Aleissa Majed, Nicol Perrine, Godeau Marion, Tournier Emilie, de Bellissen Frederic, Robic Marie-Angèle, Livideanu Cristina Bulai, Mazereeuw-Hautier Juliette, Paul Carle
Paul Sabatier University, University Hospitals of Toulouse, Toulouse, France; Department of Dermatology, University Hospitals of Toulouse, Toulouse, France.
Paul Sabatier University, University Hospitals of Toulouse, Toulouse, France; Department of Histopathology, University Hospitals of Toulouse, Toulouse, France.
Case Rep Dermatol. 2017 Jan 19;9(1):6-11. doi: 10.1159/000454876. eCollection 2017 Jan-Apr.
Azathioprine is an immunosuppressive agent used in the treatment of immune-mediated diseases. Azathioprine hypersensitivity syndrome is a rare adverse reaction occurring a few days to weeks after the administration of azathioprine.
CASE 1: A 36-year-old male with ulcerative colitis presented with erythematous plaques, pustules and erosions on the lower back, buttocks and thighs associated with high fever (39°C) 2 weeks after the initiation of azathioprine 100 mg/day. Additional findings included leukocytosis (18.6 g/L) with neutrophilia (11.1 g/L) and elevated C-reactive protein (128 mg/L). Histopathology showed a dense infiltrate of neutrophils in the hair follicles. We increased the dose of prednisone to 1 mg/kg/day (60 mg/day) and azathioprine was discontinued. He had marked improvement within 3 weeks and did not have any relapse with a 1-year follow-up.
CASE 2: A 57-year-old male with ulcerative colitis presented with erythematous plaques and pustules on the lower limbs associated with high fever (40°C) 1 week after the initiation of azathioprine 75 mg/day. Leukocytosis with neutrophilia (13.6 g/L) and elevated C-reactive protein (344 mg/L) were among the laboratory findings. Histopathology showed a dense infiltrate of neutrophils in the hair follicles. The dose of prednisone was increased to 20 mg/day and azathioprine was discontinued, which led to complete remission within 7 days. He did not have any relapse with a 6-month follow-up.
The development of acute neutrophilic dermatitis 2 weeks after the initiation of azathioprine and the complete resolution after its withdrawal were in favor of azathioprine hypersensitivity syndrome. It should not be confused with Sweet syndrome associated with inflammatory bowel disease, as maintenance of azathioprine treatment may lead to life-threatening reactions.
硫唑嘌呤是一种用于治疗免疫介导性疾病的免疫抑制剂。硫唑嘌呤超敏综合征是在使用硫唑嘌呤几天后至几周内发生的罕见不良反应。
病例1：一名36岁患有溃疡性结肠炎的男性，在开始每天服用100毫克硫唑嘌呤2周后，下背部、臀部和大腿出现红斑、脓疱和糜烂，并伴有高热（39°C）。其他检查结果包括白细胞增多（18.6 g/L）伴中性粒细胞增多（11.1 g/L）以及C反应蛋白升高（128 mg/L）。组织病理学显示毛囊中有密集的中性粒细胞浸润。我们将泼尼松剂量增加至1毫克/千克/天（60毫克/天），并停用硫唑嘌呤。他在3周内有明显改善，随访1年未复发。
病例2：一名57岁患有溃疡性结肠炎的男性，在开始每天服用75毫克硫唑嘌呤1周后，下肢出现红斑和脓疱，并伴有高热（40°C）。实验室检查结果包括白细胞增多伴中性粒细胞增多（13.6 g/L）以及C反应蛋白升高（344 mg/L）。组织病理学显示毛囊中有密集的中性粒细胞浸润。泼尼松剂量增加至20毫克/天，并停用硫唑嘌呤，这导致在7天内完全缓解。随访6个月未复发。
在开始使用硫唑嘌呤2周后出现急性中性粒细胞性皮炎，并在停药后完全消退，这支持硫唑嘌呤超敏综合征的诊断。不应将其与炎症性肠病相关的Sweet综合征相混淆，因为继续使用硫唑嘌呤治疗可能会导致危及生命的反应。
