Renal Clinical - Pathological Manifestations in Bullous Pemphigoid

Abstract

A 61-year old man was admitted with a 2-month history of skin rash, and proteinuria and intermittent hemoptysis for 2 weeks. The patient had developed circular erythema and blisters all over the body with scab formation. Edema of the legs and eyelids appeared 2 weeks prior to admission. Kidney biopsy showed membranous nephropathy (phase II) with focal segmental mesangial proliferation and deposits of IgG along the GBM. Skin biopsy demonstrated IgG deposits in the epidermal basement membrane zone. The simultaneous development of a rare renal and skin autoimmune disorder, resulting from non-cross-reactive autoantibodies, suggests that a common triggering event could be responsible for the autoimmune injury. This patient with bullous pemphigoid was treated with corticosteroids, which were tapered to an acceptable and effective maintenance dose following treatment with intravenous cyclophosphamide.