DEFINITION/OVERVIEW

• Pemphigus: a group of autoimmune dermatoses characterized by varying degrees of ulceration, crusting, and pustule and vesicle formation
  
• Affects the skin and sometimes the mucous membranes
  
• Types: pemphigus foliaceus (PF), pemphigus vulgaris (PV), pemphigus erythematosus (PE), panepidermal pustular pemphigus/vegetans (PEP), canine benign familial chronic pemphigus (Hailey–Hailey disease), paraneoplastic pemphigus (PP)
  
• PV is the primary form involving the oral cavity (90% of patients showing oral signs), and is the focus of discussion for this chapter

ETIOLOGY/PATHOPHYSIOLOGY

• PV: second most common type, the most severe form (although rare, PEP may be more severe)
  
• Mediated by tissue-bound autoantibody deposition just above the basement membrane zone; results in deeper ulcer formation
  
• Severity of ulceration and disease: related to depth of autoantibody deposition (e.g., PF—autoantibody deposition in the superficial layers of the epidermis)

SIGNALMENT/HISTORY

• Dogs and cats
  
• Usually middle-aged to old animals

CLINICAL FEATURES

• Oral ulceration frequent and may precede skin lesions
  
• Ulcerative lesions, erosions, epidermal collarettes, blisters, and crusts
  
• More severe than PF and PE
  
• Affects mucous membranes, mucocutaneous junctions, and skin; may become generalized
  
• Axillae and groin areas often affected
  
• May have positive Nikolsky’s sign (new or extended erosive lesion created with lateral pressure is applied to the skin near an existing lesion)
  
• Anorexia, depression, and fever
  
• Variable pain and pruritis
  
• Secondary bacterial infections common

DIFFERENTIAL DIAGNOSIS

• Bullous pemphigoid
  
• Systemic lupus erythematosus
  
• Toxic epidermal necrolysis
  
• Drug eruption
  
• Mycosis fungoides
  
• Lymphoreticular neoplasia
  
• Ulcerative stomatitis causes, candidiasis
  
• Erythema multiforme

DIAGNOSTICS

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