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Scripta Scientifica Medicinae Dentalis

Epidermolysis bullosa and Kindler syndrome - where is the border in oral manifestaions

Elitsa Deliverska, Assya Krasteva

Abstract

Epidermolysis bullosa (EB) represents a rare chronic autoimmune subepidermal vesiculobullous disorder that affects the skin and mucosa and is characterized by blistering and mechanical fragility of the skin. The most recent classification recognizes four major EB  groups:  intraepidermal EB (Simplex), junctional EB, dermolytic EB (Dystrophic), and mixed EB (Kindler syndrome). Kindler syndrome is a rare genodermatosis with clinical features of trauma-induced blistering, poikiloderma, skin atrophy, mucosal inflammation and varying degrees of photosensitivity. Although Kindler syndrome is classified as a subtype of epidermolysis bullosa, it has distinct clinicopathological and molecular abnormalities.

The clinical features of the syndrome have been annotated by different authors but definite criteria to confirm the diagnosis have not yet been generally accepted.


Keywords

Epidermolysis bullosa; Kindler syndrome; oral and maxillo-facial signs

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DOI: http://dx.doi.org/10.14748/.v1i1.591

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About The Authors

Elitsa Deliverska
Medical university of Sofia
Bulgaria

Faculty of dental medicine, department of oral and maxillofacial surgery

Assya Krasteva
Medical university of Sofia
Bulgaria

Faculty of dental medicine, department of oral and maxillofacial surgery

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