Bork K, Löst C
Hautarzt. 1980 Apr;31(4):179-83.
Numerous patients with Papillon-Lefèvre syndrome show scaly erythematous lesions over knees, elbows and the interphalangeal joints additionally to the well known palmo-plantar hyperkeratosis. Not rarely, these skin symptoms are misdiagnosed as psoriasis. The obligatory and highly specific periodontitis marginalis profunda should lead to the correct diagnosis. A basic immune defect, which was shown in such a patient recently, could not be verified in the patient described here.
许多掌跖角化-牙周破坏综合征患者除了众所周知的掌跖角化过度外,在膝盖、肘部和指间关节处还出现鳞屑性红斑病变。这些皮肤症状常被误诊为银屑病。必须且高度特异性的重度边缘性牙周炎应能引导出正确的诊断。最近在一名此类患者中发现的一种基本免疫缺陷,在此处描述的患者中未得到证实。