Cutaneous Bullous Diseases

Many cutaneous disorders have associated esophageal involvement. The more common of these disorders include epidermolysis bullosa, cicatricial pemphigoid (CP), lichen planus, and pemphigus vulgaris. Epidermolysis bullosa is a relatively rare cutaneous disease mediated by circulating IgG antibodies directed against type VII collagen. Clinically, patients develop intradermal blistering lesions with scarring at sites of trauma (hands and feet are most common). The proximal third of the esophagus, consisting of stratified squamous epithelium, is at risk. Strictures can occur due to concentric scarring from large blisters. Treatment is dilation. There is reported treatment of severe cases by colonic interposition free tissue grafting, to replace the stratified squamous epithelium of the proximal third of the esophagus (25).

Bullous pemphigoid (BP) is another autoimmune blistering disease, which generally affects patients over the age of 60. It is the most common of the bullous cutaneous lesions. CP is a related, more heterogenous disease. In both diseases, autoantibodies to proteins in the basement membrane are the causative agents. Oral involvement is 40% in BP and reaches up to 100% in CP. As in epidermolysis bullosa, there is a very real risk of esophageal webs and strictures due to cicatricial scarring. Treatment for BP and CP is with corticosteroids; symptomatic strictures should be dilated.

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