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.