This 16 year old girl had a 3 year history of unexplained arthralgia and was referred to our Department because of a butterfly malar rash appearance.
High titers of antinuclear antibodies and anti-nDNA autoantibodies were found; therefore hydroxychloroquine (200mg/d) and prednisone (25 mg/d) were administered.
Two months later she had a riesacerbation of SLE with fever, a cutaneous rash involving the upper trunk, vasculitic lesions on the palms and oral ulcers.
Within a few days the cutaneous lesions extended to the upper trunk developing large sheets of epidermal shedding with a toxic epidermal necrolysis-like pattern.
The prednisone daily dose was incresed to 75 mg with a rapid improvement of the cutaneous lesions.