Laboratory Diagnosis: 1. Cytological Examination (Tzanck Smear)

Laboratory Diagnosis
1. Cytological examination (Tzanck smear)

• useful for the rapid demonstration of
acantholytic keratinocytes of the spinous layer
2. Histopathological examination
• to identify the level of blister cleavage (suprabasal or intramalpighian) in
order to diagnose pemphigus
• to differentiate with other subepidermal bullous lesions
• For biopsy : choose a recent blister (less than 24 hours of appearance) that
fits inside a 4 mm punch or a small fusiform excision. If this is not possible,
a perilesional area should be biopsied. Then should be fixed in 10% formalin
3. Direct immunofluorescence examination

• The identification of IgG and C3 autoantibodies directed against the cell
surface of keratinocytes is considered as a “gold standard” for the
differential diagnosis of PV.
• In DIF, patients’ skin or mucosa may be used to demonstrate IgG and C3
deposits with intercellular distribution
4. Indirect immunofluorescence examination

• assists in the diagnosis of PV and allows the detection of circulating
autoantibodies. Patient serum is diluted from 1:20 and incubated with the
substrate (normal skin of another individual or a apecimen of monkey
esophagus).
• The reaction is revealed by anti-human (IgG) secondary antibodies
produced in animals and conjugated to fluorescein isothiocyanate.
5. Immunohistochemical examination
• combination of immunological and histological methods for the detection
of specific antigens in tissues or cells (immunocytochemistry), based on
the identification of the antigen-antibody complex
6. Serological Diagnosis
• ELISA is a very sensitive and specific method that allows detection
of IgG anti-Dsg1 (mucocutaneous PV) and anti-Dsg3 (mucosal PV)
autoantibodies in over 90% of patients using recombinant Dsg1 and
Dsg3.
• Immunoblotting and immunoprecipitation
Treatment
Systemic Treatment
1.Corticosteroid
Oral Administration
Prednisone, Prednisolone and deflazacort
Doses : 40 to 60 mg/day (prednisone) for patients with mild
PV and 60 to 100 mg/day for more severe conditions
Or full doses : 1 to 2 mg/kg/day oral
Pulse Therapy
Methylprednisolone (1 g/day IV) and dexamethasone (300
mg/day IV) are used, both for three consecutive days.
Treatment
Systemic Treatment
2. Adjuvant Drugs
 Azathioprine : 100-200 mg/day (1 to 3 mg/kg/day), orally, divided into two
doses
 Mycophenolate mofetil/sodium : 2-3g/day, divided into two doses.
 Rituximab
 Cyclophosphamide : orally (1 to 3 mg/kg/day) or intravenously
 Methotrexate : 10 to 20mg/week in case of therapeutic failure of other
adjuvants
 Dapsone : 50 to 200mg/day, orally
 Cyclosporine : 3 to 5mg/ kg/day, VO or IV
 Intravenous human immunoglobulin (IVIG) : 0.4g/kg/day for five days,
always as an adjunct to corticosteroid therapy once a month.
 Anti-TNF drugs
Treatment
Systemic Treatment
3. Plasmapheresis/immunoadsorption
In order to remove pathogenic autoantibodies from the circulation.
It is recommended that it be associated with corticosteroids and
immunosuppressants (e.g., pulse therapy with methylprednisolone and
cyclophosphamide) in monthly cycles for up to one year
4. Systemic antibiotic therapy

It is indicated only in cases with clinical and/or laboratory evidence of
secondary infection, never prophylactically. Preferably, the choice of this
treatment should be guided by culture and antibiogram of blood and skin
samples.
Treatment
Topical Treatment
• Always adjuvant to systemic treatment, topical treatment of PV lesions

aims to reduce pain and prevent secondary infection.
• It is usually performed with corticosteroid and/or antibiotic creams.
 Tacrolimus use, particularly in facial lesions.
 Potassium permanganate (1:10,000 or 1:20,000)
 Chlorhexidine.
 Clobetasol dipropionate may be used in the oral mucosa.
 Triamcinolone acetonide (10 mg/mL) may be used in the form of
intralesional injection for refractory skin lesions (e.g., pemphigus
vegetans)
Treatment
Future Therapies
• New anti-B-cell immunobiological drugs are being investigated in clinical

research regarding their efficacy, safety, and cost for patients with PV.
• These include veltuzumab (anti-CD20 subcutaneous administration
antibody), obinutuzumab, ofatumumab, ocaratuzumab, PRO 121921,
anti-BAFF, and anti-BAFF-R
Treatment Plan
Induction of remission
• At this phase, the objective is to control the condition, interrupting the

appearance of new bullous lesions and promoting re-epithelialization of
the existing lesions.
• Corticosteroids are the most effective and quickest therapeutic option in
PV control, being essential at this stage.
• It may take several weeks to achieve control (on average, three weeks)
and dose increasing may be required for this to occur.
• Adjuvant medications may be initiated at this stage
• Drug doses should be maintained until the condition is controlled, defined
as re-epithelialization of approximately 80% of skin and mucosal lesions,
and no new lesions for at least two weeks
Treatment Plan
Maintenance of remission
• The drug doses are slowly reduced to minimize the side effects.
• The ultimate goal is to keep the disease controlled with a prednisone dose
of up to 10mg/day.
Treatment withdrawal
• Complete remission of the disease is possible, and has been

observed in 38%, 50%, and 75% of the cases after three, five, and
ten years of diagnosis, respectively.
• premature withdrawal should be avoided, being rarely possible
before one year.
Prognosis
• Before the advent of corticosteroids and immunosuppressants, the two-

year mortality rate of PV was 50%.
• Currently, the mortality rate is approximately 10%. The main cause of
death in PV patients is septicemia.
• It is often possible to achieve total disease control, which allows
withdrawing the medication, but patients should be kept under
observation, since relapses are frequent.

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Laboratory Diagnosis: 1. Cytological Examination (Tzanck Smear)

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Laboratory Diagnosis

1. Cytological examination (Tzanck smear)

3. Direct immunofluorescence examination

4. Indirect immunofluorescence examination

4. Systemic antibiotic therapy

• Always adjuvant to systemic treatment, topical treatment of PV lesions

• New anti-B-cell immunobiological drugs are being investigated in clinical

• At this phase, the objective is to control the condition, interrupting the

• Complete remission of the disease is possible, and has been

• Before the advent of corticosteroids and immunosuppressants, the two-

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