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STEVEN JOHNSON SYNDROME IN PREGNANCY

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

ABSTRACT

Steven Johnson Syndrome (SJS) is a lifethreatening allergic reaction to medicationsaffecting the skin and mucous membranes. A 23 year old G2A1 37 weeks gestation, reported to uswith features of SJS after receiving Inj Cefotaxime. She was put on topical steroids, analgesics andantacids. She delivered a term female child of weight 2.5kg vaginally. Postpartum she was put onsystemic steroids. Both mother and baby were discharged in a good condition.

Case Report

Introduction :Steven Johnson Syndrome(SJS) is a severe

and lifethreatening condition. It is thought tobe a hypersensitivity reaction to certain drugsand vaccines affecting the skin and the mucousmembranes. Altered drug metabolism in somepatients causes formation of reactive metabolitesthat bind to and alter cell proteins, triggering aT-cell-mediated cytotoxic reaction to drug antigensin keratinocytes. Another possible mechanisminvolves interactions between Fas, a cell-surfacedeath receptor, and its ligand. Other causes likeviral infections and malignancies are also known.It is the most severe form of erythema multiformeknown as erythema multiforme major. It is a rarecondition with a reported incidence of one caseper million people per year. Such a rare case ofSJS in pregnancy is reported.

Case Report :A 23-year-old G2A1 with 37 weeks of

gestation reported in emergency with featuressuggestive of SJS. She had consulted a privatepractitioner 14 days back for high grade fever withchills for which she was put on oral Cefixime.

Fever subsided after treatment. Since the past 4days, she again developed fever and was startedon injectable Cefotaxime after which she hadredness of eyes, swelling of lips, iris lesions whichwere becoming congruent all over the body, onthe palms and soles and oral ulcers and wasunable to take anything orally. There was no historyof cough, cold, jaundice, or bleeding tendencies,or any allergy to drugs and food products. Therewas no history of similar complaints in the past.

On examination, patient was toxic,dehydrated, febrile (temperature - 390C), pulse -110/min, BP - 110/80 mm Hg, not pale, anicteric,maculopapular eruptions present all over thebody, with involvement of the palms and soles.Mouth was oedematous and inflamed and therewere ragged ulcers on the buccal mucosa, hardand soft palate and on the lips. No cervicallymphadenopathy, no pedal edema. Cardiorespiratoryexamination was normal. Abdominal examinationrevealed a term size uterus, relaxed. A diagnosisof SJS was made and she was put on intravenoushydration and patient was kept nil by mouth. Oraltoileting was carried out every 2 hrly, topicalsteroids, analgesics and antacids were started.

Initial investigations showed a haemoglobinof 10 gm/dl, with total counts of 9.2 x 103 cu mm

* Associate Prof., *** Resident, **** Prof. Deptt. ofObstetric & Gyneology, ** Professor & Head Deptt. ofMedicine, MGIMS, Sevagram (Wardha) MS - 442 102.

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and normal kidney and liver function tests. Swabsfrom the mouth were sterile.

The potential precipitating factor couldbe antibiotics, which was aggravated in thepresence of pregnancy.

On 2nd day of admission, patient hadPremaline Rupture of Membranes (PROM) anddelivered a female child vaginally of weight 2.5kg.The intra-partum period was uneventful.Following delivery, she developed new activelesions for which she was put on systemicPrednisolone which was gradually tapered. Thelesions subsided gradually over a period of 10days. Mother and baby were well on dischargeand follow up.

Discussion :SJS is marked by the rapid attack of fever,

skin lesions and sores on the mucous membranesof eyes, mouth, nasal passage, lips and genitals.Clusters last for about 2-4 weeks. The skin lesionsmay look like target lesions or bubble like. Thediagnosis is often obvious by the appearance oflesions and rapid progression of symptoms.Histologic examination of sloughed skin showsnecrotic epithelium, a distinguishing feature. Thecondition is charecterised by severe constitutional

disturbance and may result in death frompneumonia, septicaemia, myocarditis or renalfailure. Erosive changes may occur in thegenitalia. Severe scarring of the genital tract mayalso occur occasionally, however there is nomention of permanent damage to the femalegenital tract.

There has been one case report of vaginalstenosis following SJS in pregnancy, which wasdiscovered 6 weeks after cesarean section forbreech presentation11. However, our patientdelivered baby vaginally and there was noproblem on follow up.

The management includes promptwithdrawal of all potential causative drugs,intravenous fluid replacement. Symptomatictreatment are careful and aseptic handling,maintenance of venous peripheral access distantfrom affected areas, initiation of oral nutritionby nasogastric tube, anticoagulation, preventionof stress ulcer.

Topical antiseptics (0.5% silver nitrateor 0.05% chlorhexidine) are used to paint, bathe,or dress the patients. New dressings withApligraft, Biobrane, TransCyte etc arebeing tried. Corticosteroid use is highly debated.

Suchi Jain & et al

Fig 1 a :Maculopapular eruptions over legs Fig 1 b : Maculopapular eruptions over hands

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Tegelberg used 400 or 200 mg prednisone/day, gradually diminished over a 4 to 6 weekperiod, and observed a single death among eightpatients2.

It's difficult to prevent an initial attackof Stevens-Johnson syndrome because whattriggers it is not known. However, if Steven-Johnson syndrome occurred once, which wascaused by medication, the drug is to be avoidedto prevent another attack. A recurrence is usuallymore severe than the first episode and, may befatal.

Attack of SJS developing in pregnancycan be fatal because immunity is compromised.

However, early diagnosis and prompt managementsaved 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 toBullous Erythema Multiforme ( Steven JohnsonSyndrome)" Br J Obstet Gynaecol 1981; 88: 1156-57

2. Tegelberg-Stassen MJ, van Vloten WA, Baart de laFaille. Management of nonstaphylococcal toxicepidermal necrolysis: follow-up study of 16 casehistories. Dermatologica 1990;180:124-129.

Steven Johnson Syndrome in Pregnancy