Autoimmune Bullous Diseases
Life-threatening autoimmune blistering disorders include bullous pemphigoid and the different subtypes of pemphigus: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Major extent of skin and mucosal lesions results in the failure of m
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Pascal Joly
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Life-threatening autoimmune blistering disorders include bullous pemphigoid and the different subtypes of pemphigus: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. Major extent of skin and mucosal lesions results in the failure of main skin functions. Severe infections, including septicaemia and pneumonia and cardiovascular disorders, are the major cause of death in these patients. The aim of treatment is to stop the production of autoantibodies. Corticosteroids and immunosuppressants are widely used in the treatment of autoimmune blistering disorders. New biologic agents such as rituximab are currently proposed for the treatment of the most severe, life-threatening types of these disorders.
models [3]. Binding of auto-antibodies to their target antigens leads to the disruption of intra-epidermal or dermal–epidermal junctions [4]. The common consequence is the formation of cutaneous and/or mucosal blisters evolving to erosions. Extent of erosive areas can lead to failure of main skin functions, in particular defence against infections. Treatment of AIBD aims at stopping the production of pathogenic antibodies by B-lymphocytes and plasmocytes, using corticosteroids, immuno-suppressants and more recently, biologics such as anti-CD20 monoclonal antibodies: rituximab or intravenous immune globulins [5, 6]. Pemphigus and bullous pemphigoid (BP) are potentially life-threatening AIBD, whereas in cicatricial pemphigoid and epidermolysis bullosa acquisita, severe functional impairment may occur in some cases.
12.2 Bullous Pemphigoid 12.1 Introduction Autoimmune bullous diseases (AIBD) are a group of autoimmune disorders of the skin characterized by the production of auto-antibodies directed against adhesion proteins of the desmosomes (pemphigus), or the hemi-desmosomes (AIBD of the dermal–epidermal junction (DEJ) [1, 2]. Most of these auto-antibodies are pathogenic, as demonstrated in various animal
P. Joly Clinique Dermatologique, Hôpital Charles Nicolle, 1, rue de Germont, 76031 Rouen cedex, France
12.2.1 Incidence and Pathogenesis Bullous pemphigoid is the most frequent type of AIBD. The incidence rate of BP ranges between seven and 22 cases per million inhabitants per year [7–9]. It is six to 20 times more frequent than pemphigus in Western Europe. The disease mainly affects the elderly, with mean age being between 80 and 83 years, which is an important characteristic in the management of BP [9–11]. BP patients develop circulating and tissue-bound antibodies that are directed against two proteins of the hemidesmosomes, a key structural component of the DEJ. The target of these antibodies are proteins of 230 and 180 kD in length, and are termed bullous pemphigoid antigen 1 (BPAG1), an intracellular protein of the
J. Revuz et al. (eds.), Life-Threatening Dermatoses and Emergencies in Dermatology, DOI: 10.1007/978-3-540-79339-7_12, © Springer-Verlag Berlin Heidelberg 2009
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hemidesmosome, and bullous pemphigoid antigen 2 (BPAG2), a transmemb
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