Objective: Toxic epidermal necrolysis is a rare condition, occurring in approximately 1 in a million cases per year and usually reported in association with the use of medications such as allopurinol, anti-retroviral, anti-epileptics or viral infections. The incidence is even less common in pregnancy, especially in the first trimester. Toxic epidermal necrolysis (TEN) and Stevens-Johnson Syndrome (SJS) are life-threatening manifestations of a single disease entity. Although mortality is reportedly lower than non-pregnant cohorts, significant maternal and fetal morbidity is not uncommon and should be taken into consideration when counseling on disease prognosis. We illustrate our experience managing such a patient in early pregnancy.

Case Report: A 29-year-old multiparous lady of ethnic Malay descent, six weeks into her pregnancy presented with a three day history of fever, generalized maculopapular rashes, mouth ulcers and bilateral conjunctivitis. She was initially treated as measles but her condition deteriorated over the course of the next few days with eruptions of bullous lesions affecting about 50% of total body surface area (TBSA) and a SCORTEN 2. There was also vaginal spotting and Nikolsky sign was positive. We described in detail, challenging aspects of her management accompanied by classical images which aided the diagnosis.

Conclusion: Changes in T-cell mediated immunity in pregnancy may alter the clinical picture and the developing fetus, especially at an early gestation, presents an additional conundrum. To the authors’ knowledge, there were no other documented cases in a patient of Asian ancestry at such an early gestation. Drug-induced SJS and TEN were reportedly two to three fold higher amongst certain Asian Han Chinese compared to Caucasians, due to the association with HLA-B*15:02. A small multi-ethnic study in Malaysia has previously found similarly high inheritance of this human leukocyte antigen in the Malay population when compared to race matched, healthy controls. Management comprised mainly of supportive care but the use of corticosteroids, intravenous immunoglobulin, ciclosporin and anti-tumour necrosis factor (TNF) have been documented. TEN/SJS may result in miscarriage, preterm labour and fetal growth restriction. Long term gynaecological sequelae such as vaginal adenosis, stenosis, infertility and even endometriosis have been described. Serial fetal growth scans are imperative and mode of delivery should be discussed if vaginal stenosis is present.