可爱的松弛表现为松弛的多余皮肤褶皱和真皮弹性组织的损失。获得性皮肤松弛(ACL)的特征是发病较晚。据报道,它与各种嗜中性皮肤病有关,毒品,代谢紊乱,和自身免疫性疾病。急性全身性发疹性脓疱病(AGEP)通常被归类为以T细胞介导的中性粒细胞炎症为特征的严重皮肤不良反应。我们先前报道了一名76岁男性由吉西他滨引起的AGEP轻度病例。这里,我们报告了一例AGEP继发ACL的病例.他在吉西他滨给药后8天出现AGEP。开始化疗四周后,他的皮肤萎缩了,松散,以前受AGEP影响的区域色素沉着。组织病理学检查显示水肿和血管周围淋巴细胞浸润,但上真皮无中性粒细胞浸润。ElasticavanGieson染色显示真皮所有层中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,弹性纤维改变,表面不规则。最后,他被诊断为AGEP继发ACL.他接受了局部皮质类固醇和口服抗组胺药治疗。皮肤萎缩减少超过3个月。我们总结了36例(包括我们的病例)继发于中性粒细胞性皮肤病的ACL。我们讨论这些临床表现,致病性嗜中性粒细胞疾病,治疗,和结果。患者的平均年龄为3.5岁。五名患者因全身受累而发生主动脉病变。最常见的致病性嗜中性粒细胞疾病是Sweet综合征(24例),其次是荨麻疹样中性粒细胞性皮肤病(11例)。除了我们的病例,没有AGEP病例。虽然治疗继发于嗜中性皮肤病的ACL,如氨苯砜,口服泼尼松龙,阿达木单抗,据报道,整形手术,ACL通常是难熔的和不可逆的。由于没有持续的中性粒细胞介导的弹性溶解,我们的患者被认为是可逆治愈的。
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.