Abstract
We report a familial case of tonsil-related pustulosis palmaris et plantaris (PPP) and the HLA types. A 47-year-old female with a 18-year history of PPP visited our clinic for tonsillectomy in April 1999. She had additional symptoms of sternocostoclavicular hyperostosis (SCCH). Her elder sister underwent tonsillectomy for PPP in 1998 and her skin lesions showed a marked improvement. In this patient her skin lesions and thoracic pain were markedly improved without any medication 18 months after surgery. Although her parents did not have PPP, all 4 siblings (2 brothers and 2 sisters) and her second daughter were diagnosed with PPP. Human Leukocyte Antigen (HLA) typing revealed haplotypes A2, B46, Cw1, DR8 and DQ1 in 4 of 6 family members. We consider that these haplotypes are associated with PPP.