Erythema exudativum multiforme as a skin manifestation of acute systemic lupus erythematosus. Rowell’s syndrome

Introduction. Systemic lupus erythematosus (SLE) is a multiorgan autoimmune disease with pathological skin changes occurring in about 75-80% of patients. Abnormalities which can be found in SLE, but are not characteristic for the disease, may show morphology and location typical for erythema multiforme.
Objective. Presentation of a patient with Rowell syndrome recalcitrant to standard therapy.
Case report. A 77-year-old female patient in poor general condition was admitted to our department due to skin changes of erythema iris type located on distal parts of extremities, chest and face. Laboratory tests revealed increased erythrocyte sedimentation rate, pancytopenia, hypoalbuminaemia, hypergammaglobulinaemia and hypocomplementaemia. Circulating anti-nuclear antibody of speckled and homogeneous pattern in a titre of 640, anti-double-stranded DNA antibodies (titre 160), anti-histone and anti-nucleosome antibodies were disclosed and the diagnosis of systemic lupus erythematosus was established. Due to the significant extent of skin changes and a tendency to epidermolysis, she was treated with methylprednisolone pulse therapy (500 mg/24 h i.v. for 5 days) followed by high-dose oral methylprednisolone (48 mg/24 h) and pulses of cyclophosphamide (200 mg/24 h i.v.). The therapy had to be discontinued due to increasing leukopenia. Symptoms of incoming heart failure, disturbances of water-mineral balance and an episode of epileptiform convulsions with loss of consciousness were observed. Intravenous immunoglobulin (IVIG) administration (0.4 g/kg/24 h for 5 days) led to improvement of skin lesions and the patient’s general condition and reduction of the dose of immunosuppressive drugs.
Conclusions. In cases of Rowell syndrome introduction of IVIG therapy may have a beneficial effect.