Immunopathogenesis and Therapeutic Insights into Cicatricial Pemphigoid: A Comprehensive Review
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Abstract
Cicatricial Pemphigoid (CP), also known as mucous membrane pemphigoid, is a rare autoimmune blistering disorder characterized by subepithelial blistering and scarring of mucous membranes. The hallmark of CP is the formation of autoantibodies against structural proteins within the basement membrane zone, leading to an array of clinical manifestations affecting oral, ocular, and other mucosal surfaces. This review aims to elucidate the complex immunopathogenic mechanisms underlying CP, exploring the role of autoantigens such as BP180 and BP230, as well as the involvement of inflammatory mediators and immune cells. Additionally, the article provides a comprehensive overview of current diagnostic modalities and therapeutic approaches, ranging from systemic corticosteroids to novel immunomodulatory agents and biological therapies. The challenges in managing CP, including potential side effects of immunosuppressive treatments, are discussed alongside emerging strategies to enhance treatment efficacy and minimize adverse effects. A deeper understanding of the molecular pathways involved in CP pathogenesis is crucial for the development of targeted therapies, fostering improved patient outcomes and quality of life
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References
I. Chan Lawrence S, Razzaque Ahmed A, Anhalt Grant J, Bernauer Wolfgang, Cooper Kevin D, Elder Mark J et al. The First International Consensus on Mucous Membrane Pemphigoid. Definition, diagnostic Criteria, Pathogenic Factors, Medical Treatment, and Prognostic Indicators. Arch Dermatol 2002; 138: 370-9.
II. Sánchez Pérez A, Bermejo Fenoll A. Penfigoides: manifestaciones orales. En: Bermejo Fenoll A. Medicina Bucal. Madrid: Editorial Síntesis; 1998. p. 186-191.
III. Strassburg M, Knolle G. Mucosa Oral. Atlas a color de enfermedades. 3ª ed. Madrid: Marban; 1995.
IV. Regezi, Sciubba. Patología bucal. Correlación clínico patológica. 3ª ed. México: McGraw-Hill. Interamericana; 1999.
V. Bean SF. Diagnosis and management of chronic oral mucosal bullous diseases. Dermatol Clin 1987; 5: 751-60.
VI. Ahmed AR, Kurgis BS, Rogers IIIRS. Cicatricial pemphigoid. J Am Acad Dermatol 1991; 24: 987-1001.
VII. Laskaris G, Sklavounou A, Stratigos J. Bullous pemphigoid, cicatricial pemphigid, and pemphigus vulgaris. Oral Sug Oral Med Oral Pathol 1982; 54: 656-62.
VIII. Mobini N, Nagaraxalla N, Ahmed AR. Oral pemphigoid. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998; 85 : 37-43.
IX. Vicent SD, Lilly GE, Baker KA. Clinical, historic, and therapeutic features of cicatricial pemphigoid. Oral Surg Oral Med Oral Pathol 1993; 76: 453-9.
X. Fleming TE, Korman KJ. Cicatricial pemphigoid. J Am Acad Dermatol 2000; 43 (4): 571-91.