From OMIM
Generalized severe epidermolysis bullosa simplex-2A (EBS2A) is an autosomal dominant skin disorder characterized by extensive intraepidermal blistering after minor mechanical stress from the time of birth with herpetiform marginal spreading and central healing. Oral mucosal involvement, nail dystrophy, onychogryposis, formation of milia, and palmoplantar hyperkeratosis are common features. Blistering is more frequent in warm weather and generally improves with advancing age. The 'severe' subtype of EBS was previously known as the Dowling-Meara type (EBSDM). In addition to the intraepidermal blister formation after minor mechanical stress common to all forms of EBS, skin biopsies from patients with the severe EBS subtype show aggregation and clumping of basal keratins on electron microscopy, resulting in a total collapse of the keratin cytoskeleton of basal keratinocytes (summary by Muller et al., 1999). For a discussion of genetic heterogeneity of the subtypes of EBS, see EBS1A (131760).  http://www.omim.org/entry/619555