Download Color Atlas of Orofacial Health and Disease in Children and by Scully Crispian PDF

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By Scully Crispian

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Extra info for Color Atlas of Orofacial Health and Disease in Children and Adolescents

Sample text

Hemangioma involving lip and skin. Congenital and hereditable disorders with sole or prominent orofacial involvement 29 HEREDITARY ANGIOEDEMA (C1-ESTERASE INHIBITOR DEFICIENCY) Hereditary angioedema (HANE; Fig. 65) mimics allergic angioedema (see p. 131), although it produces a more severe reaction. Despite its hereditary nature, usually as an autosomal dominant trait, the disease may not present until later childhood or adolescence and nearly 20% of cases are caused by spontaneous mutation. Rare cases of C1-esterase inhibitor or deficiency are acquired.

Dentinogenesis imperfecta. Onlays have been placed on the premolars and molars to prevent loss of tooth substance. 5% of the general population, of unknown etiology, in which the filiform papillae desquamate to form oval to irregular patches. A burning sensation may be experienced, especially when ingesting acidic or spicy foods or beverages. The characteristic lesions include well-demarcated patches of erythema that have a predilection for the anterior two-thirds of the tongue. These somewhat circular patches are devoid of filiform papillae and often surrounded by a yellowish-white scalloped border.

82). In OFD type I, which is seen only in girls, there is also clinodactyly, cleft palate, hamartomas of the tongue, missing teeth and sometimes renal defects and learning disability. OFD type II (Mohr syndrome) is a less severe autosomal recessive condition, but there is often conductive deafness. Other orofacial–digital syndromes are rare. Diagnosis Diagnosis is clinical, supported by imaging. PAPILLON–LEFÈVRE SYNDROME Papillon–Lefèvre syndrome (Figs 83, 84) is a rare, genetically-linked disorder of cathepsin manifesting with prepubertal periodontitis in association with palmar-plantar hyperkeratosis.

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