Unusual presentation of childhood Systemic Lupus Erythematosus

Bullous systemic lupus erythematosus (SLE) is a rare, distinctive subepidermal blistering disorder that occurs in systemic lupus erythematosus [1]. It is characterized clinically by a pemphigoid-like eruption with tense fluid-filled vesicles and bullae, often with a background of maculopapular or urticated erythema. It can affect any area of the body, including non-sun-exposed sites and the mucous membranes. Pruritus is usually present in variable severity. The lesions form erosions and crusts before healing, usually but not invariably without scarring. Treatment with dapsone results in promising results. We describe a 13 years old girl who presented with bullous SLE.A 13-year-old girl presented with recurrent fever associated with increasing fatigue, arthraliga, hair loss and erythematosus bullous lesions over face, neck and extremities of 2 months duration. She also had one episode of generalised tonic clonic seizures prior to admission.She was treated with oral antibiotics and topical hydrocortisone for her skin lesions. On examination she had numerous bullae and vesicles on a background of urticated plaques affecting the back, abdomen, neck and flexures of the arm and groin regions (Fig. 1). Nikolsky's sign was negative. Erosions with crusts characterized older lesions. No scarring was seen at the sites of healed lesions. There were no mucosal erosions or blisters. No malar rash or oral ulcers. No raynaud's phenomenon. Cardiovascular and respiratory system examination were within normal limits. Abdominal examination did not reveal hepatosplenomegaly. Her fundoscopy did not reveal evidence of hypertensive encephalopathy or vasculitis. Otherwise neurologically she was normal.Investigations revealed hemoglobin was 90 g/L, white blood cell was 21 × 109/L with neutrophils of 2.5 × 109/L, lymphocytes of 1 × 109/L, bands 0.03 × 109/L. Her platelets were 20 × 109/L. Her INR and PTT were within normal limits. ESR was 55 mm at 1 hour and CRP was negative. Her serum creati