Based on the clinical features and investigations a diagnosis of bullous SLE with lupus nephritis was made. He was started on oral prednisolone 2 mg/kg/day dapsone 100 mg/day and losartan 50 mg/day. Within 4 weeks of above treatment, his edema resolved, bullous lesions healed completely leaving depigmented areas and he became normotensive. After 2 months of daily steroid therapy was over, an attempt was made to taper steroid. When prednisolone was tapered to 0.5 mg/kg/day he developed erythematous patches and painful swelling of both soles suggestive of vasculitis and was unable to walk. Prednisolone was increased to 2 mg/kg again and the vasculitis resolved with in a week. He has been on close follow up for the last 12 months. Prednisolone, dapsone and losartan were stopped after 10 months from onset. At recovery the repeat ESR was 11 mm/hr, ANA level was 7.979 (positive) and DS DNA level was 0.240 (negative). Without any medication the child is doing well for the last 2 months and there is no recurrence of bullous skin lesions or relapse of nephrotic syndrome.

Bullous SLE is an extremely uncommon subset of systemic lupus erythematosus with an incidence of 0.26 per million population per year in adults(2). Clinically, BSLE is characterized by rapid develop-ment of widespread vesiculobullous lesions. Blisters range from large tense bullae to small vesicles. Bullae contain clear or hemorrhagic fluid and rupture spontaneously resulting in erosion and crusts which heal with hypopigmentation or hyperpigmentation. The blisters are distributed over the neck, face, trunk and extremities. Among the cutaneous manifes-tations of SLE, BSLE accounted for 2 percent in THAI children with SLE(3). Rarely, BSLE may be the initial clinical manifestation of SLE(4,5). Though, bullous skin lesions were the initial manifestation of SLE in our case, he had features of nephrotic syndrome also at admission. Bullous SLE presenting in a localized linear pattern has been reported(6). The histological examination in BSLE shows neutrophilic dermal infiltrate and subepidermal separation(7). Direct immunofluore-scence (DIF) of perilesional skin reveals deposition of IgG with or without Ig M and Ig A and complement in a linear or granular bandlike pattern at the basement membrane. Our patient’s skin biopsy has shown typical histologic and DIF features. Class V membranous nephritis occurs in 10% to 20% of patients with lupus nephritis(8). The clinical presentation ranges from mild proteinuria with hematuria to nephrotic range proteinuria. Hypertension occurs in 30% of cases. Our child had Class V lupus nephritis and presented with nephrotic syndrome. The association of lupus nephritis with BSLE has been reported in adults(5). Bullous SLE shows dramatic response to dapsone(5,9). Fifty to 100 mg/day is often used (10). In our child cessation of new blister formation occurred within 48 hours after start of dapsone therapy and existing lesions healed within a week. Most patients with Class V lupus nephritis require only low doses of steroids for a short period(8). Only a minority of patients require a prolonged course of steroids or use of a second immunosuppressive agent like cyclosporin or azathioprine. Our child responded well to oral steroids. Our case illustrates that generalized vesiculobullous eruption can be the initial manifestation of SLE and the close relationship between BSLE and lupus nephritis.