Inherited Junctional Epidermolysis Bullosa (Herlitz Type) in German Black-Headed Mutton Sheep

SummaryThis report describes the microscopical, immunohistochemical and ultrastructural findings in the first ovine cases of the Herlitz type of inherited junctional epidermolysis bullosa. Sixteen German black-headed mutton lambs and one crossbred lamb had blisters and ulceration of the skin and mucous membranes in addition to alterations of the horn of the hooves. Microscopically, there was separation of the dermoepidermal junction, which was confirmed to be located in the lamina lucida of the basement membrane by electron microscopy. In areas of subepidermal splitting the hemidesmosomes were missing and in adjacent areas they appeared to be rudimentary and reduced in number. Immunohistochemistry for laminin 5 revealed a markedly reduced expression of this molecule on the dermal side of the blisters, while expression of collagen VII was normal.