|Color Atlas & Synopsis of Pediatric Dermatology, 2e|
Section 5. Primary Bullous Dermatoses
Topics Discussed: dermatology; epidermolysis bullosa; epidermolysis bullosa dystrophica; epidermolysis bullosa simplex; junctional epidermolysis bullosa; linear iga dermatosis.
Excerpt:"Epidermolysis bullosa (EB) defines a group of rare inherited
mechanobullous skin disorders that are characterized by skin fragility
and bullae formation. There are three forms of the disease: EB simplex,
junctional EB, and dystrophic EB with over 20 different phenotypes
representing mutations in the genes of at least 7 structural proteins
of the skin (in the epidermis, dermalepidermal junction,
or upper papillary dermis).Melanocytic nevi in children with recurrent blistering disorders
may appear clinically atypical (large and dark with irregular borders)
while having reassuring histological patterns.AgeType and DistributionAge Onset of blisters at birth
or early infancy (Fig. 5-2A).Type and DistributionAge Blistering in both forms appears
at birth.Type and DistributionAge Mostly preschool children (mean
age 4.5 years).Type Large tense bullae, central
crusting (Fig. 5-4A).1. Epidermolysis bullosa simplex (EBS, with intraepidermal
- Major subtypes:
- EBS, WeberCockayne.
- EBS, Koebner.
- EBS, DowlingMeara.
- EBS with muscular dystrophy.
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