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770 Pemphigus Complex

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PEMPHIGUS COMPLEX Pemphigus foliaceus in 7-year-old terrier cross: close-up
view of lesions affecting the feet (pododermatitis). Note almost complete loss of
normal footpad architecture. (Courtesy Dr. Caroline de Jaham.)
PEMPHIGUS COMPLEX Pemphigus erythematosus in 3-year-old collie. Note
superficial erosions and crusts on bridge of nose. Depigmentation of the nasal
planum is also observed. (Courtesy Dr. Caroline de Jaham.)
• PV: a deep and severe form of pemphigus
presenting with transient, flaccid vesiculo-
pustules that are rapidly replaced by large
erosions and ulcers of the mucosal surfaces cutaneous lupus erythematosus, mucocuta- refractory to initial systemic glucocorticoids
and mucocutaneous junctions. Affected areas neous lupus erythematosus, systemic lupus should be referred to a dermatologist.
include oral cavity (>70% of cases), concave erythematosus
pinnae, nasal planum, lip margins, genitalia, Acute General Treatment
and anus. Erosions of the nail beds are Initial Database Systemic glucocorticoids alone induce remission
reported in 14% of cases. A milder form of • Cytologic exam of content of intact pustules in most cases of PE and in one-third of cases
PV is reported where lesions are restricted to or exudate under a crust may strongly suggest of PF in dogs and most cats. Other forms of
one body area (e.g., nail beds, nasal planum, pemphigus. Intact neutrophils, various pemphigus are more refractory. Response should
oral cavity). numbers of eosinophils, and clusters of be seen within 10-14 days.
• PVeg and PPP: severe erosions and ulcer- acantholytic keratinocytes can be seen in • Dogs (initially): prednisone/prednisolone
ations of the oral cavity, nose, vulva, and most cases, but skin biopsies are still required 2-4 mg/kg PO q 24h; these drugs can also be
haired skin because other disorders may have similar used as high-dose pulse therapy (e.g., 10 mg/
findings. kg PO q 24h for 3 consecutive days followed
Etiology and Pathophysiology • CBC, serum biochemistry profile: nonspecific by reduced daily dosage [<2 mg/kg]).
Intraepithelial acantholysis (loss of intercel- changes, mild to moderate leukocytosis with • Cats (initially)
lular cohesion between keratinocytes) leads to neutrophilia, mild nonregenerative anemia, ○ Prednisolone 2-4 mg/kg PO q 24h, or
vesicles/pustules formation due to autoantibod- and hypoalbuminemia ○ Dexamethasone 0.2-0.4 mg/kg PO q 24h,
ies (IgG) binding to components of desmosome or
complex. Advanced or Confirmatory Testing ○ Triamcinolone 1-2 mg/kg PO q 24h
• Skin biopsies: confirmatory test. Histopatho-
DIAGNOSIS logic findings of acantholysis with pustule Chronic Treatment
formation are diagnostic. Epidermal location • Maintenance dosages of drugs are the
Diagnostic Overview of lesions is related to depth of autoantibody lowest doses that result in a stable degree
The diagnosis of PF, the most common form deposition: subcorneal and intragranular of disease that is acceptable to the owner and
of pemphigus, is suspected in a dog or cat layers in PF and PE, suprabasilar level in clinician.
presented for evaluation of a bilaterally sym- PV and PPP, and panepithelial in PNP. • When combinations of medications are used,
metrical, progressive, crusting, and pustular • Immunohistochemical and immunofluores- the first ones to be tapered are those with
dermatitis that does not typically respond to cent analysis of skin biopsies or serum may the greater likelihood of adverse effects.
appropriate systemic antibiotic therapy. The be helpful but depends on the sensitivity • The glucocorticoid dosage is slowly reduced
definitive diagnosis requires skin biopsies of the methods being used; not routinely on a daily basis over 30-40 days after remis-
showing histopathologic changes of acantholysis. performed. sion of active skin lesions is attained (2-4
weeks). Lowering to an alternate-day regimen
Differential Diagnosis TREATMENT with an ideal maintenance dose of 1 mg/kg
• PF, PE, and PNP: bacterial folliculitis, q 48h of prednisone/prednisolone or less is
demodicosis, dermatophytosis, eosinophilic Treatment Overview the ultimate goal.
folliculitis and furunculosis, cornification The treatment goal is to implement immu- • Concurrent immunosuppressive drugs are
disorders, cutaneous (discoid) and systemic nosuppressive therapy after a firm diagnosis used in conjunction with glucocorticoids
lupus erythematosus, leishmaniosis, drug of pemphigus is made that can induce and (initially or added later) to achieve and main-
eruptions maintain remission of skin lesions without tain remission with fewest/no glucocorticoid
• PV and PPP: bullous pemphigoid, epi- significant or life quality–altering side effects. adverse effects.
dermolysis bullosa acquisita, erythema This is best achieved by using combination ○ Azathioprine 2.2 mg/kg PO q 24-48h in
multiforme, toxic epidermal necrolysis, drug therapy. The therapeutic modalities and conjunction with prednisone/prednisolone;
Stevens-Johnson syndrome, epitheliotropic response to treatment vary with the type of first choice in dogs, contraindicated in
lymphoma, ulcerative stomatitis, vesicular pemphigus and the species treated. Cases cats, or

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