Valenzuela-Ubiña Sandra, Jiménez-Gallo David, Russo-de la Torre Francisco, Linares-Barrios Mario
Unidad de Gestión Clínica de Dermatología Médico-Quirúrgica y Venereología, Hospital Universitario Puerta del Mar, Cádiz, Spain.
Consulta Dermatológica Dr. Russo, Algeciras, Cádiz, Spain.
Dermatol Ther. 2020 Jul;33(4):e13692. doi: 10.1111/dth.13692. Epub 2020 Jun 28.
Elastosis perforans serpiginosa (EPS) is a rare condition within the group of perforating dermatoses. It is characterized by the synthesis of anomalous elastic fibers that are eliminated through perforating channels (transepidermal elimination). It is classified into three subtypes. One of them is drug-induced by prolonged treatment with d-penicillamine. This drug is a heavy metal chelator used to treat diseases such as rheumatoid arthritis, cystinuria, and Wilson's disease. Years of treatment with d-penicillamine at high doses are required for developing EPS, with occasional slow regression after drug withdrawal. There is no established treatment for EPS, with described cases using various treatment options such as corticoids, retinoids, tazarotene, cryotherapy, imiquimod, photodynamic therapy, electrosurgery, and CO2 laser among others with inconsistent results. We present a case of EPS induced by d-penicillamine with favorable response to cyclosporine and allopurinol in a patient with a history of Wilson's disease since childhood. They maybe considered as possible therapeutic options not described so far for an entity with variable response to current treatments. We highlight the extensive involvement of the case with progression, despite the suspension of d-penicillamine and failure to previous treatments with photodynamic therapy and retinoids.
匐行性穿通性弹力纤维病(EPS)是穿通性皮肤病中的一种罕见病症。其特征是合成异常弹性纤维,并通过穿通通道(经表皮排出)将其清除。它分为三个亚型。其中之一是由长期使用d-青霉胺治疗引起的药物性亚型。这种药物是一种重金属螯合剂,用于治疗类风湿性关节炎、胱氨酸尿症和威尔逊氏病等疾病。使用高剂量d-青霉胺治疗数年才会发展为EPS,停药后偶尔会缓慢消退。目前尚无针对EPS的确立治疗方法,文献报道的病例采用了各种治疗方案,如使用皮质类固醇、维甲酸、他扎罗汀、冷冻疗法、咪喹莫特、光动力疗法、电外科手术和二氧化碳激光等,但结果并不一致。我们报告一例由d-青霉胺引起的EPS病例,该病例中一名自幼患有威尔逊氏病的患者对环孢素和别嘌醇治疗反应良好。对于目前治疗反应各异的这种病症,它们可能被视为迄今为止尚未描述过的可能治疗选择。我们强调该病例尽管停用了d-青霉胺且先前的光动力疗法和维甲酸治疗无效,但仍广泛进展。