Stevens-Johnson Syndrome

Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: Associations, Outcomes, and Pathobiology-Thirty Years of Progress but Still Much to Be Done

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Nearly a century ago, Stevens and Johnson first described Stevens-Johnson syndrome (Heng 2015, Roujeau 1995). More than 60 years ago, Lyell first described epidermal necrolysis (Heng 2015, Su 2016). Most of the progress in defining the spectrum of disease now known as Stevens-Johnson syndrome, overl…

Just today, I discharged a patient with SJS (to Gabapentin or Amoxicillin/Clavulanate), whom nearly didn’t make it.

He has tophaceous gout treated to target on Febuxostat, and hypertension on Losartan.  He is allergic to Allopurinol (rashes).  He is a chronic smoker, and has alcoholic hepatitis with mild thrombocytopaenia.  I last reviewed him in April this year.

He was recently diagnosed by a dermatologist with facial discoid lupus based on skin biopsy and a positive ANA.  Other serologies were negative, and only the C3 was slightly low.

He returned from a holiday in early August with painful swelling around the left ankle.  He also had an episode of gross haematuria then, and was prescribed a course of antibiotics by his family doctor.  He consulted me on 21/8/17 for the persistent left ankle pain, which I found not to be due to ankle synovitis.  Doppler ultrasound revealed a partial peroneal vein thrombosis, and the deep perforators were found to be incompetent.   Urine culture returned growing E coli, and I started him on Amoxicillin/Clavulanate on 25/8/17.  Celecoxib and Colchicine were also prescribed, which he previously had repeated courses of without adverse reaction.

The brawny oedema over the left medial malleolus ulcerated.   His family doctor prescribed him Gabapentin on 30/8/17 for refractory pain.  By the next day, he started feeling unwell, with lethargy and loss of appetite progressing over the next few days.  On 5/9/17, he developed a low grade fever and a maculopapular rash on the right leg, which gradually extended to the trunk and face.  By 8/9/17, he had to be admitted as his throat was sore and he could no longer take orally.

I saw him upon returning from my overseas course on 10/9/17.  He had developed a generalized non-bullous maculopapular rash, with ulcerations of the hard palate, oral thrush, and cheilitis with crusting.  He also had SIADH, mixed hepatitis, DIC and ground-glass pneumonitis.  All cultures and PCR were negative, except for scanty Staph aureas growth from the BAL.  Hydrocortisone 200mg tds and Nystatin failed to improve his condition over 4 days, and trismus ensued.

In view of the pathogenic role of IL15 in SJS, Tofacitinib was started off-label at 10mg bd on 14/9/17, with informed consent.  Hydrocortisone was stopped.  By the next day (after 1 dose of 10mg), the patient reported that his throat no longer hurt, and could swallow.  Safe swallowing was verified by speech therapist.  The rashes were visibly faded by 16/9/17, the laboratory parameters had mostly normalized (or normalizing), and he was discharged.