P18: A RARE CASE OF CHRONIC NON GRANULOMATOUS SUPRAGLOTTITIS AND TOXIC EPIDERMAL NECROLYSIS (TEN) SECONDARY TO IT'S TREATMENT IN A TEENAGE GIRL

Abstract

TEN is an extremely rare complication of drug treatment (estimated at 1-2 cases per million each year).1 Chronic non-granulomatous supraglottitis is an unusual disease rarely reported in paediatric medical literature.2 Case history: A 13-year-old girl presented to our regional hospital on 6 February 2017 with gradually worsening history of dysphonia, lethargy, and weight loss (7.3%) since October 2016. She had two courses of oral Augmentin-duo for ongoing symptoms of fever, dyspnoea and cough by November 2016. A chest X-ray was suggestive of left sided consolidation at the time. Paired sera 4 weeks apart in January and February showed positive IgA, IgG and negative IgM antibody titres to Chlamydiae Pneumoniae and she was given a 5-week course of doxycycline for a presumptive diagnosis of atypical pneumonia. Cough, dyspnoea and chest X-ray abnormalities resolved by January 2011. Her only significant past medical history was eczema. Systemic examination was normal except mild inspiratory stridor. Direct flexible laryngoscopy (DFL) revealed appearance of supraglottitis. She was unsuccessfully treated with Dexamethasone and Intravenous Ceftriaxone for 5 days. Subsequent CT 'neck and chest' suggested thickened epiglottis and aryepiglottic folds, confirmed by DFL 2 days later. An elaborate range of investigations has been negative for respiratory pathogens and autoantibodies. On transfer to a tertiary centre, high dose prednisolone was commenced. Six days later, histopathology (HP) of the epiglottis and arytenoid folds was suggestive of chronic non-granulomatous supraglottitis on 22 February 2017. She was discharged home on oral prednisolone and omeprazole. Hydrochloroquine 400 mg was started on 18 March 2017 for ongoing symptoms. A mild rash on her jaw and arms was noted on 22 March 2017. Repeat HP that day showed persistent non-granulomatous supraglottitis. Rash progressively worsened by 25 March 2017 when target lesions became evident, hydrochloroquine was ceased. Genital and oral lesions also started on same day and progressed to severe TEN involving >70% of body area by 29 March 2017. Omeprazole was later thought to contribute to Hydroxychloroquine to cause TEN presentation. She was electively intubated for 6 weeks for TEN management and has spent another 2 months in rehabilitation thereafter to return towards a normal life. Conclusion(s): Persistent and progressive drug rash needs careful evaluation and high index of suspicion.