Author Affiliations: Department of Pediatrics, Nagahama City Hospital, Nagahama, Shiga, Japan (Omoya K, Naiki Y, Yoshioka S, Uchida Y, Taga T); Department of Pediatrics, Graduate School of Medicine, Gifu University, Gifu, Japan (Omoya K, Kato Z, Kondo N); Center for Emerging Infectious Diseases (CEID), Gifu University, Japan (Kato Z, Kondo N); Center for Advanced Drug Research (CADR), Gifu University, Japan (Kato Z, Kondo N); Department of Dermatology, Nagahama City Hospital, Nagahama, Shiga, Japan (Aoki Y, Deguchi H)

Corresponding Author: Zenichiro Kato, MD, PhD, Department of Pediatrics, Graduate School of Medicine, Gifu University, Yanagido 1-1, Gifu 501-1194, Japan (Tel: +81 (58) 230 6386;  Fax: +81 (58) 230 6387; Email: zen-k@gifu-u.ac.jp)

doi: 10.1007/s12519-011-0308-2

Background: 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).

Methods: A 5-week-old Japanese girl with Sweet's syndrome confirmed by skin biopsy was successfully treated and HLA analysis was performed.

Results: 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.

Conclusion: 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.

Key words: acute febrile neutrophilic dermatosis; human leukocyte antigen; infant; rectovaginal fistula; Sweet's syndrome

World J Pediatr 2012;8(2):181-184