Maffucci’s Syndrome: report of a case with oral manifestation / Síndrome de Maffucci: relato de caso com manifestação oral

Introduction: Maffucci’s Syndrome is a rare nonhereditary mesodermal dysplasia consisting of multiple haemangioma of the soft tissue and enchondromas, mostly affecting phalanges and long bones. The syndrome can also be associated with a variety of other benign and malignant tumors. Case report: Here we report a case of Maffucci’s Syndrome and haemangioma of lip and palate which is rare in this syndrome. This case report describes an 18 year old boy with multiple nodular soft tissue swellings involving the anterior hard palate and lower labial mucosa and bony abnormalities (enchondromas) involving the lower limb.

Introdução: A Síndrome de Maffucci é uma displasia mesodermal não hereditária rara. Consiste de múltiploshemangiomas de tecido mole e encondromas, a maioria afetando as falanges e os ossos longos. A síndromepode também estar associada a uma variedade de tumores benignos e malignos. Relato de caso: O presente trabalho apresenta um caso de Síndrome de Maffucci e hemangioma de lábio e palato, o que é raro nessa síndrome, descrevendo o caso de um garoto de 18 anos de idade com inchaços nodulares múltiplos de tecido mole envolvendo o palato duro anterior e a mucosa labial inferior, além de anomalias ósseas (encondromas)acometendo um membro inferior.

Oro-facial aspects of leprosy: Report of two cases with literature review

Leprosy is a chronic infectious disease affecting primarily the skin, peripheral nerves, respiratory system andthe eyes. Leprosy induces various types of clinical presentation affecting the patient´s immune response. Cellmediatedimmunity is considered to be the crucial defence against the disease and the magnitude of this immunitydefines the extent of the disease. The article presents two case reports of manifestations of leprosy inthe oro-facial region, with a brief review of various other important oro-facial manifestations of leprosy. Thefirst report deals with granulomatous nodules in the palate while the second report presents bilateral facialpalsy in leprosy patients. Both the reports gain importance due to rare oral manifestation in a borderline leprosypatient in the first case, while the second case presents a rare bilateral Bell´s sign. The role of the dentalprofession and especially the Oral Medicine specialist is of great importance in early diagnosis of oral lesions (AU)

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Oro-facial aspects of leprosy: report of two cases with literature review.

Leprosy is a chronic infectious disease affecting primarily the skin, peripheral nerves, respiratory system and the eyes. Leprosy induces various types of clinical presentation affecting the patient's immune response. Cell-mediated immunity is considered to be the crucial defence against the disease and the magnitude of this immunity defines the extent of the disease. The article presents two case reports of manifestations of leprosy in the oro-facial region, with a brief review of various other important oro-facial manifestations of leprosy. The first report deals with granulomatous nodules in the palate while the second report presents bilateral facial palsy in leprosy patients. Both the reports gain importance due to rare oral manifestation in a borderline leprosy patient in the first case, while the second case presents a rare bilateral Bell's sign. The role of the dental profession and especially the Oral Medicine specialist is of great importance in early diagnosis of oral lesions.