P033 Toxic epidermal necrolysis-like lupus treated with etanercept

Abstract

A 61-year-old woman with cutaneous and systemic lupus erythematous presented with a new extensive rash for 2 weeks. The rash began after commencing gabapentin for back pain. Baricitinib was recently stopped as it was ineffective in controlling arthralgia. She became more fatigued and her blood count developed both anaemia and lymphopenia. The rash was annular and polycyclic in pattern. It was most pronounced in a photodistributed pattern on the face, upper chest, upper back and lower limbs, covering > 30% of the body. It was dusky purple in colour and there were areas of desquamation. There was no mucus membrane involvement. Histology was in keeping with epidermal necrosis. Vacuolar interface changes were not detected but direct immunofluorescence (DIF) of lesional and nonlesional skin showed extensive IgG antinuclear antibodies in the epidermis in keeping with a connective tissue disease. Toxic epidermal necrolysis (TEN)-like lupus was diagnosed. She was treated with 5 days of IVIg and 3 days of intravenous methylprednisolone. She was administered etanercept 50 mg subcutaneously on day 4 and day 8. Optimal topical skincare, antibiotics and analgesia were provided. Full re-epithelization occurred 2 weeks after admission and the patient was discharged after 4 weeks. The unifying acute syndrome of apoptosis pan-epidermolysis (ASAP) describes acute and massive epidermal cleavage from keratinocyte apoptosis seen in TEN, and TEN-like conditions such as TEN-like lupus, graft-versus-host disease and pseudoporphyria. Differentiating TEN from TEN-like lupus is a clinical diagnosis as histology findings are inconsistent. DIF can be supportive of lupus. Clinical findings of active cutaneous systemic lupus erythematosus, a lack of mucosal involvement, predominantly photodistributed location of lesions, lupus serology and signs of a lupus flare (such as cytopenia) support TEN-like lupus. New drugs may trigger an event in 25% of cases.