Background: Cutaneous squamous cell carcinoma (SCCA) is an important cause of death in patients with the severe form of recessive dystrophic epidermolysis bullosa (RDEB). These cancers often present with features that are different from those of actinically induced SCCA.
Objective: To emphasize the unusual features with which SCCA can present in RDEB and discuss early recognition of such cancers.
Methods: We present the case of a patient with RDEB who developed a cutaneous ulcer that appeared to be filled with heaped-up granulation tissue. Biopsy, however, showed moderately differentiated squamous cell carcinoma.
Results: The patient underwent wide surgical excision of the cancer, with split-thickness skin graft. She has remained disease-free three years later.
Conclusion: In patients with RDEB, any ulcer that appears to be filled with heaped-up granulation tissue should be biopsied to rule out SCCA.