Development of bullous pemphigoid in junctional epidermolysis bullosa.

Abstract

Junctional epidermolysis bullosa, generalized intermediate subtype (JEB-GI), is a heterogeneous group of mild to moderate skin blistering phenotypes caused by mutations in laminin-332 and collagen XVII encoding genes1 . Instead, circulating autoantibodies directed to both collagen XVII, also named BP180, and BP230, are hallmarks of the autoimmune blistering disease bullous pemphigoid (BP). The presence of anti-skin autoantibodies in patients suffering from congenital EB has been reported2-5 .