Author Information
Save S*, Nayak C**, Tambe S***.
(*Registrar, **Professor & Head,
***Assistant Professor, Department of Dermatology, Leprology and Venereology, B.Y.L. Nair Hospital & Topiwala National
Medical College ,
Mumbai , India
Abstract
Pemphigoid gestationis is an autoimmune
subepidermal bullous disorder typically seen in the second or third trimester
of pregnancy, and has very rarely been reported in the postpartum period. The
clinical, histopathological and immunopathological features are similar to
those of pemphigoid group of disorders. We would like to report a case of PG
which presented during the immediate post partum period.
Introduction
Pemphigoid gestationis (PG) is a rare,
self-limited autoimmune bullous disorder that presents mainly in the second or
third trimester of pregnanacy or the immediate postpartum period.[1]
PG usually presents as urticarial plaques topped with vesicles that subsequently develop into blisters. It
is usually associated with severe pruritus and the periumbilical area is
commonly the first site to be affected. The face, mucous membranes, palms and
soles are usually spared.[2]
Case Report
A 35 year old housewife presented with
multiple red raised lesions topped with fluid filled lesions all over her body
of 8 days duration. She indicated that these lesions started appearing 2 days
following the delivery of her fourth child by a cesarean section. The lesions,
which initially appeared as multiple reddish raised lesions on her limbs,
abdomen and neck, were associated with severe pruritus and within 24 hours she
noticed the development of multiple fluid-filled blisters over these reddish
lesions. The lesions were increasing in size and number since then. She did
give history of similar lesions following the delivery of her second child in
2005, but the lesions had resolved spontaneously in the next 15 days. The
patient did not have any other significant medical history.
Clinical examination revealed the
presence of multiple erythematous urticarial plaques topped with tense vesicles
and crusted erosions. (figures 1a & 1b) Lesions were seen mainly over the
neck, abdomen, back, upper and lower limbs. Nikolskiy and bulla spread sign
were negative. Both the genital and the oral mucosa as well as the palms and
soles were spared. Systemic examination was found to be within normal limits.
She had delivered a healthy baby by
a cesarean section and the baby did not
show any evidence of skin or mucosal lesions.
Routine hemogram, liver and renal
function tests and biochemical tests produced normal results and the thyroid
hormones were all within normal ranges.
A provisional diagnosis of pemphigoid
gestationis was made from these findings.
Histopathology: Two punch biopsy
specimens were obtained from the leg from the lesional and perilesional skin
for hematoxylin (H) and eosin (E) stain and direct immunofluorescence (DIF)
study, respectively. H & E staining revealed a subepidermal split with
plenty of eosinophils along with neutrophils in the blister cavity. There was
evidence of dermal edema with mixed inflammatory infiltrate in the dermis (figures
2a and 2b). DIF demonstrated a linear deposition of C3 along the basement
membrane zone. (figure 3). Thus the diagnosis of Pemphigoid gestationis was
confirmed.
Figure 1a. Urticarial plaques on the back
topped with vesicles, erosions and crusts.
Figure 1b. Closer view of the vesicles (white arrow)
Figure 2a. Histopathology of skin showing the presence of subepidermal blister. (10X, H & E)
Figure 2b. Blister cavity filled with eosinophils
(white arrows). (40X, H & E)
Figure 3. Linear deposition of complement C3 along basement membrane zone
seen on Direct Immunofluorescence of
perilesional skin biopsy.
Figure 4. Post treatment
photograph showing resolution of the lesions.
Treatment: The patient was treated with
daily 30 mg of oral prednisolone along with 100 mg of dapsone and
antihistamines. There was marked regression in the number of lesions after 1
week. The prednisolone was gradually tapered over the next few weeks to 5 mg
per day. She had a relapse after about 3 weeks, with few lesions appearing over
extremities and the dose was increased to 10 mg and maintained for the next two
weeks following which there was significant improvement, with resolution of
existing lesions and no appearance of new blisters. (figure 4)
Discussion
Apart from its association with pregnancy
and puerperium, PG can also very rarely occur in association with trophoblastic
tumors like choriocarcinoma and hydatidiform mole. It tends to recur in subsequent
pregnancies, with usually earlier onset and increasing severity. Rarely (5%) a
pregnancy may not be affected and passed over ("skip pregnancies").[2,3]
The etiology of PG is not entirely
understood. It is presumed that auto-antibodies are formed against the
placenta, which then cross-react with certain antigens on the basement membrane
of the skin. This immune response is mainly directed against the NC16A
(noncollagenous) domain of the bullous pemphigoid antigen with a molecular
weight of 180 kDa, BP180. Less commonly IgG autoantibodies to BP230 are also
present.[4]
Diagnosis is
confirmed by the following factors:
(a)
Skin biopsy
showing the presence of subepidermal blister with eosinophils within blister cavity during pregnancy or in the
immediate post-partum period.
(b)
DIF reveals
the linear accumulation of complement C3 and IgG in the basement membrane zone
at the interface of epidermis and dermis.
Measurement of serum BP180 antibody titres correlates
with the degree of disease severity.[5,6]
Most patients
undergo spontaneous regression over weeks to months postpartum. Recurrent
disease flares can occur from 2 weeks up to 12 years postpartum.[2]
Recurrence with menstruation or with subsequent use of oral contraceptives has
been reported. Fetal risks may also be present and include miscarriages,
prematurity, low birth weight babies, transient erythema and blistering.[7]
For mild symptoms, the current guidelines recommend
the use of topical potent or very potent corticosteroids combined with the use
of oral histamines.[8]
Oral corticosteroids form the first-line systemic
therapy of severe pemphigoid gestationis.[8,9] Cyclosporine,
tetracycline, cyclophosphamide, azathioprine, dapsone and rituximab are some ot
the other treatment options.[10] Plasmapheresis, immunoadsorption
and intravenous immunoglobulin G-infusion, have in some cases been used to
treat PG prior to delivery.[11,12,13,14]
References
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