Bullous Diseases of the Skin and Mucous Membranes
Autoimmune blistering diseases are characterized by epidermal or subepidermal blistering of the skin and mucosal surfaces. Both genetic and environmental factors play a role in their development. Pathogenic autoantibodies against epidermal and basement membrane adhesion molecules facilitate the development of cutaneous lesions, although the exact mechanisms are still not known. Other components of the immune system, including complement, T-cells, other cytokines, and heat shock proteins, are all implicated in varying degrees in these diseases.Therapy for moderate to severe disease is not just based on what is known about the pathogenesis of the specific disorder but is tailored based on the comorbidities and health of the patient. Oral prednisone is first line therapy for moderate to severe disease in several of these conditions, such as pemphigus, but has unwanted side effects. Steroid-sparing agents are therefore important in the management of these patients. Newer, more targeted immunotherapies such as rituximab may provide more rapid and sustained remission, while minimizing steroid side effects.
