DOMINANTLY INHERITED EPIDERMAL ACANTHOLYSIS IN DOGS, SIMULATING HUMANBENIGN FAMILIAL CHRONIC PEMPHIGUS (HAILEY-HAILEY DISEASE)

We report on dominantly inherited epidermal acantholysis in three dogs , a sire and two female offspring. The skin lesions were characterized by hairless, hypertrophic plaques. Histopathologically, these lesions showed epidermal hyperplasia with individual enlargement of keratinoc ytes, extensive acantholysis and minimal dyskeratosis. Ultrastructural analysis revealed that attachment plaques of desmosomes were stilt in tact while some tonofilaments were detached from them in early lesions ; there were well-developed microvilli at dissociated cell surfaces. T he data imply that these animals have undergone a process similar to h uman benign familial chronic pemphigus (BFCP). Immunohistochemical exa mination revealed that staining for E-cadherin and actin variably rema ined in dissociated keratinocytes. Focal intracellular staining for de smosomal glycoproteins and desmosomal proteins were observed within th e dissociated keratinocytes. This dominantly inherited acantholytic di sease in dogs could be a useful animal model for investigating the pat hogenesis of BFCP in humans.