Journal of the European Academy of Dermatology and Venereology

JEADV
ELSEVIER 5 (1995) 41-43

Case report
Pemphigus vulgaris during pregnancy -
a multistep triggered disease
Joseph Ophir, Anat Bialy-Golan, Ronnie Wolf, Sarah Brenner *
Department of Dermatology, Tel Aviv Sourasky Medical Center, Ichilov Hospital, and Sackler Faculty of Medicine, Tel Aviv University,
Tel Aviv, Israel

Abstract

Pemphigus vulgaris (PV) during pregnancy is exceedingly rare. Only 16 cases have been reported in
the English literature. Some of the etiologic fact,ors that may induce pemphigus are well known while
others are only suspected or unknown. We present a patient with pemphigus during her second
pregnancy and in whom several factors might have induced the disease.

Keywords: Pemphigus vulgaris; Pemphigus vulgaris during pregnancy

1. Introduction oral steroids; the disease disappeared after one

year and treatment was stopped. In 1990 she
Pemphigus vulgaris (PV) during pregnancy is became pregnant and remained disease-free
exceedingly rare [l]. It may occur for the first throughout the pregnancy and for 7 months post-
time or flare during pregnancy [2], as in our partum, at which time lesions began to appear
patient. The etiologic factors for the flare-up in intermittently, limited to the vulva. They were
the second pregnancy are discussed. treated topically only and did not require sys-
temic steroid treatment. A lesion developed in
1992 two months after dental treatment. In Au-
2. Case report gust 1992 an atypical nevus was excised from her
back under local anesthesia with lidocaine, and in
A 30-year-old Jewish Israeli pregnant woman September 1992 she was treated with
of Bulgarian-Turkish origin was admitted to hos- cephalosporines for an upper respiratory tract
pital with lesions on the scalp, face, chest, back, infection. Three weeks later there was exacerba-
limbs and oral mucosa. She had been diagnosed tion of the lesions in the vulva, which lasted for
with pemphigus vegetans in 1988 and treated with one year until September 1993 and for which she
was treated with topical steroids. She became
* Corresponding author. Department of Dermatology, Tel pregnant a second time in July 1993, and in
Aviv Sourasky Medical Center, 6 Weizman Street, Tel Aviv September the lesions spread to the scalp, face,
64239, Israel. Tel.: 972-3-6973356; Fax: 972-3-6979580. chest, back, limbs and oral muccosa.

0926-9959/95/$09.50 0 1995 Elsevier Science B.V. All rights reserved

SSDZ 0926-9959(95)00032-l
42 J. Ophir et al. /J. Eur. Acad.
Physical examination revealed no abnormality
and blood chemistry was within normal limits.
The lesions consisted of flaccid bullae which broke
rapidly, leaving erosions and crusts. Histological
examination showed intraepidermal split above
the basement membrane with acantholytic cells.
Direct immunofluorescence and indirect im-
munofluoresence demonstrated intracellular de-
posits of IgG and C, in the epidermis in the titer
of 1:400. Fetal ultrasound was normal. HLA alle-
les included DQWl, DRl, All, A28, B14, B35,
CW4 and BW6.
3. Discussion
The cause of pemphigus is unknown, although
numerous mechanisms, both intrinsic and extrin-
sic, have been proposed as inducing or triggering
the disease. Among the endogenous factors eth-
nic background is well known, with PV more
common in Jews and probably in people of
Mediterranean origin. There is a strong associa-
tion of certain HLA alleles with the disease,
including HLA-AlO, 25, 26, DR4 and DR6, DQl.
The HLA in our patient was DQWl, like the
typical pemphigus-associated alleles [3-51. Preg-
nancy is another factor implicated in the develop-
ment of pemphigus [21.
One of the main exogenous factors considered
responsible for the onset or exacerbation of pem-
phigus is drugs [6,7]: penicillamine and the broad
group of penicillin and cephalosporines [6-121
are the most common culprits, and the pyra-
zolones and ACE inhibitors 113-141 are also im-
plicated. Other suspected causative factors in-
clude viral and bacterial infections; preexisting
diseases like carcinoma, myasthenia gravis and
thymoma; physical agents like ultraviolet light,
ionizing radiation and burns; emotional stress;
and certain dietary antigens [15-211. Trauma was
noted in one case of pemphigus that developed at
the orifice of a surgical draining site on the chest
wall 2 months after surgery for lung carcinoma
[22]. Another report describes pemphigus erythe-
matosus exacerbated by allergic contact dermati-
tis to tincture of benzoin [231. Two other patients
Dematol. Venereol. 5 (1995) 41-43
developed pemphigus following cosmetic proce-
dures [24].
Factors that might have evoked or exacerbated
PV in our patient include pregnancy, cephalo-
sporines drug treatment, excision of an atypical
nevus, and dental treatment. Any one or combi-
nation of these factors could have played a role in
the development of the disease.
Induced pemphigus is a well-known phe-
nomenon. We have presented a rare case of
pemphigus in pregnancy in which several factors
are suspected of inducing or exacerbating the
disease in a multistep fashion.
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