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A brief review of aetiological aspects, clinical features and treatment of Papillon-Lefèvre syndrome, is mentioned. A case is reported of a girl having typical features of syndrome. In part II of this article we include oral rehabilitation of these patients using osseointegrated implants.

Although prepubertal periodontitis (PP) is often associated with the Papillon-Lefèvre (PLS), the etiopathogenetic mechanism in this disease has not been clarified as yet. A three and half year old female with leukocyte chemotaxis deficiency and decrease of T-lymphocytes is presented. The immunohistochemical evaluation of neutrophils showed a reduction of myeloperoxidase content, whereas ultrastructural studies revealed no particular change. The possible role of such a deficiency in the pathogenesis of the periodontal lesion is discussed.

A case is reported of a boy with Papillon-Lefevre syndrome. Despite prolonged systemic antibiotic therapy and repeated professional plaque control, the periodontal condition lead to loss of all primary and permanent teeth soon after eruption.

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.

A case of Papillon-Lefèvre syndrome is presented. Hyperkeratosis of the palms and soles along with precocious destruction of the periodontal support of both the primary and permanent dentitions were observed. Ectopic intra-cranial calcified deposits, seen in some cases of the syndrome, were absent in this case. A greater awareness of this condition among dentists and dermatologists would be helpful in identifying more cases for study.