Department of Dermatology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan.
Department of Urology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan.
J Dermatol. 2024 Feb;51(2):287-293. doi: 10.1111/1346-8138.16860. Epub 2023 Jun 19.
Cutis laxa presents as loose redundant skin folds and loss of dermal elastic tissue. Acquired cutis laxa (ACL) is characterized by later onset. It has been reported in association with various kinds of neutrophilic dermatoses, drugs, metabolic disorders, and autoimmune disorders. Acute generalized exanthematous pustulosis (AGEP) is usually classified as a severe cutaneous adverse reaction characterized by T cell-mediated neutrophilic inflammation. We previously reported a mild case of AGEP caused by gemcitabine in a 76-year-old man. Here, we report a case of ACL secondary to AGEP in this patient. He developed AGEP 8 days after gemcitabine administration. Four weeks after beginning chemotherapy, his skin had become atrophic, loose, and darkly pigmented in areas previously affected by AGEP. Histopathological examination revealed edema and perivascular lymphocytic infiltration but no neutrophilic infiltration in the upper dermis. Elastica van Gieson staining showed that the elastic fibers in all layers of the dermis were sparse and shortened. Electron microscopy showed elevated numbers of fibroblasts and altered elastic fibers with irregular surfaces. Finally, he was diagnosed with ACL secondary to AGEP. He was treated with topical corticosteroids and oral antihistamines. Skin atrophy decreased over 3 months. We summarize 36 cases (including our case) with ACL secondary to neutrophilic dermatosis. We discuss these clinical manifestations, causative neutrophilic disorders, treatments, and outcomes. The mean age of patients was 3.5 years. Five patients had an aortic lesion as systemic involvement. The most common causative neutrophilic disorders were Sweet syndrome (24 cases), followed by urticaria-like neutrophilic dermatosis (11 cases). There were no cases of AGEP except for our case. Although treatment for ACL secondary to neutrophilic dermatosis, such as dapsone, oral prednisolone, adalimumab, and plastic surgery were reported, ACL is generally refractory and irreversible. Our patient was considered reversibly cured due to the absence of continuous neutrophil-mediated elastolysis.
皮肤松弛症表现为皮肤褶皱松弛和真皮弹性组织丧失。获得性皮肤松弛症（ACL）的特点是发病较晚。它与各种中性粒细胞性皮肤病、药物、代谢紊乱和自身免疫性疾病有关。急性泛发性发疹性脓疱病（AGEP）通常被归类为一种严重的皮肤不良反应，其特征是 T 细胞介导的中性粒细胞炎症。我们之前报道了一例由吉西他滨引起的 76 岁男性轻度 AGEP 病例。在此，我们报告了一例该患者的 AGEP 继发 ACL 病例。他在吉西他滨给药后 8 天出现 AGEP。开始化疗 4 周后，他以前患有 AGEP 的部位的皮肤变得萎缩、松弛和色素沉着。组织病理学检查显示真皮上层水肿和血管周围淋巴细胞浸润，但无中性粒细胞浸润。弹力纤维 Van Gieson 染色显示所有真皮层的弹性纤维稀疏且缩短。电子显微镜显示成纤维细胞数量增加，弹性纤维表面不规则。最终，他被诊断为 AGEP 继发的 ACL。他接受了局部皮质类固醇和口服抗组胺药治疗。皮肤萎缩在 3 个月内得到改善。我们总结了 36 例（包括我们的病例）中性粒细胞性皮肤病继发 ACL 的病例。我们讨论了这些临床表现、病因中性粒细胞疾病、治疗和结局。患者的平均年龄为 3.5 岁。有 5 例患者有主动脉病变作为全身受累。最常见的病因性中性粒细胞疾病是Sweet 综合征（24 例），其次是荨麻疹样中性粒细胞性皮炎（11 例）。除我们的病例外，没有 AGEP 病例。尽管报道了中性粒细胞性皮肤病继发 ACL 的治疗方法，如氨苯砜、口服泼尼松龙、阿达木单抗和整形手术，但 ACL 通常是难治性和不可逆转的。我们的患者被认为是可逆治愈的，因为不存在持续的中性粒细胞介导的弹性溶解。
