Dystrophic Epidermolysis Bullosa
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Dystrophic epidermolysis bullosa (DEB) is the most severe presentation of epidermolysis bullosa with skin fragility occurring in the sublamina densa region. DEB is the second most common presentation of EB after EBS. There are two subtypes of DEB. The first is the dominant dystrophic phenotype (DDEB) and the second is the recessive dystrophic phenotype (RDEB). Both subtypes have mutations in COL7A1, which correlates to type VII collagen.
- 3.Pourreyron C, Cox G, Mao X, Volz A, Baksh N, Wong T, Fassihi H, Arita K, O’Toole EA, Ocampo-Candiani J, Chen M, Hart IR, Bruckner-Tuderman L, Salas-Alanis JC, McGrath JA, Leigh IM, South AP. Patients with recessive dystrophic epidermolysis bullosa develop squamous-cell carcinoma regardless of type VII collagen expression. J Investig Dermatol. 2007;127(10):2438–44.CrossRefGoogle Scholar