其次是阿维A（13.3%；2/15）。在生物制剂中，古塞库单抗（极佳缓解率：100%；2/2）、司库奇尤单抗（极佳缓解率：42.9%；3/7）和阿达木单抗（极佳缓解率：20.0%；2/10）最为有效。药物的中位生存期为7.0个月，非生物制剂和生物制剂治疗亚组之间无显著差异。据我们所知，这是ACH领域调查患者特征和治疗结果的最大病例系列。根据我们的治疗经验，我们建议采用以阿维A或甲氨蝶呤作为一线治疗，随后使用生物制剂的治疗方案。环孢素可用于短期控制。然而，在我们的队列中，所应用的系统治疗方法均未产生令人满意的疗效。对于初始无反应的患者，应根据个体情况及时评估治疗方案的转换，同时考虑可能出现的不可逆疾病并发症，如指甲脱落。需要更多前瞻性设计的研究来进一步评估传统以及特别是新型抗银屑病药物在ACH中的疗效。
