A 22-year-old woman was referred by the critical care team of a district general hospital (DGH) to our regional burns unit with 100% TBSA involvement following toxic epidermal necrolysis (TEN) on the background of GPA. The patient initially presented to the DGH with a fever, shortness of breath and feeling generally unwell. A chest X-ray demonstrated multiple opacities and on computerised tomography (CT) of the chest and abdomen, she was found to have multiple cavitations, a pulmonary embolism and a femoral thrombosis. Granulomatosis with polyangiitis (GPA) was confirmed and the patient was commenced on warfarin, rituximab, methylprednisolone, Immunoglobulins (IgG) and fluconazole, to which she responded well. As part of this regime she had a second and third infusion of rituximab and prophylactic co-trimoxazole.
Three days following co-trimoxazole, she presented back to the DGH with angioedema and had developed a rash with an estimated 90% total body surface area (TBSA), involving the oral and ophthalmic mucosa. Fluconazole and co-trimoxazole were immediately stopped and the steroids increased with the initial suspicion of Stevens-Johnson syndrome (SJS). The patient deteriorated, progressing to 100% TBSA, and required significant support from the critical care team. TEN was suspected and subsequently confirmed by skin biopsy. She had an initial SCORTEN (SCORe of Toxic Epidermal Necrosis) score of 3, predicting a 35.3% mortality risk, and was accordingly referred and transferred to our specialist burns centre for management of her extensive wounds. Figure 1a and b show the large extent of the TBSA involved in this patient.
Piperacillin/Tazobactam (Tazocin) and Vancomycin antibiotics were commenced following sputum and wound swab sensitivities. One week following admission, prednisolone was reduced to 40 mg daily. After 10 days of treatment, there was good re-epithelisation of the skin. The patient was discharged at 3 weeks following a period of physiotherapy.