DYSPLASIA OF ENAMEL OR DENTINE IN CONGENIAL HEREDITARY DISEASE
Dysplasia of dental tissue may appear as a symptom in rare congenital diseases. The present article describes selected examples of rare, congenital diseases in which dysplasia of dental tissues may occur. Dysplasia of enamel occurs in certain diseases of the skin: epidermolysis bullosa (EB) and focal dermal hypoplasia (FDH). EB, Junctional type, has hypomineralised and hypoplastic enamel. FDH has hypoplastic enamel with an irregular surface and an atypical crown morphology. Both conditions entail major pedodontic and prosthodontic treatment needs. Dysplasia of dentine occurs in certain diseases of the skeleton: osteogenesis imperfecta (OI), which is caused by a collagen defect, and X-linked hypophosphatemia (XLH), which is a metabolic bone disease. In OI, the dental symptom is dentinogenesis imperfecta (DI), which mainly occurs in severe OI. DI leads to an increased risk of tooth fractures and tooth loss. In XLH, irregularities occur in the entire pulp-dentineorgan, and the patients experience a risk of spontaneously evolving necrosis of the pulp. In XLH, elements of enamel dysplasia (enamel cracks) may also be present. Dysplasia of the cementum occurs in another disease of the skeleton: hypophosphatasia (HPP). According to degree of severity, HPP is divided into 6 subtypes. To varying degrees, the cemental layer of the root is acellular. Premature exfoliation of primary teeth may occur. In adults, tooth loss not related to periodontitis may occur. Severe HPP may also be associated with enamel dysplasia.
CONCLUSION – Enamel or dentine dysplasia can occur in rare diseases and lead to extraordinary dental treatment needs. This requires referral to the dental knowledge centres for professional advice on diagnostics and treatment.