Copaescu Ana-Maria, Bouffard Danielle, Masse Marie-Soleil
1Allergy and Immunology Department, Centre Hospitalier de l'Université de Montréal (CHUM), 264 Boulevard René-Lévesque E, Montréal, QC H2X 1P1 Canada.
2Pathology Department, Centre Hospitalier de l'Université de Montréal (CHUM), Montréal, Canada.
Allergy Asthma Clin Immunol. 2020 Feb 4;16:9. doi: 10.1186/s13223-020-0407-5. eCollection 2020.
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.
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.
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.
急性泛发性脓疱性皮病(AGEP)和中毒性表皮坏死松解症(TEN)是严重的皮肤不良反应。这些罕见病症在临床表现、病理特征、治疗及预后方面存在差异,但已有重叠情况的描述,这意味着临床管理具有挑战性。
我们描述了一例77岁因复杂性胆管炎接受治疗的患者,可能因β-内酰胺类药物继发TEN与AGEP重叠的病例。我们回顾了这两种病症的诊断与管理。TEN的诊断依据最初的临床表现,包括严重的血流动力学不稳定、皮肤剥脱、尼氏征阳性及黏膜受累。然而,皮肤活检以及皮肤病变的迅速改善对AGEP具有鉴别意义。这表明存在重叠表现。遗憾的是,患者拒绝进行过敏检查以找出致病药物。整合了医学照片、恰当的体格检查及组织病理学结果。
尽管临床特征提示TEN诊断,但组织病理学确诊为AGEP,从而表明这两种病症可能存在临床病理重叠,这在医学文献中鲜有描述。据我们所知,这是少数几例可能继发于哌拉西林他唑巴坦的TEN-AGEP重叠病例之一。了解这些病症的免疫学影响有助于我们更好地鉴别和管理这些严重反应。