ABSTRACT
Hyalinosis cutis et mucosae is a rare, autosomal recessive disorder characterized by diffuse deposition of a hyaline-like material in the skin and mucous membrane of the oral cavity, upper respiratory tract, and internal organs. In the first weeks of life it begins with typical hoarseness due to hyaline deposits in the larynx. Rough, yellowish-white papular deposits in the skin and the oral mucosa usually develop during childhood. The etiology and pathogenesis are unknown and the treatment is only symptomatic. A 14-year old boy developed several typical clinical features of the disease since birth and remained undiagnosed until he was referred by his dentist to our department for oral evaluation. The clinical, histopathological and immunological aspects of the patient are discussed in detail. Oral and systemic manifestations of the disease are also reviewed. The oral mucosa appeared nodular, diffusely enlarged and thickened because of infiltration with waxy-yellowish-white plaques and nodules. The patient also exhibited a thickened, furrowed appearance of the skin with several skin scars, eyelid nodules, loss of eyelashes and voice hoarseness. The clinical diagnosis of hyalinosis cutis et mucosa was confirmed histologically.
Subject(s)
Lipoid Proteinosis of Urbach and Wiethe/pathology , Mouth Diseases/pathology , Mouth Mucosa/pathology , Adolescent , Humans , Hyalin/metabolism , Lipoid Proteinosis of Urbach and Wiethe/complications , Male , Mouth Diseases/complications , Mouth Mucosa/metabolism , Pediatric DentistryABSTRACT
In the present study the degree of density of three anatomic regions in 215 orthopantomographs was evaluated. The three anatomic regions which were selected were: the maxillary sinus, the hard palate and the hard tissues (enamel and dentin) of the right lower first molar. The degree of density was correlated with the age and sex of the patients and it was found to increase with the age. The difference of the density was found to be statistical significant between the decade 1-10 compared to the other decades of age. The degree of density was greater in the women in all three anatomic regions but statistical significant only in the first molar region and in the decades 1-10 and 31-40.
