Epidermolysis Bullosa Simplex With Circinate Migratory Erythema

Epidermolysis bullosa simplex with circinate migratory erythema (EBS-migr) is a basal subtype of epidermolysis bullosa simplex (EBS, see this term) characterized by belt-like areas of erythema with multiple vesicles and small blisters at the advancing edge of erythema.

Epidemiology

Prevalence is unknown but 2 families have been reported to date.

Clinical description

Onset of the disease is usually at birth. The lesions occur on the limbs and trunk and heal with brown pigmentation but no scarring. Extracutaneous involvement is absent.

Etiology

EBS-migr is due to a specific mutation in the KRT5 (12q13.13) gene, encoding keratin 5.

Genetic counseling

Transmission is autosomal dominant.