Abstract

Background: SJS occurs almost exclusively secondary to drugs but very rarely, SLE can be an inciting factor for SJS without presence of an offending drug. The association is extremely rare, however few cases have been reported. Case presentation: We present a case report of a thirty year old female who presented to the rheumatology clinic with complaints of rash on her face, swelling of the lips and oral ulcers with severe hemorrhagic cheilitis. She also complained of persistent high grade fever with development of blisters on her face and oropharyngeal mucosa. She had been suffering from pain in multiple joints which did not respond to NSAIDs. A diagnosis of SLE presenting as SJS was established. Patient was given good supportive care, steroids, DMARDs and analgesics, to which she responded well and recovered. Conclusion: Stevens Johnson Syndrome is an uncommon, severe dermatological condition usually caused secondary to drugs. Secondary cause of the disease may rarely be co-existence of an autoimmune condition like SLE, as present in our patient.

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