Dhadke S V, Kulkarni P M, Dhadke V N, Deshpande N S, Wattamwar P R
Department of Medicine Dr. VM Medical College Solapur.
J Assoc Physicians India. 2006 Mar;54:246-7.
Papillon Lefèvre syndrome is a rare disease characterized by skin lesions caused by palmar-plantar hyperkeratosis, and severe periodontal destruction involving both the primary and permanent dentitions. It is transmitted as an autosomal recessive condition and consanguinity of parents is evident in about one-third of cases. Pyogenic liver abscess is an increasingly recognized complication. We report a new case of this association and review the current literature.
帕皮永-勒费夫尔综合征是一种罕见疾病,其特征为掌跖角化过度引起的皮肤病变,以及累及乳牙列和恒牙列的严重牙周破坏。它以常染色体隐性遗传病形式遗传,约三分之一的病例中可见父母近亲结婚。肝脓肿是一种越来越被认识到的并发症。我们报告了这一关联的一例新病例并对当前文献进行了综述。