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CASE STUDY: EPIDERMOLYSIS BULLOSA

Noman Amanat, Muhammad Afzal, Ms. Hajra sarwar

Epidermolysis Bullosa is a group of rare genetic skin conditions, which is characterized by extremely fragile skin and recurrent blister formation, resulting from minor mechanical friction or trauma. Discoveries of the molecular basis of epidermolysis bullosa have resulted in the development of diagnostic tools, including prenatal and preimplantation testing. Historically, epidermolysis bullosa subtypes have been classified according to skin morphology. Based on a better understanding of the basement membrane zone (BMZ) and the genes responsible for its components, newer treatments (e.g., gene or protein therapy) may provide solutions to the skin fragility found in patients with epidermolysis bullosa. Abstract: Epidermolysis bullosa is a large rare group of genetically determined disorders that are inherited. As autosomal dominant or recessive. Most common feature of this disease is blisters formation on the skin and oral mucosa at different Levels following trauma or traction. Exact cause still unknown, but mostly appears to be related to abnormal enzyme activity and collagen. The most common oral manifestations involve, enamel hypoplasia, rampant caries, microstomia Tongue atrophy, oral ulceration with scaring and constriction of buccal vestibules. Dental treatment of these patients is a great challenge that involves preventive measures by rein- Forcing oral hygiene and regular fluoride applications, in addition to treatment of carious teeth and Extraction of teeth with poor prognosis. In this study two cases of Epidermolysis Bullosa, simplex and dystrophic types are reported and their specific clinical Manifestations with their dental clinical treatment are described.

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