Deep pemphigus (pemphigus vulgaris, pemphigus vegetans and paraneoplastic pemphigus) in dogs, cats and horses: a comprehensive review

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.

A case report of the beneficial effect of oclacitinib in a dog with pemphigus vulgaris

“…11 Although IgG antibodies against DSG3 have been identified in cases of canine PV, it also has been proposed that IgG-independent factors such as helper T-cell (Th)2 cytokines, tumour necrosis factor a and Fas ligand also may play a role in the pathogenesis of acantholysis in PV. 3 An in vitro study on the effects of oclacitinib on T-cell proliferation and cytokine production, found that oclacitinib significantly reduced the secretion of clonal activator cytokines [interleukin (IL)-2, IL-15], proinflammatory cytokines (interferon-gamma, IL-18) and the regulatory cytokine IL-10; tumour necrosis factor a and IL-6 production was inhibited mildly. 12 This effect was seen only when oclacitinib was used at much higher concentrations (3-4 mg/kg) than the labelled 0.4-0.6 mg/kg once daily dose.…”

“…1,2 Treatments that often are used to treat PV in the dog include glucocorticoids, azathioprine, ciclosporin, chlorambucil, aurothioglucose, heparin and doxycycline. 3 A recent review of deep pemphigus, 3 found that of the reported cases of PV in the veterinary literature, clinical remission (CR) was achieved in only 65% (26 of 40) of treated dogs. Spontaneous remission without treatment was reported in only one of 40 dogs (3%).…”

Pemphigus vulgaris (PV) in a miniature schnauzer treated with a combination of glucocorticoids, ciclosporin, chlorambucil and oclacitinib

“…Prognosis is guarded at best as clinical remission is difficult to achieve. Relapses with reoccurrence of painful lesions are frequent ; and so are drug side-effects 1 . > Treatment relies on immunomodulation, either with glucocorticoids alone or with steroid sparing agents that are less commonly used as sole therapy 2 .…”

Blefarite secundária a pênfigo foliáceo em cão: relato de dois casos / Blepharitis secondary to pemphigo foliaceous in dog: report of two cases

“…Poderíamos ter como principal diagnóstico diferencial os pênfigos profundos (pênfigo vulgar, pênfigo vegetante e pênfigo paraneoplásico). Nesses casos os autoanticorpos têm como alvo os desmossomos nas camadas profundas da epiderme e na histopatologia encontraríamos acantólise suprabasal (Tham et al, 2020). Na pele, desmogleína-1 é expresso em toda a epiderme, porém mais intensamente nas camadas superficiais, enquanto desmogleína-3 é expresso nas camadas mais profundas, ou seja, camada basal (pênfigo profundo).…”