The immune system is composed of two closely collaborative systems, an innate and an adaptive system. The innate immune system is a constitutive present system that can act rapidly to eradicate microbes. The primary cells of the innate immune system are macrophages, granulocytes, natural killer cells, and dendritic cells. The adaptive system can be divided in a humoral and cellular response. The humoral response is characterized by activation of B lymphocytes with subsequent maturation into plasma cells and production of antibodies, whereas a cellular immune response is characterized by transformation of T lymphocytes into cytotoxic T cells, capable of killing virally infected cells.
Autoreactive B and T lymphocytes can induce autoimmune diseases. Autoimmune bullous diseases are the result of type II hypersensitivity, e.g., autoantibodies are directed against cell or matrix components. In pemphigoid diseases, antibodies are directed against hemidesmosomal components, whereas pemphigus is characterized by antibodies against desmosomal proteins.
Immune system Autoimmunity Pemphigus Pemphigoid
This is a preview of subscription content, log in to check access.
Berkowitz P, Hu P, Warren S, Liu Z, Diaz LA, Rubenstein DS. p38MAPK inhibition prevents disease in pemphigus vulgaris mice. Proc Natl Acad Sci U S A. 2006;103:12855–60.CrossRefPubMedPubMedCentralGoogle Scholar
Waschke J, Spindler V, Bruggeman P, Zillikens D, Schmidt G, Drenckhahn D. Inhibition of Rho A activity causes pemphigus skin blistering. J Cell Biol. 2006;175:721–7.CrossRefPubMedPubMedCentralGoogle Scholar
Caldelari R, de Bruin A, Baumann D, Suter MM, Bierkamp C, Balmer V, Müller E. A central role for the armadillo protein plakoglobin in the autoimmune disease pemphigus vulgaris. J Cell Biol. 2001;153:823–34.CrossRefPubMedPubMedCentralGoogle Scholar
Aoyama Y, Kitajima Y. Pemphigus vulgaris-IgG causes a rapid depletion of desmoglein 3 (Dsg3) from the Triton X-100 soluble pools, leading to the formation of Dsg3-depleted desmosomes in a human squamous carcinoma cell line, DJM-1 cells. J Invest Dermatol. 1999;112:67–71.CrossRefPubMedGoogle Scholar
Dranoff G. Cytokines in cancer pathogenesis and cancer therapy. Nat Rev Cancer. 2014;4:11–22.CrossRefGoogle Scholar
Kumar V, Abbas AK, Fausto N, Aster J. Robbins & Cotran pathologic basis of disease. 8th ed. Philadelphia: Elsevier Saunders; 2010. p. 185.Google Scholar