获得性穿孔性皮肤病伴并发蜂窝织炎及截肢一例血液透析患者。

Clinical Nephrology. Case Studies Pub Date : 2021-03-11 eCollection Date: 2021-01-01 DOI:10.5414/CNCS110297
Ana Domingos, Roberto Calças, Eduarda Carias, Joana Vidinha, Anabela Malho Guedes, Viriato Santos, Patrick Agostini, Francisco Ildefonso Mendonça, Pedro Leão Neves
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引用次数: 1

摘要

与慢性肾脏疾病(CKD)相关的皮肤表现是常见的,并且与高发病率相关。获得性穿孔性皮肤病(APD)多见于糖尿病或CKD患者,即接受血液透析的患者。病例报告:一名58岁男性2型糖尿病患者,长期使用胰岛素,有微血管和大血管并发症,维持血液透析5年,左前臂、双手和双截肢残端疼痛、瘙痒、脐丘疹和一些点状坏死病变1周。没有感染或血管改变的证据,患者对局部皮质类固醇的初始疗程没有反应。这些病变迅速发展为更大的、新生的坏死性损伤,疼痛加重,左侧皮肤剧烈脱皮,躯干出现类似病变,需要住院治疗。怀疑有钙化反应和APD。进行皮肤活检并开始定向治疗,包括分析性硫代硫酸钠。组织学结果与APD一致,也排除了提示血管炎或钙化反应的结果。不久之后,难以治疗的左手和前臂蜂窝织炎进展为严重缺血和截肢。微生物学分析显示粘质沙雷菌为病原菌。讨论:据我们所知,以前没有报道过apd相关蜂窝织炎的病例。其治疗尤其具有挑战性,因为病变可能持续存在并复发,并且可能形成慢性疤痕。粘质葡萄球菌表现为一种机会性和难以治疗的病原体,使预后复杂化。结论:APD可能与严重血管病变患者的蜂窝织炎及其所有并发症有关。认识到APD的这一并发症,早期转诊和积极治疗可能会改善这类患者的预后和生活质量。
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Acquired perforating dermatosis with associated complicated cellulitis and amputation in a hemodialysis patient.

Introduction: Cutaneous manifestations related to chronic kidney disease (CKD) are common and associated with high morbidity. Acquired perforating dermatosis (APD) occurs mostly in diabetic or CKD patients, namely those undergoing hemodialysis.

Case report: A 58-year-old male with type 2 diabetes, with long-term insulin use, several microvascular and macrovascular complications, and on maintenance hemodialysis for 5 years presented with a 1-week history of painful, pruritic, umbilicated papules and some punctiform necrotic lesions on his left forearm, both hands, and both amputation stumps. There was no evidence of infection or vascular alterations, and the patient was not responsive to an initial course of topical corticosteroid. These lesions rapidly evolved to larger, coalescent necrotic injuries, with aggravated pain, intense left-hand skin peeling, and the appearance of similar lesions in the trunk, requiring hospital admission. Calciphylaxis and APD were suspected. Skin biopsy was performed and directed treatment initiated, including intradialytic sodium thiosulfate. Histology findings were compatible with APD and also excluded findings suggestive of vasculitis or calciphylaxis. Soon after, difficult-to-treat cellulitis of the left hand and forearm progressed to critical ischemia and amputation. Microbiology analysis revealed Serratia marcescens as the causative agent.

Discussion: To our knowledge, there are no previously described cases of APD-related cellulitis. Its treatment can be particularly challenging, as lesions can persist and relapse, and chronic scars can develop. S. marcescens behaves as an opportunistic and difficult-to-treat pathogen, complicating the prognosis.

Conclusion: APD can be associated with cellulitis and all of its complications in patients with underlying severe vasculopathy. Awareness of this complication in APD with early referral and aggressive treatment might improve the outcomes and quality of life of such patients.

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