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Sweet 综合征:来自马来西亚两家三甲医院的回顾。

Sweet's syndrome: A review from two tertiary hospitals in Malaysia.

机构信息

Hospital Queen Elizabeth, Department of Dermatology, Sabah, Malaysia.

Hospital Pulau Pinang, Department of Dermatology, Penang, Malaysia.

出版信息

Med J Malaysia. 2022 Nov;77(6):669-675.

PMID:36448383
Abstract

INTRODUCTION

Sweet's syndrome (SS) also known as acute febrile neutrophilic dermatosis, is an uncommon disease characterised by acute onset of tender, violaceous or erythematous, oedematous papules, nodules or plaques, with fever. It is classified into classic, malignancyassociated, and drug-induced subtypes.The aims of this study is to evaluate the subtypes, clinical features, laboratory profiles, and treatment of patients with SS.

MATERIALS AND METHODS

We did a retrospective medical record review of all patients with SS from July 2014 to July 2018 at Hospital Queen Elizabeth and Hospital Pulau Pinang, both tertiary hospitals in Malaysia.

RESULTS

Twenty-nine patients were included. Approximately half of the patients (15) were females with a mean age of onset of 50.93 (± 11.52) years. The most common subtype was classic (62.0%) followed by malignancy-associated (31.0%) and drug-induced (6.9%). Among the patients with the classic subtype, infective-related causes (50.0%) were the most common. Among the patients with malignancy, eight had haematological malignancy and one had a solid tumour. Two-third of the malignancies were diagnosed within a year after the diagnosis of SS. Eight of our patients in Sabah had mycobacterial infections with three having concomitant haematological malignancies. Patients with malignancy-associated SS had lower mean haemoglobin (p=0.018) and mean platelet count (p=0.031). Itch was associated with the presence of pustules (p=0.038). Histopathological examination of all skin lesions showed dermal neutrophilic infiltrates and 25 (86.2%) of them had papillary dermal oedema. The study was limited by its retrospective design. The sample size was small likely due to the uncommon occurrence of this condition.

CONCLUSION

SS is an uncommon dermatosis with distinctive clinical and histopathological features. Screening for underlying malignancy is essential especially for those who present with anaemia, thrombocytopenia, and pathergy phenomenon. Mycobacterial infection should be considered in this region due to high tuberculosis burden.

摘要

简介

Sweet 综合征(SS),又称急性发热性嗜中性皮病,是一种少见的疾病,其特征为急性发作的触痛性、紫红色或红色、水肿性丘疹、结节或斑块,伴有发热。它分为经典型、恶性肿瘤相关型和药物诱导型。本研究旨在评估 SS 患者的亚型、临床特征、实验室特征和治疗方法。

材料和方法

我们对 2014 年 7 月至 2018 年 7 月期间在马来西亚两家三级医院(伊丽莎白女王医院和槟城医院)就诊的所有 SS 患者的病历进行了回顾性研究。

结果

共纳入 29 例患者。约一半患者(15 例)为女性,平均发病年龄为 50.93(±11.52)岁。最常见的亚型是经典型(62.0%),其次是恶性肿瘤相关型(31.0%)和药物诱导型(6.9%)。在经典型患者中,感染相关病因(50.0%)最为常见。恶性肿瘤患者中,8 例为血液系统恶性肿瘤,1 例为实体瘤。2/3 的恶性肿瘤在 SS 诊断后一年内被诊断。我们沙巴的 8 例患者有分枝杆菌感染,其中 3 例同时伴有血液系统恶性肿瘤。恶性肿瘤相关 SS 患者的平均血红蛋白(p=0.018)和平均血小板计数(p=0.031)较低。瘙痒与脓疱存在相关(p=0.038)。所有皮肤病变的组织病理学检查均显示真皮中性粒细胞浸润,其中 25 例(86.2%)有乳头层水肿。本研究受到其回顾性设计的限制。由于这种疾病罕见,样本量较小。

结论

SS 是一种少见的皮肤病,具有独特的临床和组织病理学特征。筛查潜在恶性肿瘤至关重要,尤其是那些有贫血、血小板减少和穿刺现象的患者。由于结核病负担较高,应考虑本地区分枝杆菌感染。

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