Department of Pediatrics, Nagahama City Hospital, Nagahama, Shiga, Japan.
World J Pediatr. 2012 May;8(2):181-4. doi: 10.1007/s12519-011-0308-2. Epub 2011 Aug 27.
Sweet's syndrome (acute febrile neutrophilic dermatosis) is characterized by fever, polymorphonuclear leukocytosis of blood, painful plaques on the limbs, face and neck, and histologically a dense dermal infiltration with mature neutrophils. Sweet's syndrome is often a complication of hematologic malignant disease or drug-induced sensitivity reactions and has a significant susceptibility correlated with certain human leukocyte antigen (HLA).
A 5-week-old Japanese girl with Sweet's syndrome confirmed by skin biopsy was successfully treated and HLA analysis was performed.
The patient was one of the youngest patients reported with Sweet's syndrome, suggesting the importance of the genetic background. Although the HLA types of the patient did not have B54, which was reported as a significant susceptibility correlation, structural analysis of the patient's HLAs suggested a similar possible motif for the bound peptides.
Studies on the HLA bound peptides and HLA structural analysis for patients with Sweet's syndrome would be valuable for understanding the molecular mechanism of the pathogenesis.
Sweet 综合征（急性发热性嗜中性皮病）的特征为发热、血液中性粒细胞增多、四肢、面部和颈部出现疼痛性斑块，组织学上表现为真皮内成熟中性粒细胞弥漫性浸润。Sweet 综合征常继发于血液恶性肿瘤或药物诱导的过敏反应，与某些人类白细胞抗原（HLA）显著相关。
对 1 例经皮肤活检证实的 5 周龄日本 Sweet 综合征女婴进行成功治疗，并进行了 HLA 分析。
该患者是报道的最年轻的 Sweet 综合征患者之一，提示遗传背景的重要性。虽然患者的 HLA 类型不具有报告的显著相关性 B54，但对患者 HLA 的结构分析提示结合肽的类似可能基序。
对 Sweet 综合征患者的 HLA 结合肽和 HLA 结构分析的研究将有助于理解发病机制的分子机制。
