Death from metastatic, cutaneous, squamous cell carcinoma in autosomal recessive dystrophic epidermolysis bullosa despite permanent inpatient care.

ABSTRACT

A 36-year-old female patient with severe autosomal recessive dystrophic epidermolysis bullosa, who had spent her entire life from age 2 as an inpatient in the dermatology unit, recently died of metastatic squamous cell carcinoma of the skin. The development of malignancy was not prevented by continuous medical and nursing supervision and, despite early detection, rapidly led to her death. Oral phenytoin and topical mupirocin ointment had not reduced blistering.