Case Report

STEVEN JOHNSON SYNDROME IN PREGNANCY

SUCHI JAIN*, AP JAIN**, S PALAPARTHY***, S SAMAL****

ABSTRACT

Steven Johnson Syndrome (SJS) is a lifethreatening allergic reaction to medications

affecting the skin and mucous membranes. A 23 year old G2A1 37 weeks gestation, reported to us
with features of SJS after receiving Inj Cefotaxime. She was put on topical steroids, analgesics and
antacids. She delivered a term female child of weight 2.5kg vaginally. Postpartum she was put on
systemic steroids. Both mother and baby were discharged in a good condition.

Introduction : Fever subsided after treatment. Since the past 4

Steven Johnson Syndrome(SJS) is a severe
and lifethreatening condition. It is thought to
be a hypersensitivity reaction to certain drugs
and vaccines affecting the skin and the mucous
membranes. Altered drug metabolism in some
patients causes formation of reactive metabolites
that bind to and alter cell proteins, triggering a
T-cell-mediated cytotoxic reaction to drug antigens
in keratinocytes. Another possible mechanism
involves interactions between Fas, a cell-surface
death receptor, and its ligand. Other causes like
viral infections and malignancies are also known.
It is the most severe form of erythema multiforme
known as erythema multiforme major. It is a rare
condition with a reported incidence of one case
per million people per year. Such a rare case of
SJS in pregnancy is reported.
Case Report :
A 23-year-old G2A1 with 37 weeks of
gestation reported in emergency with features
suggestive of SJS. She had consulted a private
practitioner 14 days back for high grade fever with
chills for which she was put on oral Cefixime.
* Associate Prof., *** Resident, **** Prof. Deptt. of
Obstetric & Gyneology, ** Professor & Head Deptt. of
Medicine, MGIMS, Sevagram (Wardha) MS - 442 102.
days, she again developed fever and was started
on injectable Cefotaxime after which she had
redness of eyes, swelling of lips, iris lesions which
were becoming congruent all over the body, on
the palms and soles and oral ulcers and was
unable to take anything orally. There was no history
of cough, cold, jaundice, or bleeding tendencies,
or any allergy to drugs and food products. There
was no history of similar complaints in the past.
On examination, patient was toxic,
dehydrated, febrile (temperature - 39 0C), pulse -
110/min, BP - 110/80 mm Hg, not pale, anicteric,
maculopapular eruptions present all over the
body, with involvement of the palms and soles.
Mouth was oedematous and inflamed and there
were ragged ulcers on the buccal mucosa, hard
and soft palate and on the lips. No cervical
lymphadenopathy, no pedal edema. Cardiorespiratory
examination was normal. Abdominal examination
revealed a term size uterus, relaxed. A diagnosis
of SJS was made and she was put on intravenous
hydration and patient was kept nil by mouth. Oral
toileting was carried out every 2 hrly, topical
steroids, analgesics and antacids were started.
Initial investigations showed a haemoglobin
of 10 gm/dl, with total counts of 9.2 x 103 cu mm

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and normal kidney and liver function tests. Swabs
from the mouth were sterile.
The potential precipitating factor could
be antibiotics, which was aggravated in the
presence of pregnancy.
On 2 nd day of admission, patient had
Premaline Rupture of Membranes (PROM) and
delivered a female child vaginally of weight 2.5kg.
The intra-partum period was uneventful.
Following delivery, she developed new active
lesions for which she was put on systemic
Prednisolone which was gradually tapered. The
lesions subsided gradually over a period of 10
days. Mother and baby were well on discharge
and follow up.
Discussion :
SJS is marked by the rapid attack of fever,
skin lesions and sores on the mucous membranes
of eyes, mouth, nasal passage, lips and genitals.
Clusters last for about 2-4 weeks. The skin lesions
may look like target lesions or bubble like. The
diagnosis is often obvious by the appearance of
lesions and rapid progression of symptoms.
Histologic examination of sloughed skin shows
necrotic epithelium, a distinguishing feature. The
condition is charecterised by severe constitutional
Fig 1 a :Maculopapular eruptions over legs
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Suchi Jain & et al
disturbance and may result in death from
pneumonia, septicaemia, myocarditis or renal
failure. Erosive changes may occur in the
genitalia. Severe scarring of the genital tract may
also occur occasionally, however there is no
mention of permanent damage to the female
genital tract.
There has been one case report of vaginal
stenosis following SJS in pregnancy, which was
discovered 6 weeks after cesarean section for
breech presentation1 1 . However, our patient
delivered baby vaginally and there was no
problem on follow up.
The management includes prompt
withdrawal of all potential causative drugs,
intravenous fluid replacement. Symptomatic
treatment are careful and aseptic handling,
maintenance of venous peripheral access distant
from affected areas, initiation of oral nutrition
by nasogastric tube, anticoagulation, prevention
of stress ulcer.
Topical antiseptics (0.5% silver nitrate
or 0.05% chlorhexidine) are used to paint, bathe,
or dress the patients. New dressings with
Apligraft ® , Biobrane ® , TransCyte ® etc are
being tried. Corticosteroid use is highly debated.
Fig 1 b : Maculopapular eruptions over hands
J MGIMS, September 2011, Vol 16, No (ii), 48-50
Steven Johnson Syndrome in Pregnancy
Tegelberg used 400 or 200 mg prednisone/
day, gradually diminished over a 4 to 6 week
period, and observed a single death among eight
patients 2 .
It's difficult to prevent an initial attack
of Stevens-Johnson syndrome because what
triggers it is not known. However, if Steven-
Johnson syndrome occurred once, which was
caused by medication, the drug is to be avoided
to prevent another attack. A recurrence is usually
more severe than the first episode and, may be
fatal.
Attack of SJS developing in pregnancy
can be fatal because immunity is compromised.
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However, early diagnosis and prompt management
saved the mother and the child.
Disclosure of Interest :
No conflicts of interest.
References :
1. Graham R.A.C., Cochrane G.W., Swihone J.R.,
Sarkany. I., Epsztein L.J., "Vaginal Stenosis due to
Bullous Erythema Multiforme ( Steven Johnson
Syndrome)" Br J Obstet Gynaecol 1981; 88: 1156-57
2. Tegelberg-Stassen MJ, van Vloten WA, Baart de la
Faille. Management of nonstaphylococcal toxic
epidermal necrolysis: follow-up study of 16 case
histories. Dermatologica 1990;180:124-129.
J MGIMS, September 2011, Vol 16, No (ii), 48-50