Guerin Clemence, Beylot-Barry Marie, Frouin Eric, Hainaut Ewa, Masson Regnault Marie
Dermatology, Centre Hospitalier Universitaire de Poitiers, Poitiers, FRA.
Dermatology, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, FRA.
Cureus. 2021 Aug 13;13(8):e17147. doi: 10.7759/cureus.17147. eCollection 2021 Aug.
Subcorneal pustular dermatosis (SPD), also known as Sneddon-Wilkinson disease, is a skin condition for which treatments are poorly codified. Anti-tumor necrosis factor alpha (TNFα) efficacy has been reported in multidrug-resistant SPD, as in our two cases. In the first case, an 83-year-old woman was monitored for SPD, associated with monoclonal IgA gammopathy. After multiple-line treatment failure, infliximab (5mg/kg) led to clinical improvement, noted few days following the first injection, and with complete remission at one month. At 12 months, the patient relapsed and concomitant serum anti-TNFα antibodies were found. A switch to adalimumab led to complete remission in three months with a follow-up of six months. In the second case, a 62-year-old woman was monitored for SPD associated with monoclonal IgA gammopathy recalcitrant to different lines of treatment. Treatment with adalimumab (40mg every two weeks) in combination with dapsone led to significant improvement after two injections. Five months later, she relapsed. It was then decided to reduce the interval between injections to once a week. Rapid improvement was achieved in one month allowing resumption of the original frequency of the injection without relapse after 20 months of follow-up. In conclusion, our cases confirm the previously reported efficacy of anti-TNFα in resistant SPD. They also highlight a risk of secondary loss of efficacy, reinforced by the literature data. Substitution of another TNFα blocker or shortening of interval between injections provided a renewal in response to treatment.
角层下脓疱性皮肤病(SPD),也称为斯内登 - 威尔金森病,是一种治疗方法缺乏明确规范的皮肤病。如我们的两个病例所示,抗肿瘤坏死因子α(TNFα)在耐多药的SPD中已显示出疗效。在第一个病例中,一名83岁女性因SPD接受监测,该疾病与单克隆IgA丙种球蛋白病相关。在多线治疗失败后,英夫利昔单抗(5mg/kg)使病情得到临床改善,首次注射后几天即有改善迹象,一个月时完全缓解。12个月时患者复发,检测到血清抗TNFα抗体。换用阿达木单抗后,三个月时完全缓解,并进行了六个月的随访。在第二个病例中,一名62岁女性因与单克隆IgA丙种球蛋白病相关的SPD接受监测,该病对不同治疗方案均耐药。阿达木单抗(每两周40mg)联合氨苯砜治疗,注射两次后病情显著改善。五个月后复发。随后决定将注射间隔缩短至每周一次。一个月内病情迅速改善,之后恢复原来的注射频率,随访20个月未复发。总之,我们的病例证实了先前报道的抗TNFα在耐药SPD中的疗效。这些病例还突出了继发疗效丧失的风险,文献数据也强化了这一点。换用另一种TNFα阻滞剂或缩短注射间隔可使治疗反应恢复。