Congenital and neonatal blistering disorders

Question 1

Epidermolysis bullosa

Answer 1

Epidermolysis bullosa appears at birth or in infancy. There are numerous forms and clinically they cannot be differentiated in initial assessment. They all are due to mutations in proteins in the basement membrane
Differential diagnosis is mainly with infectious diseases: Staph, Pseudomonas, herpesviruses.
Some forms are severe and usually lethal, others improve with age Some have more mucosal involvement (predisposing to malabsorption, cornnal abrasions and UTI). Other manifestations may include: aplasia cutis, loss of nails or nail dystrophy, alopecia, dental problems. pseudodactyly and skin cancer.
Diagnosis is with immunofluorescence and genetic mutation analysis
Treatment is wound care and treatment and prevention of complications

Question 2

Eczema herpeticum

Answer 2

Herpes affecting area of eczema