Purple glove syndrome

Purple glove syndrome (PGS) is a poorly understood skin disease in which the extremities become swollen, discoloured and painful, most commonly occurring in elderly patients receiving multiple large intravenous doses of the antiepileptic drug phenytoin紫手套综合征（PGS）是一种病因未明的皮肤疾病，肢体变得肿胀、变色和疼痛，最常见于接受多次大剂量静脉注射抗癫痫药物苯妥英的老年患者。

Wikidata maps the condition to Q7261474 with UMLS C1141936 as a cross-reference, and the condition is classified under dermatology and drug adverse effectsWikidata 将该病症映射到 Q7261474，UMLS C1141936 作为交叉引用，该病症归类于皮肤科和药物不良反应。

The syndrome was first described in 1989 and has an estimated incidence of 1.7 to 5.9 per cent among patients receiving intravenous phenytoin该综合征在1989年首次被描述，在接受静脉苯妥英的患者中估计发病率为1.7%至5.9%。

Terminology and classification

• Purple glove syndrome is classified as a cutaneous adverse drug reaction, specifically a localized drug reaction related to intravenous phenytoin administration紫手套综合征归类为皮肤药物不良反应，特别是与静脉苯妥英给药相关的局限性药物反应。^[1]
• Wikidata maps the condition to Q7261474, with UMLS C1141936 as an additional identifierWikidata 将该病症映射到 Q7261474，UMLS C1141936 作为额外标识符。
• The syndrome is distinct from extravasation, which involves leakage of drug into surrounding tissue, although PGS can occur with or without extravasation该综合征与外渗不同，后者涉及药物漏入周围组织，尽管 PGS 可伴有或不伴有外渗发生。^[1]
• Purple glove syndrome is also known as phenytoin-induced peripheral edema and vascular occlusion syndrome紫手套综合征也称苯妥英诱导的周围水肿和血管闭塞综合征。^[1]
• The condition is classified under dermatology and pharmacology in clinical taxonomies在临床分类中，该病症归类于皮肤科和药理学。^[1]
• The term purple glove reflects the characteristic purple to black discoloration of the extremities, particularly the hands紫手套这一术语反映了肢体（特别是手部）特征性的紫色至黑色变色。^[1]

History

• Purple glove syndrome was first described in 1989 by Osterer and colleagues, who reported a case of limb swelling and discoloration following intravenous phenytoin紫手套综合征在1989年由 Osterer 等首次描述，他们报告了一例静脉苯妥英后肢体肿胀和变色的病例。^[1]
• The condition was initially confused with extravasation and allergic reactions, but was recognized as a distinct entity with characteristic clinical features该病症最初与外渗和过敏反应混淆，但后来被认识为具有特征性临床特征的独立实体。^[1]
• Additional case reports in the 1990s established the association with intravenous phenytoin and expanded the clinical spectrum1990年代的额外病例报告确立了与静脉苯妥英的关联并扩展了临床谱系。^[1]
• The condition was recognized as a rare but serious complication of phenytoin therapy, requiring discontinuation of the drug该病症被认识到是苯妥英治疗的罕见但严重的并发症，需要停药。^[1]
• The mechanism remains poorly understood, with several hypotheses proposed including direct drug toxicity and hypersensitivity reaction机制仍然知之甚少，提出了几种假设，包括直接药物毒性和过敏反应。^[1]
• Recent reports have described cases with fosphenytoin, a phenytoin prodrug, suggesting the reaction may be related to the phenytoin molecule itself最近的报告描述了磷苯妥英（苯妥英前药）的病例，表明该反应可能与苯妥英分子本身有关。^[1]

Epidemiology

• The incidence of purple glove syndrome among patients receiving intravenous phenytoin is estimated to be 1.7 to 5.9 per cent在接受静脉苯妥英的患者中，紫手套综合征的发病率估计为1.7%至5.9%。^[1]
• The condition is more common in elderly patients and those receiving multiple large doses of intravenous phenytoin该病症在老年患者和接受多次大剂量静脉苯妥英的患者中更常见。^[1]
• Patients with peripheral vascular disease or limited venous access may be at higher risk有周围血管疾病或静脉通路有限的患者可能风险更高。^[1]
• The condition typically develops within hours to days after the start of intravenous phenytoin therapy该病症通常在静脉苯妥英治疗开始后数小时至数天内发展。^[1]
• No gender predilection has been reported未报告性别偏好。^[1]

Pathophysiology

• The exact mechanism of purple glove syndrome is unknown, but it is believed to involve a combination of direct drug toxicity and inflammatory response紫手套综合征的确切机制未知，但被认为涉及直接药物毒性和炎症反应的组合。^[1]
• One hypothesis suggests that phenytoin causes direct endothelial damage and vasculitis, leading to vascular occlusion and tissue ischaemia一种假设认为苯妥英引起直接内皮损伤和血管炎，导致血管闭塞和组织缺血。^[1]
• Another hypothesis proposes a hypersensitivity reaction to phenytoin, with immune-mediated damage to blood vessels and surrounding tissue另一种假设提出了对苯妥英的过敏反应，伴有免疫介导的血管和周围组织损伤。^[1]
• The syndrome may involve increased vascular permeability leading to oedema and extravasation of blood products into the tissues该综合征可能涉及血管通透性增加，导致水肿和血液成分渗入组织。^[1]
• The discoloration is likely due to haemorrhage and necrosis in the affected tissues变色可能是由于受累组织中的出血和坏死。^[1]
• The condition can progress to compartment syndrome if the swelling causes increased pressure within the muscle compartments如果肿胀导致肌肉间隔内压力增加，该病症可进展为骨筋膜室综合征。^[1]

Clinical features

• The characteristic presentation is swelling, discolouration and pain in the extremities, particularly the hands and fingers特征性表现是肢体（特别是手和手指）的肿胀、变色和疼痛。^[1]
• The discoloration ranges from purple to black and is typically distal to the site of intravenous injection变色从紫色到黑色不等，通常位于静脉注射部位的远端。^[1]
• The swelling can be severe and may involve the entire limb, extending beyond the injection site肿胀可能严重，可涉及整个肢体，超出注射部位。^[1]
• Pain is often severe and may be out of proportion to the visible changes疼痛通常严重，可能与可见变化不成比例。^[1]
• Complications include compartment syndrome, tissue necrosis, amputation and rarely, death并发症包括骨筋膜室综合征、组织坏死、截肢，罕见情况下死亡。^[1]
• The condition is typically unilateral but can be bilateral in patients receiving infusions to multiple sites该病症通常为单侧，但在多个部位接受输液的患者中可为双侧。^[1]

Diagnosis

• Diagnosis is based on the clinical presentation of swelling, discoloration and pain following intravenous phenytoin administration诊断基于静脉苯妥英给药后肿胀、变色和疼痛的临床表现。^[1]
• The condition should be distinguished from extravasation, which typically involves the injection site directly and has a different distribution该病症应与外渗区分，后者通常直接涉及注射部位，分布不同。^[1]
• Measurement of compartment pressures may be needed to diagnose compartment syndrome可能需要测量筋膜室压力以诊断骨筋膜室综合征。^[1]
• The differential diagnosis includes extravasation, allergic reaction, deep vein thrombosis, arterial occlusion and necrotizing fasciitis鉴别诊断包括外渗、过敏反应、深静脉血栓、动脉闭塞和坏死性筋膜炎。^[1]
• Laboratory testing including complete blood count and inflammatory markers may show non-specific changes包括全血细胞计数和炎症标志物在内的实验室检查可能显示非特异性变化。^[1]
• Imaging studies including Doppler ultrasound can help exclude vascular occlusion or deep vein thrombosis包括多普勒超声在内的影像学研究有助于排除血管闭塞或深静脉血栓。^[1]

Management

• The first step in management is immediate discontinuation of intravenous phenytoin管理的第一步是立即停止静脉苯妥英。^[1]
• Fosphenytoin can be substituted as an alternative if antiepileptic treatment is still needed如果仍需抗癫痫治疗，可用磷苯妥英作为替代。^[1]
• Supportive care includes limb elevation, warm compresses and analgesics支持性护理包括肢体抬高、热敷和镇痛药。^[1]
• If compartment syndrome develops, surgical fasciotomy is required to relieve pressure如果发生骨筋膜室综合征，需要手术筋膜切开术以减压。^[1]
• Severe cases may require amputation if tissue necrosis occurs如果发生组织坏死，严重病例可能需要截肢。^[1]
• The prognosis depends on the severity and promptness of intervention, with most cases resolving after phenytoin discontinuation预后取决于严重程度和干预的及时性，大多数病例在停用苯妥英后消退。^[1]

Research and controversies

• The exact mechanism of purple glove syndrome remains unknown, with several hypotheses proposed but none confirmed紫手套综合征的确切机制仍然未知，提出了几种假设但均未确认。^[1]
• The optimal management approach, particularly regarding the use of corticosteroids, is not well established最佳管理方法（特别是关于皮质类固醇的使用）尚未充分确立。^[1]
• Several clinical trials are registered on ClinicalTrials.gov for phenytoin alternatives and adverse effect managementClinicalTrials.gov 上注册了多项苯妥英替代品和不良反应管理的临床试验。^[1]
• The relationship between purple glove syndrome and other phenytoin adverse reactions is not well characterized紫手套综合征与其他苯妥英不良反应之间的关系尚未充分描述。^[1]
• The long-term outcomes and recurrence rates after recovery are not well studied恢复后的长期结局和复发率尚未充分研究。^[1]

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