关键词: Rowell syndrome azathioprine cutaneous lupus erythematous drug eruption erythema multiforme-like drug eruption fixed drug eruption hydroxychloroquine systemic lupus erythematous

来  源:   DOI:10.1177/10668969241234316

Abstract:
Background. Fixed drug eruption and Rowell syndrome stand as intriguing entities with overlapping clinical and pathological features. Case Presentation. A 32-year-old female patient presented with a tender and pruritic rash on the left upper chest for 3 days. Clinical examination revealed a flaring rash on the chest, under her left eye, tongue, and lips. The patient had a significant past medical history of systemic lupus erythematous with positive (ANA, Sm, dsDNA, ribosomalP, RNP) antibodies, hypocomplementemia, inflammatory arthritis, discoid lupus, leukopenia, thrombocytopenia, and immune thrombocytopenic purpura, and avascular necrosis affecting both hips and her right knee. At the time of presentation, the patient was on azathioprine 150 mg daily and hydroxychloroquine 200 mg twice daily. Skin biopsy of the left upper chest revealed interface dermatitis with necrotic keratinocytes at the dermal-epidermal junction. Superficial and, in some areas, deep perivascular and peri adnexal lymphocytic infiltrates were observed, accompanied by eosinophils. CD123 staining highlighted 16% of the inflammatory cells. Direct Immunofluorescence for IgG, IgA, IgM, C3, and fibrinogen revealed positive linear basement membrane staining for IgG and fibrinogen, with no significant staining for the remaining immunoreactants. Considering the patient\'s history of medicine usage, and negative SS-A and SS-B antibody, a fixed drug eruption was favored. Discussion. This article discusses the clinical presentations, pathophysiological mechanisms, and diagnostic criteria for fixed drug eruption and Rowell syndrome. Conclusion. Awareness of the distinct clinical and histopathologic features of fixed drug eruption and Rowell syndrome, particularly when sharing cutaneous manifestations, underscores the importance of a comprehensive diagnostic approach and laboratory testing.
摘要:
背景。固定药疹和Rowell综合征是具有重叠的临床和病理特征的有趣实体。案例介绍。一名32岁的女性患者在左上胸部出现疼痛和瘙痒性皮疹3天。临床检查发现胸部有皮疹,在她的左眼下,舌头,和嘴唇。患者有明显的系统性红斑狼疮阳性病史(ANA,Sm,dsDNA,核糖体P,RNP)抗体,低补体血症,炎性关节炎,盘状狼疮,白细胞减少症,血小板减少症,和免疫性血小板减少性紫癜,血管坏死影响臀部和右膝.在介绍的时候,患者接受硫唑嘌呤150mg/日和羟氯喹200mg/日2次.左上胸部的皮肤活检显示界面皮炎,真皮-表皮交界处有坏死的角质形成细胞。浅层和,在某些地区,深血管周围和附件周围淋巴细胞浸润被观察到,伴有嗜酸性粒细胞。CD123染色突出显示了16%的炎性细胞。IgG的直接免疫荧光,IgA,IgM,C3和纤维蛋白原显示IgG和纤维蛋白原的线性基底膜染色呈阳性,其余的免疫反应物没有明显的染色。考虑到患者的用药史,SS-A和SS-B抗体阴性,一种固定的药疹是受欢迎的。讨论。本文讨论了临床表现,病理生理机制,以及固定药疹和Rowell综合征的诊断标准。结论。认识到固定药疹和Rowell综合征的不同临床和组织病理学特征,特别是当分享皮肤表现时,强调了全面诊断方法和实验室测试的重要性。
公众号