The cumulative risk of developing at least one SCC is depicted in the following table. Of note, the occurrence of multiple primary tumors is the rule in this disease. Although usually histologically well-differentiated, most of these SCCs act aggressively.
Local recurrence is common, as is regional spread and eventual metastasis and death.
Therapy involves wide excision of primary lesions. Although not contraindicated, most of these tumors, once metastatic, are poorly responsive to chemotherapy and radiotherapy. These data demonstrate that patients with RDEB are at significant risk of developing at least one SCC, with earliest onset during mid-adolescence. After that time, there is a strikingly steep increase in this cumulative risk. Of importance, since 1995, a few patients with non-Herlitz junctional EB have also developed SCCs, although too few have been reported to permit accurate lifetable projections.