Blistering eruption on the chest of a 30-year-old pregnant
Haider K. Bangash, MD, Shaily Patel, MD, Keyoumars Soltani, MD, Christopher R. Shea, MD
and Aisha Sethi, MD
Section of Dermatology, University of
Chicago Medical Center, IL, USA
Haider Khan Bangash, MD
2219 Periwinkle Lane
What is your diagnosis?
A previously healthy 30-year-old G2P1 African-American
woman, at 11 weeks’ gestation, presented with a three-
week history of painful and pruritic blisters on her trunk
and upper extremities. She had no drug allergies and was
taking only prenatal vitamins.
Physical examination revealed multiple intact flaccid
bullae and large erosions. On closer inspection, there
were smaller vesicles arranged in an annular configuration
and violaceous patches with overlying crust (Fig. 1). Mul-
tiple erosions were present on the oral mucosa.
Laboratory data revealed a normal blood count and
metabolic panel. Her antinuclear antibody titer was
slightly elevated at 1 : 160. However, her anti-double-
stranded DNA antibodies were normal.
Two punch biopsy specimens were submitted to derma-
topathology (Figs. 2 and 3).
Figure 2 Biopsy specimen of the skin (hematoxylin and eosin
stain; original magnification · 200). Vesiculopustules within
the epidermis, containing abundant neutrophils and
eosinophils. The intact epidermis exhibits spongiosis. Focal
acantholysis and dyskeratosis are seen. The dermis has a
superficial and deep perivascular infiltrate of lymphocytes
admixed with eosinophils
Figure 1 Photograph of the skin lesions. Vesiculopustules in
an annular configuration with some areas of hyperpigmenta-
tion as well
Figure 3 Direct immunofluorescence IgG (original magnifica-
tion · 200). Antidesmoglein IgG autoantibodies are shown
distributed in an intercellular pattern
ª 2012 The International Society of Dermatology International Journal of Dermatology 2012, 51, 1429–1431
Herpetiform pemphigus (HP).
Histopathological findings and clinical course
Hematoxylin and eosin stain slides showed intraepider-
mal vesicles and pustules with abundant neutrophils and
eosinophils, as well as suprabasilar acantholysis, consis-
tent with pemphigus vulgaris (PV). Direct immunofluo-
rescence showed florid intercellular deposition of IgG
and C3. Indirectimmunofluorescence
monkey esophagus substrate was positive for IgG pem-
phigus autoantibodies observed in an intercellular bind-
In view of the clinical, histopathological, and immuno-
fluorescence findings, a diagnosis of HP was made.
The patient was started on prednisone (1 mg/kg/d) for
two weeks and subsequently discharged on a long steroid
taper with complete resolution of lesions and residual
postinflammatory hyperpigmentation. Unfortunately, at
eight months’ gestation, the fetus died because of cord
entanglement, thought to be an obstetrical complication
unrelated to HP.
HP was first described by Jablonska et al.1in 1975 and is
thought to be a clinical variant of the pemphigus group
of diseases. It usually presents with clinical features of
dermatitis herpetiformis and the immunopathological
findings of pemphigus.1There is some controversy over
whether it is a distinct clinical entity or whether it is a
variant of either PV or pemphigus foliaceus.2
Our patient showed multiple intact flaccid bullae and
erosions suspicious for PV. However, on closer exami-
nation, the distinct vesiculopustules arranged in an
annular, ‘‘herpetiform’’ configuration
diagnosis of HP. Additionally, the patient’s history of
pruritus was consistent with HP.3,4
patient showed mucosal erosions, which are rarely a
feature of HP.
Histopathology typically shows the presence of eosino-
philic spongiosis, intraepidermal vesicles, polymorphonu-
clear infiltrate, and occasional acantholytic cells.3,5In our
patient, there was significant acantholysis, a feature not
typical to HP.3
The direct immunofluorescence (DIF) findings are simi-
lar to PV. DIF of perilesional skin and indirect immuno-
fluorescence (IIF) shows the presence of antidesmoglein
(Dsg) IgG antibodies.3Most cases described have anti-
Dsg1 antibodies and occasionally anti-Dsg3 antibodies
directed towards the keratinocyte cell surface.6Other
reports have described HP with antibodies to both Dsg1
and desmocollin-III (Dsc-3),7antibodies to desmocollin-1
(Dsc-1),8and a case with absent Dsg1 and Dsg3 anti-
bodies.9This illustrates the variability of the type of
circulating antibodies seen in HP.
Antibody titers detected through enzyme-linked immu-
nosorbent assay (ELISA) and immunoblotting techniques
correlate with severity of disease in HP.7Unfortunately,
an ELISA was not done in our patient.
In this case, the discrete annular arrangement of vesi-
cles, histopathological findings of eosinophilic spongiosis
and intraepidermal vesicles, immunofluorescence showing
the presence of intercellular IgG deposition, and existence
of circulating IgG antibodies were key clues to the diag-
nosis of HP.1,10
The distinction of HP from PV is important as pemphi-
gus IgG antibodies can cross the placenta, and numerous
cases of neonatal pemphigus have been described in
women with PV.11Most infants tend to have a mild self-
limiting cutaneous eruption, and the cumulative perinatal
mortality rate is reported to be close to 12%.11
A literature search with ‘‘herpetiform pemphigus’’,
‘‘pemphigus herpetiformis’’, and ‘‘pregnancy’’ as key
words did not reveal reports of HP associated with preg-
nancy. PV, on the other hand, has been rarely reported in
pregnancy, mostly in the second and third trimesters. To
date, only 38 reports have been published in the litera-
ture. Surprisingly, there are fewer than 10 reports of PV
arising during the first trimester.11,12The low incidence
of PV in pregnant women could be due to the later age of
onset (40–60 years) when fertility rates are lower. Fur-
thermore, in previous reports, most women had extensive
disease and required high doses of prednisone. Thus, it
may be inferred that only very extensive disease is
Vesiculobullous eruptions in pregnancy that may mimic
HP include pemphigoid gestationalis, pemphigus folia-
ceus, PV, dermatitis herpetiformis, bullous pemphigoid,
and linear IgA bullous dermatosis.3
Systemic corticosteroids, dapsone, and other steroid-
sparing agents have been shown to be effective in the
treatment of HP.3,5Though not used in our patient, dap-
sone is thought to be the drug of choice in treating
HP.5,13In addition, azathioprine has been used safely in
pregnant women with PV.11Our patient responded to a
two-week course of oral prednisone and has not had a
recurrence of symptoms since.
A clear diagnosis of HP is often difficult because of sig-
nificant overlap in clinical and immunopathological find-
ings with other vesiculobullous diseases.5,9
because of the distinct clinical morphology of lesions and
supportive immunopathological findings, a diagnosis of
HP was made.
International Journal of Dermatology 2012, 51, 1429–1431
ª 2012 The International Society of Dermatology
Clinicopathological challenge Blistering eruption on the chest of a pregnant womanBangash et al.
References Download full-text
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ª 2012 The International Society of DermatologyInternational Journal of Dermatology 2012, 51, 1429–1431
Bangash et al. Blistering eruption on the chest of a pregnant womanClinicopathological challenge