Sweet综合征口腔黏膜表现1例报道及文献回顾

Oral mucosal manifestations of Sweet’s syndrome: a case report and literature review

摘要

目的 探讨Sweet综合征的口腔黏膜病损表现，为临床早发现并正确诊断该病提供参考。方法 报道1例60 岁女性Sweet综合征患者的口腔黏膜病损表现，并结合相关文献对Sweet综合征进行分析。结果 患者双下肢皮肤红斑伴口腔黏膜破溃疼痛3 d，患者皮损症状较轻，但口腔黏膜多发性大面积糜烂，疼痛明显，故首诊于口腔科。患者发病过程中伴有发烧，体温38.5 ℃；实验室检查示C反应蛋白升高（35.2 mg/L），血沉加快（77.00 mm/h）；患者两侧膝盖及下肢可见散在分布红色斑块，轻度压痛，皮损组织病理学检查示：真皮全层见散在不成熟中性粒细胞聚集灶浸润。根据患者临床体征和实验室检查，皮损组织病理结果的镜下符合Sweet综合征的表现，诊断为Sweet综合征。给予患者1 mL复方倍他米松注射液肌肉注射仅1次；患者对糖皮质激素治疗效果反应良好；口腔采用复方氯己定溶液含漱，病损黏膜处重组牛碱性成纤维细胞生长因子溶液外用，3次/d，疗程为1周；用药4 d后复诊，体温恢复正常，口腔病损明显减轻；2周后复诊，小腿及膝盖处红斑几乎全部消退，口腔黏膜病损基本消失；半年后随访，结果显示皮损未复发；2年后随访，病情稳定，病损未见复发。回顾相关文献表明，Sweet综合征是一种少见的病因不明的炎性反应性皮病，临床上可分为3种类型：特发型、肿瘤相关型以及药物诱导型，男女患病比为1:4，典型临床表现为急性出现的疼痛性红色斑块或结节，病损多位于四肢，常伴发热，外周血中性粒细胞数目增多，血沉加快，C反应蛋白阳性。系统应用糖皮质激素是本病最重要的治疗方法，大多数患者可在短期内改善皮损，但可能会存在潜在感染或停药后复发的情况。部分Sweet综合征患者可伴有口腔病损，但目前有关Sweet综合征在口腔黏膜表现的病例报道却很少，使得临床容易误诊。结论 口腔黏膜病损可能为Sweet综合征的皮肤外表现，应综合考虑患者的全身情况，尽快完善皮肤活检以明确诊断，以免延误病情。

Abstract

Objective To explore the oral mucosal manifestations of Sweet’s syndrome and provide a reference for its early detection and correct diagnosis. Methods The oral mucosal manifestations of a 60-year-old female patient with Sweet’s syndrome are described in detail, followed by a discussion of the related literature. Results The patient had skin erythema of both lower extremities, which was accompanied by oral mucosal ulceration and pain for 3 days. The patient presented with mild cutaneous lesions and diffuse large-scale erosion in the oral mucosa with obvious pain. During the onset of the disease, the patient was accompanied by fever with a temperature of 38.5°C. After visiting the Department of Stomatology, laboratory tests showed an increase in C-reactive protein (35.2 mg/L) and an accelerated erythrocyte sedimentation rate (77.00 mm/h). Scattered red plaques and mild tenderness were observed in the knees and lower limbs. Histopathological examination of the skin lesions revealed scattered infiltration of immature neutrophils across the entire dermis. The patient responded well to glucocorticoid therapy. According to the clinical signs and laboratory examination, combined with the lesion histopathological results, a diagnosis of Sweet’s syndrome was given. The patient was administered 1 mL compound Betamethasone injection only once intramuscularly. In the meantime, the patient was asked to gargle with compound chlorhexidine solution and topically apply recombinant bovine basic fibroblast growth factor solution to the damaged mucosa three times a day for 1 week. After 4 days of medication, the patient’s body temperature had returned to normal and the oral lesions were significantly reduced. After 2 weeks, the erythema in the leg and knee had almost all subsided, and the oral mucosal lesions had disappeared. The patient was followed up 6 months after treatment, with no recurrence of skin lesions. After 2 years of follow-up, the disease was stable with no recurrence. A review of the relevant literature shows that Sweet’s syndrome is a rare inflammatory reactive dermatosis with unknown etiology, which can be divided into three clinical types: specific, tumor-related, and drug-induced. The male/female prevalence ratio is 1:4. The salient clinical manifestations are abrupt onset of painful erythematous plaques or nodules most commonly involving the extremities, often accompanied by pyrexia, elevated neutrophil count, elevation of the erythrocyte sedimentation rate, and positive C-reactive protein. The use of glucocorticoids is the most common treatment for this disease, and most patients see a rapid improvement in skin lesions; however, some may experience infection or recurrence after withdrawal. Some patients with Sweet’s syndrome are accompanied by oral lesions, but cases of oral mucosal damage have been rarely reported, and this condition is easily misdiagnosed. Conclusion Oral mucosal lesions may be extraterritorial manifestations of Sweet’s syndrome, and the patient’s systemic condition should be comprehensively considered. Skin biopsy should be completed as soon as possible to make a clear diagnosis, so as not to delay the disease.

关键词

Sweet综合征 / 急性发热性嗜中性皮病 / 口腔糜烂 / 红斑 / 口腔黏膜 / C反应蛋白 / 糖皮质激素 / 鉴别诊断

Key words

Sweet syndrome / acute febrile neutrophilic dermatosis / oral erosion / erythema / oral mucosa / C-reactive protein / glucocorticoid / differential diagnosis

Author summay

牛玉芬，住院医师，硕士研究生，Email：[e-mail]