Pemfigoid

Gestasional

Suman Jaro (2016-84-029)

Supervisor:
Dr. Rita S. Tanamal, Sp.KK
PRESENTED AS ASSIGNMENT TO FULFILL REQUIREMENT OF CLINICAL CO-
ASSISTANCE IN DEPARTEMENT OF DERMATOVENEOROLOGY RSUD DR. M.
HAULUSSY MEDICAL FACULTY OF PATTIMURA UNIVERSITY
AMBON
2017
INTRODUCTION
1. Benign dermatoses  normal
physiological / hormonal changes;
2. Expansion of pre-existing dermatoses
due to hormonal changes;
3. Some pregnancy-specific dermatoses
can occur.
 CLASSIFICATION

Ambrus-Rudolph (2006)
- Gestational pemphigoid (PG),
- Pregnancy polymorphic eruptions (PEP),
- Intrahepatic cholestasis of pregnancy (ICP), and
- Atopic pregnancy eruption (AEP).
DEFINITION
Gestational pemphigoid is an
autoimmune disease with a very pruritic
bulusic bulusic eruption, which occurs during
pregnancy or other conditions such as
trophoblastic tumor, hydatidiform mole,
choriocarcinoma.
 EPIDEMIOLOGY

France, Kuwait and Germany (2004 and 2009)  0.5 to

2.0 cases per 1 million people.
Epidemiological data 1 of about 40,000 - 50,000
pregnancies.
Campbel SM et al (2011)  1: 7,000 pregnancies
 ETIOPATOGENESIS
- Immune response to the placenta.
- Circulation of Ig G antibodies and complement
with epithelial amnion of placental tissue and basal
membrane of the skin.
- Autoimmune responses Precipitation of immune
complexes, complement activation, eosinophil and
degranulation chemosates respectively  Causes
tissue damage and blister formation.
 CLINICAL SYMPTOMS
 Clinical features: Pruritus and annular plaques,
followed by vesicles and eventually large tense bulls
with an erythematical background.
 Place of eruption: periumbilical area, spread to
other belly and, even to thigh, palms, and soles of
feet.
 CLINICAL SYMPTOMS
 Appears in the second or third trimester of
pregnancy, (first trimester and postpartum
period). Remission occurs in the last month of
pregnancy or first 3 months after delivery,
but the lesions may persist for several weeks,
months, up to many years from the
postpartum.
DIAGNOSIS
 Histopathology  Blisters of subepidermal
vesicles in lamina lucida, basal cell
necrosis at the tip of the dermal papilla
and perivascular infiltration and skin
inflammation consisting of lymphocytes,
eosinophils and histiocytes.
DIAGNOSIS
 Immuniflureence  Accumulation of C3
on the basement membrane of the
epidermal and dermis interfaces. IgG is
positive.
 Serology ELISA  BP180 serum.
 DIAGNOSIS

 Clinical
evaluation, histologic findings, and direct
immunofluorescence (DIF) as well as serologic
examination.
DIFFERENSIAL DIAGNOSIS
 Atopic pregnancy eruption (AEP),
 Pregnancy polymorphic eruption (PEP)
and
 Intrahepatic cholestasis of pregnancy
(ICP).
 TERATMENT
 Mild symptoms: Topical corticosteroids and
systemic antihistamines.
 Symptoms Weight: Systemic corticosteroids
(prednisolone 20-30 mg / day  More severe
require prednisolone 40-80 mg / day)
PROGNOSIS
 PG self-healing postpartum,
 Can last for many years before complete
resolution.
 Rare cases  The disease develops bullous
pemphigoid.
 Experiencing recurrence in pregnancy with
more severe symptoms, with earlier onset.
 Complications of the fetus Primary labor
and low birth weight or small infants - against -
gestational age.
 CONCLUSION
 Gestational pemphigoid is an autoimmune disease that
is very pruritic bulusic eruption (pruritus), develops in
relation to pregnancy or other rare events such as
trophoblastic tumor, hydatidiform mole,
choriocarcinoma. The incidence of gestational
pemphigoid ranges from 1: 10,000 to 1: 60,000
pregnancies.
 PG is an autoimmune disease, mediated by antibodies.
The placenta and connective tissue contain a foreign
paternal Ag tissue against the maternal immune system,
but the maternal immune system usually does not react
against these foreign antigens.
 CONCLUSION
 Laboratory tests that can be done are
histopathology, fluorescence immuno and ELISA.
 PG diagnosis is established by history, clinical
features, physical examination, and serological,
histopathological and direct immunofluorescence
tests for investigations.
 The main goal of treatment is to improve pruritus and
prevent the appearance of new blisters. The main
option of treatment is to use corticosteroids.
Gestational pemphigoid tend to heal by itself
postpartum.
 CONCLUSION
In most cases regressive within three months of
delivery, it may last for weeks, months or years before
complete resolution. PG is most common in the first
pregnancy, but may appear in subsequent
pregnancies with more severe clinical features.
Complications of the major fetus are preterm
labor and low birth weight or small infant- to-
gestational age. In PG there is no increased risk of
fetal death.