Squamous cell carcinoma in dominant type epidermolysis bullosa dystrophica

The unusual genetic disorder epidermolysis bullosa dystrophica has been reported in several patients in whom the chronic cutaneous scars led to the development of cutaneous squamous cell carcinoma. However, only one of these previously reported cases involved the autosomal dominant form of the disease; the remainder occurred in its recessive counterpart. We report the second and third patients with squamous cell carcinoma associated with the dominant form of epidermolysis bullosa dystrophica. In addition, we not only observed the previous electron microscopic findings of decreased numbers of anchoring fibrils beneath the basal lamina but have also noted marked disruption of the basal lamina itself.