Linear IgA disease associated with membranous glomerulonephropathy

A 33-year-old Caucasian man developed an acute nephrotic syndrome with a pr oteinuria of 15 g/day. His past medical history was significant for a chole cystectomy secondary to complications of hypercholesterolemia, and his medi cations included niacinamide, simvastatin, and multivitamins. A renal biops y revealed a membranous glomerulonephropathy, with thickening of the capill ary loops by light microscopy and 4+ granular immunoglobulin G (IgG) staini ng of the capillary loops by immunofluorescence. Multiple discrete subepith elial deposits were also noted by electron microscopy. He was treated with cyclophosphamide, chlorambucil, and high dose steroids. Laboratory data at that time were notable for an elevated erythrocyte sedimentation rate (ESR) , decreased albumin, a negative antinuclear antibody, and normal complement levels. His symptoms improved, and the proteinuria stabilized to 7 g/day. His systemic therapy was discontinued without a flare in his renal disease. Approximately 9 months later, he developed a pruritic, blistering eruption which began on his face and neck. An initial skin biopsy showed a subepider mal blister with many eosinophils and eosinophilic spongiosis. A direct imm unofluorescence (DIF) study revealed linear deposits of IgG and IgA, and fo cally linear C3 at the basement membrane zone. This pattern was interpreted to be most consistent with bullous pemphigoid. He was referred to the Phil adelphia VA Medical Center for further evaluation and treatment. Cutaneous examination revealed multiple urticarial plaques with tense bulla e studding the peripheral margin, in a rosette pattern, distributed on his face, trunk, groin, extremities, and feet, with one healing erosion on the hard palate. Laboratory data revealed abnormal, but stable, renal disease, with an elevated blood urea nitrogen (21.1) and creatinine (1.5). Serum IgA was elevated (400), and serum albumin (3.3) and a total protein (5.5) were decreased. A second skin biopsy from a blister edge demonstrated a subepidermal bulla with numerous eosinophils and collections of neutrophils at the dermal papi llae tips (Fig. 1). DIF revealed 3+ linear IgA deposition at the basement m embrane zone (Fig. 2), and an indirect immunofluorescence study on monkey e sophagus showed linear IgA deposition in the basement membrane zone. A diag nosis of linear IgA disease was made. Dapsone 50 mg p.o. daily was started, with a dramatic improvement on his blistering within 2 weeks. His renal di sease remained stable.