Altaweel Fatmah, Ahmad Adnan, Albazali Abeer, Mandani Yasmeen, Mosbeh Alsadat
Dermatology, Kuwait Institute for Medical Specializations, Sulibekhat, KWT.
Dermatology, Farwaniya Hospital, Kuwait City, KWT.
Cureus. 2024 Dec 24;16(12):e76349. doi: 10.7759/cureus.76349. eCollection 2024 Dec.
Wells' syndrome is a rare inflammatory disease characterized by recurrent, erythematous plaques with histological flame figures, which can be associated with idiopathic hypereosinophilic syndrome (IHES). We present a case of a nine-year-old boy who presented with a one-year history of an itchy rash on his legs associated with peripheral eosinophilia. The rash initially started as an annular plaque and developed raised borders with central hyperpigmentation. A skin biopsy revealed histopathological features consistent with Wells' syndrome, including flame figures and eosinophilic infiltrate. Other investigations revealed hypereosinophilia and bone marrow eosinophilia. Our case demonstrates Wells' syndrome associated with IHES in a child. This overlap renders both diagnosis and treatment challenges. Corticosteroids are effective for Wells' syndrome but may require augmentation with additional therapies for IHES, such as dupilumab. The diagnosis of Wells' syndrome with IHES in this patient underlines the necessity for clinicians to consider and rule out other eosinophilic disorders. It highlights the value of a multidisciplinary strategy to enhance management and optimize outcomes. This case provides valuable insight into the comprehension of rare eosinophilic disorders, but additional research is required in this field to better understand the underlying pathophysiologic mechanisms of these disorders.
威尔斯综合征是一种罕见的炎症性疾病,其特征为反复出现的红斑性斑块并伴有组织学上的火焰状图像,可与特发性嗜酸性粒细胞增多综合征(IHES)相关。我们报告一例9岁男孩,其腿部出现瘙痒性皮疹1年,伴有外周血嗜酸性粒细胞增多。皮疹最初表现为环状斑块,边界隆起,中央色素沉着。皮肤活检显示组织病理学特征与威尔斯综合征一致,包括火焰状图像和嗜酸性粒细胞浸润。其他检查显示嗜酸性粒细胞增多和骨髓嗜酸性粒细胞增多。我们的病例显示威尔斯综合征在儿童中与IHES相关。这种重叠给诊断和治疗带来了挑战。皮质类固醇对威尔斯综合征有效,但可能需要联合其他治疗IHES的疗法,如度普利尤单抗。该患者威尔斯综合征合并IHES的诊断强调了临床医生考虑并排除其他嗜酸性粒细胞疾病的必要性。它凸显了多学科策略在加强管理和优化治疗效果方面的价值。本病例为理解罕见的嗜酸性粒细胞疾病提供了有价值的见解,但该领域还需要更多研究以更好地了解这些疾病的潜在病理生理机制。