Deep pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, cats and horses: a compreh
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REVIEW
Open Access
Deep pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, cats and horses: a comprehensive review Heng L. Tham1 , Keith E. Linder2,3
and Thierry Olivry4*
Abstract Pemphigus is the term used to describe a group of rare mucocutaneous autoimmune bullous diseases characterized by flaccid blisters and erosions of the mucous membranes and/or skin. When the autoantibodies target desmosomes in the deep layers of the epidermis, deep pemphigus variants such as pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus develop. In this article, we will review the signalment, clinical signs, histopathology and treatment outcome of pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus in dogs, cats and horses; where pertinent, we compare the animal diseases to their human homologue. Canine, feline and equine pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus have many features similar to the human counterpart. These chronic and often relapsing autoimmune dermatoses require aggressive immunosuppressive therapy. In animals, the partial-to-complete remission of pemphigus vulgaris and pemphigus vegetans has been achieved with high dose glucocorticoid therapy, with or without adjunct immunosuppressants; the prognosis is grave for paraneoplastic pemphigus. Keywords: Pemphigus, Vulgaris, Vegetans, Paraneoplastic, Desmoglein, Suprabasal, Acantholysis, Canine, Feline, Equine
Background Desmosomes are intercellular adhesion structures that are well developed in tissues that experience considerable mechanical stress, such as the epidermis, mucosae, and myocardium. Desmosomes maintain cell-to-cell structural integrity, anchor internal cytoskeletal intermediate filaments, and participate in many functions, such as signaling, differentiation, inflammation and carcinogenesis [1]. Diseases that disrupt or weaken desmosomes may lead to loss of cell-cell adhesion, a process called acantholysis [1]. In animals, the spectrum of skin * Correspondence: [email protected] 4 Department of Clinical Sciences, College of Veterinary Medicine, North Carolina State University, Raleigh, NC, USA Full list of author information is available at the end of the article
diseases that can lead to acantholysis is wide, ranging from genetic acantholytic dermatoses (i.e., suprabasal epidermolysis bullosa simplex in cattle) to infectious proteolytic acantholytic dermatoses (i.e., exfoliative superficial pyodermas in dogs), and finally to autoimmune acantholytic dermatoses (i.e., pemphigus variants) [2]. Pemphigus is the term used to describe a group of rare mucocutaneous autoimmune bullous diseases characterized by flaccid blisters and/or pustules, with secondary erosions of the mucous membranes and/or skin [3, 4]. In these diseases, autoantibodies (AA) most often target specific epidermal and/or mucosal desmosomal adhesion proteins required to maintain the strength of keratinocyte intercellular adhesion [5], which leads to a loss of
© The Author(s). 2020 Open Access Thi
