Pemphigus Vulgaris
Pemphigus Vulgaris
Pemphigus vulgaris is one of a group of autoimmune disorders that are caused by autoantibodies against the desmoglein adhesion molecules of squamous epithelial cells. It is a rare form of immune dysfunction that can prove vexing to the patient and physician, but it has distinct clinical and histologic findings. We report a case of a patient with the autoimmune blistering disease pemphigus vulgaris localized to the oral cavity and discuss the important clinical, immunopathologic, and therapeutic factors of this disease. This case report highlights the unusual nature of pemphigus vulgaris, the modalities used in its diagnosis, and effectiveness of therapy. Pemphigus vulgaris is an uncommon disease blistering disorder due to desmoglein autoantibodies, whose presentation can be alarming and puzzling to the clinician. Awareness of the disease's presentation and mechanism will allow for an efficient diagnostic evaluation and timely treatment.
Pemphigus vulgaris (PV) is one of a group of unique, related blistering disorders that includes pemphigus foliaceous (PF) and paraneoplastic pemphigus (PNP). The incidence of PV in the United States is estimated at 0.42 per 100,000. They are histologically characterized by suprabasilar acantholysis and immunopathologically characterized by autoantibodies to desmosomal cadherins. In PF the autoantibody is to desmoglein 1 (Dsg1), in PV to desmoglein 3 (Dsg3). The mechanism that initiates the formation of anti-DSG antibodies is unknown, but may be induced by medications, such as d-penicillamine, and may resolve once the inciting medication is withdrawn. In the past, untreated pemphigus had a very high mortality rate. Therapy aimed at immunosuppression has reduced the mortality to 5 to 15%. Specifically, therapy with steroids has been the most significant factor in reducing the mortality of this disease, often in conjunction with other immunosuppressants such as cyclophosphamide, azathioprine, and mycophenolate as well as plasmapheresis.
Pemphigus vulgaris is one of a group of autoimmune disorders that are caused by autoantibodies against the desmoglein adhesion molecules of squamous epithelial cells. It is a rare form of immune dysfunction that can prove vexing to the patient and physician, but it has distinct clinical and histologic findings. We report a case of a patient with the autoimmune blistering disease pemphigus vulgaris localized to the oral cavity and discuss the important clinical, immunopathologic, and therapeutic factors of this disease. This case report highlights the unusual nature of pemphigus vulgaris, the modalities used in its diagnosis, and effectiveness of therapy. Pemphigus vulgaris is an uncommon disease blistering disorder due to desmoglein autoantibodies, whose presentation can be alarming and puzzling to the clinician. Awareness of the disease's presentation and mechanism will allow for an efficient diagnostic evaluation and timely treatment.
Pemphigus vulgaris (PV) is one of a group of unique, related blistering disorders that includes pemphigus foliaceous (PF) and paraneoplastic pemphigus (PNP). The incidence of PV in the United States is estimated at 0.42 per 100,000. They are histologically characterized by suprabasilar acantholysis and immunopathologically characterized by autoantibodies to desmosomal cadherins. In PF the autoantibody is to desmoglein 1 (Dsg1), in PV to desmoglein 3 (Dsg3). The mechanism that initiates the formation of anti-DSG antibodies is unknown, but may be induced by medications, such as d-penicillamine, and may resolve once the inciting medication is withdrawn. In the past, untreated pemphigus had a very high mortality rate. Therapy aimed at immunosuppression has reduced the mortality to 5 to 15%. Specifically, therapy with steroids has been the most significant factor in reducing the mortality of this disease, often in conjunction with other immunosuppressants such as cyclophosphamide, azathioprine, and mycophenolate as well as plasmapheresis.
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