CHAPTER

29

Pemphigus
A B

C D

Fig. 29.8 Pemphigus foliaceus. A,B Multiple erosions arising on an erythematous base as well as the characteristic scale-crust; the latter can vary in thickness. A
 

common site of involvement is the trunk. C As the disease progresses, the lesions become confluent, but because the vesicles are fragile and rupture easily, only
erosions with scale-crust are observed. D The scales have been likened to cornflakes.

Fig. 29.9 Pemphigus erythematosus. Erythematous plaques with scale-crust
and erosions on the nose and malar area of the face. Courtesy, Ronald P Rapini, MD.
appear in the malar region of the face (Fig. 29.9) and in other “seb-
orrheic” areas. Originally, the term “pemphigus erythematosus” was
introduced to describe patients with immunologic features of both
lupus erythematosus (LE) and pemphigus, i.e. in vivo IgG and C3
deposition on cell surfaces of keratinocytes as well as the basement
membrane zone, in addition to circulating antinuclear antibodies45.
However, only a few patients have been reported to actually have the
two diseases concurrently.
Herpetiform Pemphigus
Most patients with herpetiform pemphigus have a clinical variant of
pemphigus foliaceus and the remainder may have a variant of pem-
phigus vulgaris. This disorder is characterized by: (1) erythematous
urticarial plaques and tense vesicles that present in a herpetiform
arrangement; (2) eosinophilic spongiosis and subcorneal pustules
with minimal or no apparent acantholysis histologically; and (3) IgG
autoantibodies directed against the cell surfaces of keratinocytes46.
The target antigen is Dsg1 in most cases and Dsg3 in the remain-
der47. Some patients with herpetiform pemphigus will have fea-
tures of pemphigus foliaceus or vulgaris during the course of their
disease, and some patients will evolve into having pemphigus folia-
ceus or vulgaris. It is assumed that the pathogenic blister-inducing
activity of the IgG autoantibodies in herpetiform pemphigus might
be weaker than that seen in classic forms of pemphigus. Although
often clinically less severe than pemphigus vulgaris, the course may
be more chronic48.
Drug-Induced Pemphigus
There are sporadic cases of pemphigus associated with the use of drugs,
in particular penicillamine and captopril49. In patients receiving penicil-
lamine, pemphigus foliaceus is seen more commonly than pemphigus
vulgaris, with a ratio of approximately 4 : 1. Although most patients
with drug-induced pemphigus are shown to have autoantibodies against
the same molecules involved in sporadic pemphigus, evidence suggests
that some drugs may induce acantholysis without the production of 501
antibodies. Both penicillamine and captopril contain sulfhydryl groups