Discussion
BPP is a rare, autoimmune bullous skin disorder classified as a variant of mucous membrane pemphigoid (MMP). It was first reported in 1957 by Brunsting and Perry, as a scarring disease localised to the head and neck, with no mucosal involvement.4
The pathogenesis of BPP includes an attack on the target proteins of the basement membrane by autoreactive antibodies. Antibodies targeting laminin 332, type VII collagen, and BP180 have been identified up to now. Autoreactive IgG targets these proteins with subsequent deposition of C3 complement, which can be demonstrated by DIF. This autoimmune cascade results in a lymphocytic infiltrate with significant eosinophilia. This disrupts the normal structure of the basement membrane, causing a separation below the epidermis.1
Treatment of BPP is challenging and should be initiated as early as possible. Mild cases can be treated with potent topical or intralesional corticosteroids whereas moderate-to-severe disease requires systemic therapy with immunosuppressive drugs such as prednisone, azathioprine, mycophenolate mofetil, and cyclophosphamide. 5,6
Rituximab is a humanised IgG1 type monoclonal antibody against CD20 antigen, which is found on the surface of mature B cells. Rituximab is indicated for the treatment of non-Hodgkin’s lymphoma, chronic lymphocytic leukemia, rheumatoid arthritis, microscopic polyangiitis and granulomatosis with polyangiitis (Wegener’s Granulomatosis). Rituximab has become the first-line therapy for moderate to severe pemphigus vulgaris. Intralesional rituximab has been shown to be effective in recalcitrant mucosal lesions of PV. 7 In severe resistant MMP cases, rituximab has been quite successful, in combination with systemic immunsupressive or immunmodulatory treatment.8
Our patient was resistant to treatment with potent corticosteroids so we decided to administer intralesional rituximab as a novel therapeutic approach. Our case is the first reported case of Brunsting-Perry pemphigoid treated successfully with intralesional rituximab.