Pemphigus

Andrea Marie Bernales-Mendoza, MD

Disease entities
included in this
group:

Pemphigus Vegetans

Pemphigus Vulgaris

Pemphigus Foliaceous

Pemphigus Erythematosus / Senear-Usher Syndrome

Paraneoplastic pemphigus

IgA Pemphigus

Subcorneal pustular dermatosis

Intraepidermal neutropilic dermatosis

Pemphigus
spectrum
More localized disease - Pemphigus
vegetans of Hallopeau
More extensive - Pemphigus vegetates
of Neumann
Finally More severe disease Pemphigus Vulgaris

Pemphigus
foliaceus
- sporadic, disease of mostly middle-aged
and older patients.
Localized disease - Pemphigus
erythematosus / Senear -Usher syndrome
Crusted erosions in seb derm distribution at
times with concurrent with more lupus like
discoid lesions with carpet-tack scale.
Demonstrates the cell surface staining
pattern classic for pemphigus, approx 30%
have (+)ANA titers, 80% (+)lupus band tests

Endemic PF in Brazil - Fogo selvagem

(wild fire)
occurs in children and young adults, in
genetically related family members,
altho not contagious

Mechanism of
Pathology
Acantholysis
Pemphigus Vulgaris Suprabasal layer
Pemphigus Foliaceus Granular layer

Antigen
Antibody
Interaction

Pemphigus Foliaceous Desmoglein 1

Pemphigus Vulgaris
Mucosal type Desmoglein 3
Muco-cutaneous Desmoglein 1 and 3
*Desmoglein 4 expressed in hair cortex and
superficial epidermis
Desmoglein 4 - has cross-reactivity to Desmoglein
1, hence can be seen in mucocutaneous PV and PF.

DIF: Intracellular
IgG

* Pemphigus Erythematosus : C3 and

IgG

IIF:
For PV: Monkey esophagus
For PF: Guinea pig esophagus or Human
skin

Desmoglein
compensation

The concept originates in the assumption

that autoantibodies against one desmoglein
isoform inactivate only that isoform and
that another isoform coexpressed in the
same area can compensate in adhesion.
--Mahoney MG et al: Explanation for the clinical and
microscopic localization of lesions in pemphigus foliaceus and
vulgaris. J Clin Invest 103:461, 1999

Desmoglein compensation also offers an

explanation for the differing sites of blister
formation in PV and PF, both in regard to
the histology (i.e., suprabasal or

Others
Neonatal Pemphigus Mother with PV.
Remits upon catabolism of maternal
antibodies.
Drug-induced pemphigus
Penicillamine and Captopril

Associated
Disease
Myasthenia gravis
Thymoma

*Still unusual
*Course of disease is independent from
each other

Treatment
Steroids

Adjuvants

Topical
Oral
IV

Azathioprine
Mycofenolate Mofetil
Cyclophosphamide
Dapsone

Others
Rituximab
Plasmapheresis
Pulse Steroid

 This review found insufficient information to

conclude which is the most effective and safest
treatment plan.
We found that mycophenolate mofetil appears to
be more effective than azathioprine in controlling
disease, although no difference was seen in
remission.
We found that taking azathioprine and
cyclophosphamide decreased the amount of
glucocorticoids required.
Topical epidermal growth factor decreased time
required for lesions to heal by 6 days (median).