Papillon-Lefevre syndrome is a very rare syndrome of autosomal recessive inheritance characterized by palmar-plantar hyperkeratosis and early onset of a severe destructive periodontitis, leading to premature loss of both primary and permanent dentitions. Various etiopathogenic factors are associated with the syndrome but a recent report has suggested that the condition is linked to mutations of the cathepsin C gene. Two cases of Papillon-Lefevre syndrome in the same family, having all of the characteristic features, are presented. An 11-year-old girl and a 9-year-old boy presented with the complaints of loose teeth. Both expressed hyperkeratosis of palms, soles, and knees. Severe generalized periodontal destruction, with mobility of teeth, was evident on intraoral examination; orthopantomograph examination showed severe generalized loss of alveolar bone in both the patients.

GD and GCBD showed a greater reduction in dentin hypersensitivity than SBU.

Malignant myoepithelioma (MM) of parapharyngeal space provide both a diagnostic and therapeutic challenge. Malignant myoepitheliomas are rare tumour of salivary glands. Most occur in the parotid gland, few other sites of origin are described. Malignant myoepithelioma of the parapharyngeal space are extremely rare with only a few cases has been reported. We present a case of malignant myoepithelioma in the parapharyngeal space and discuss its diagnostic and therapeutic aspects.

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