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Acute generalized exanthematous pustulosis simulating toxic epidermal necrolysis: case presentation and literature review.
Allergy, Asthma & Clinical Immunology ( IF 2.7 ) Pub Date : 2020-02-04 , DOI: 10.1186/s13223-020-0407-5
Ana-Maria Copaescu 1 , Danielle Bouffard 2 , Marie-Soleil Masse 1
Affiliation  

Background Acute generalized exanthematous pustulosis (AGEP) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions. These rare conditions differ in clinical presentation, pathological features, treatment and prognosis, but overlap has been described implying a challenging clinical management. Case presentation We describe a case of overlap between TEN and AGEP probably secondary to beta-lactams in a 77-year-old patient treated for a complicated cholangitis. We review the diagnosis and the management of these two conditions. The diagnosis of TEN was suggested by the initial clinical presentation with severe hemodynamic instability, skin detachment, positive Nikolsky sign and mucosal involvement. However, the skin biopsy as well as the rapid improvement of the skin lesions were discriminative for AGEP. This indicated an overlap presentation. Unfortunately, the patient refused allergy investigations in order to find the culprit drug. Medical photographs, proper physical examination and histopathological results are integrated. Conclusion Despite clinical features indicating a diagnosis of TEN, histopathology was conclusive for AGEP thus indicating a possible clinical-pathological overlap between the two conditions, a scarcely described situation in the medical literature. To our knowledge, this is one of the few cases that portrays a TEN-AGEP overlap probably secondary to Piperacillin Tazobactam. Understanding the immunological implications of these conditions can help us better distinguish and manage these severe reactions.

中文翻译:

模拟中毒性表皮坏死松解症的急性泛发性发疹性脓疱病:病例介绍和文献回顾。

背景急性泛发性发疹性脓疱病(AGEP)和中毒性表皮坏死松解症(TEN)是严重的皮肤不良反应。这些罕见的情况在临床表现、病理特征、治疗和预后方面有所不同,但已经描述了重叠,这意味着临床管理具有挑战性。病例介绍 我们描述了一名 77 岁患有复杂性胆管炎的患者中 TEN 和 AGEP 之间可能继发于 β-内酰胺的重叠病例。我们回顾了这两种情况的诊断和管理。最初的临床表现表明严重的血流动力学不稳定、皮肤脱离、Nikolsky 征阳性和黏膜受累,提示了 TEN 的诊断。然而,皮肤活检以及皮损的快速改善对AGEP具有鉴别性。这表明存在重叠展示。不幸的是,患者拒绝过敏调查以找到罪魁祸首药物。综合了医学照片、适当的身体检查和组织病理学结果。结论 尽管临床特征表明诊断为 TEN,但组织病理学对 AGEP 是决定性的,因此表明这两种情况之间可能存在临床-病理学重叠,医学文献中很少描述这种情况。据我们所知,这是描述 TEN-AGEP 重叠可能继发于哌拉西林他唑巴坦的少数病例之一。了解这些疾病的免疫学意义可以帮助我们更好地区分和管理这些严重反应。综合了医学照片、适当的身体检查和组织病理学结果。结论 尽管临床特征表明诊断为 TEN,但组织病理学对 AGEP 是决定性的,因此表明这两种情况之间可能存在临床-病理学重叠,医学文献中很少描述这种情况。据我们所知,这是描述 TEN-AGEP 重叠可能继发于哌拉西林他唑巴坦的少数病例之一。了解这些疾病的免疫学意义可以帮助我们更好地区分和管理这些严重反应。综合了医学照片、适当的身体检查和组织病理学结果。结论 尽管临床特征表明诊断为 TEN,但组织病理学对 AGEP 是决定性的,因此表明这两种情况之间可能存在临床-病理学重叠,医学文献中很少描述这种情况。据我们所知,这是描述 TEN-AGEP 重叠可能继发于哌拉西林他唑巴坦的少数病例之一。了解这些疾病的免疫学意义可以帮助我们更好地区分和管理这些严重反应。组织病理学对AGEP是决定性的,因此表明这两种情况之间可能存在临床-病理学重叠,医学文献中很少描述这种情况。据我们所知,这是描述 TEN-AGEP 重叠可能继发于哌拉西林他唑巴坦的少数病例之一。了解这些疾病的免疫学意义可以帮助我们更好地区分和管理这些严重反应。组织病理学对AGEP是决定性的,因此表明这两种情况之间可能存在临床-病理学重叠,医学文献中很少描述这种情况。据我们所知,这是描述 TEN-AGEP 重叠可能继发于哌拉西林他唑巴坦的少数病例之一。了解这些疾病的免疫学意义可以帮助我们更好地区分和管理这些严重反应。
更新日期:2020-04-22
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