Upper airway and external genital involvement in epidermolysis bullosa dystrophica

Radiographic findings in two patients with uncommon manifestations of epidermolysis bullosa dystrophica are described. A girl with recurrent urinary tract infections had scarring of the external genitalia producing chronic vaginal and uterine reflux and retention of urine. A second patient with long-standing stridor was found to have subglottic narrowing due to localized subglottic edema associated with an inflammatory membrane. The second case illustrates the occasional involvement of columnar epithelial surfaces which can occur in both epidermolysis bullosa dystrophica and epidermolysis bullosa hereditaria letalis.