 |  | Color Atlas & Synopsis of Pediatric Dermatology, 2eSection 5. Primary Bullous Dermatoses | |
Topics Discussed: dermatology; epidermolysis bullosa; epidermolysis bullosa dystrophica; epidermolysis bullosa simplex; junctional epidermolysis bullosa; linear iga dermatosis.
Sections:
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, dermal—epidermal 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
bullae)- Major subtypes:
- EBS, Weber—Cockayne.
- EBS, Koebner.
- EBS, Dowling—Meara.
- EBS with muscular dystrophy.
The content above is only an
excerpt. For full access, log into an existing user account below, purchase an annual subscription, or
purchase a short-term subscription to the complete website.
offers pediatrics students,
educators, and practioners access to leading McGraw-Hill texts, interactive imaging
content, exclusive multimedia, and flexible curricular tools.
Timed access to all of AccessPediatrics
24 hours for $29.95
48 hours for $49.95
Or