Pain and quality of life evaluation in patients with localized epidermolysis bullosa simplex
Pain and quality of life evaluation in patients with localized epidermolysis bullosa simplex
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DOI：
10.1186/s13023-017-0666-5
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发表时间：
2017-06-28
影响因子：
3.7
通讯作者：
Lacour, Jean-Philippe
中科院分区：
文献类型：
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作者：
Brun, Jennifer;Chiaverini, Christine;Lacour, Jean-Philippe
背景资料：局限性单纯大疱性表皮病（EBS-I）被认为是大疱性表皮病（EB）的最温和形式之一，其水疱局限于手掌和脚底。然而，这些病变可能非常痛苦。该研究的目的是表征患有EBS-I的患者的疼痛并评估其对生活质量（QoL）的影响。通过法国儿科皮肤病学会研究小组和EB患者协会（DEBRA France）联系患者。一名研究人员使用了一个标准化的问卷调查，其中包括验证量表的疼痛和QoL的电话interview.Results：我们包括57例患者（27名儿童）。所有患者均出现疼痛：当出现水泡时，大多数成人（90%）和≥ 8岁儿童（94%）在10 mm视觉模拟量表上的平均疼痛>5，而在更换敷料时，大多数成人（73%）和约一半≥ 8岁儿童（53%）的平均疼痛> 5。年轻患者也有类似的结果。总体而言，75%的患者患有神经性疼痛;对于55%的儿童和73%的成人，疼痛对QOL有中度至重度影响。只有7名患者在更换敷料前使用术前用药，7名患者定期使用口服治疗慢性疼痛。分别有21%和23%的患者使用非甾体类镇痛药和2级镇痛药。这些治疗对神经性疼痛无效。结论：EBS-l患者疼痛频繁、剧烈，具有神经病理特征。这种疼痛治疗不足，影响生活质量。
Background: A localized form of epidermolysis bullosa simplex (EBS-l) is considered one of the mildest forms of epidermolysis bullosa (EB), with blisters limited to the palms and soles. However, these lesions can be very painful. The aim of the study was to characterize pain in patients with EBS-l and evaluate its impact on quality of life (QoL). Patients were contacted via the Research Group of the French Society of Pediatric Dermatology and the association of EB patients (DEBRA France). One investigator used a standardized questionnaire that included validated scales for pain and QoL for a telephone interview.Results: We included 57 patients (27 children). All patients had pain: the mean pain on a 10-mm visual analog scale was >5 for most adults (90%) and children >= 8 years old (94%) when blisters were present and for most adults (73%) and about half of the children >= age 8 (53%) during dressing changes. Similar results were found for younger patients. Overall, 75% of patients had neuropathic pain; for 55% of children and 73% of adults, the pain had a moderate to severe impact on QOL. Only seven patients used premedication before changing dressings and seven regularly used oral treatment for chronic pain. A total of 21% and 23% of patients used non-steroidal antiinflammatory drugs and grade 2 analgesics, respectively. These treatments were not effective for neuropathic pain. Six patients tried 5% lidocaine plasters on their feet, with good efficacy.Conclusions: EBS-l patients have frequent and severe pain with neuropathic characteristics. This pain is undertreated and affects QoL.