Papillon-Lefevre syndrome is a rare autosomal recessively inherited condition which shows features common to both dentistry and dermatology. This disease is characterized by palmoplantar hyperkeratosis and severe periodontitis. Therefore both the dentist and the dermatologist should be aware of the same because an early diagnosis of the syndrome can help to preserve the teeth by early institution of treatment, using a multidisciplinary approach. Here we present a case report of Papillon-Lefevre syndrome, along with a comprehensive review of the etiology, pathology, clinical features, differential diagnosis and management of the condition. .
Segmental neurofibromatosis is a rare disorder, characterized by neurofibromas or cafι-au-lait macules limited to one region of the body. Its occurrence on the face is extremely rare and only few cases of segmental neurofibromatosis over the face have been described so far. We present a case of segmental neurofibromatosis involving the buccal mucosa, tongue, cheek, ear, and neck on the right side of the face.
Hemangiopericytoma, initially described by Stout and Murray in 1942 (1), is a rare vascular tumor arising from mesenchymal cells with pericytic differentiation. Hemangiopericytomas usually occur in the 5th decade of life and account for 3-5% of all soft tissue sarcomas and 1% of all vascular tumors (2). The tumor usually occurs in the limbs, pelvis, or head and neck region; 15-30% of all hemangiopericytomas occur in the head and neck (2,3). Here we present a case of hemangiopericytoma of the submandibular region with keratocystic odontogenic tumors in an adolescent patient.
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