Desmoglein Compensation Theory: An explanations for Early Appearance of Oral lesions as Compared to Skin Lesions in Pemphigus Vulgaris

  • Safia Siddiqui Department of Oral Pathology and Microbiology, Sardar Patel Post Graduate Institute of Dental & Medical Sciences, Lucknow- 226025 (U.P.)
  • Mohammad Asad Haroon Assistant Professor, Department of Dermatology, Integral Institute Medical Sciences and Research, Lucknow
  • Shamim ul Hasan Assistant Professor, Department of Oral Medicine and Radiology, Faculty of Dentistry, Jamia Milia Islamia, New Delhi
  • Aeman Khalid Post Graduate Student (MD pathology), Jawaharlal Nehru Medical College, AMU, Aligarh
Keywords: Pemphigus, Desmoglein Compensation Theory, Autoimmune disorder, Blister

Abstract

Pemphigus is a life-threatening autoimmune blistering disease targeting skin and mucous membranes. It is clinically characterized by flaccid blisters and erosions, while histologically shows intraepithelial acantholysis.  The disruption of desmoglein-dependent cell adhesion by autoantibodies is the basic pathophysiology in blister formation of pemphigus.

The clinical and histological spectrum of pemphigus is complex and differs in various variants of pemphigus. This review offers an answer to why the splits associated with pemphigus foliaceus occur in the superficial layer of the epidermis, while those of pemphigus vulgaris occur deep in the epidermis. With the help of desmoglein compensation theory, it logically explains why oral erosions develop in patients with pemphigus vulgaris, but not in patients with pemphigus foliaceus and why some patients with pemphigus vulgaris have only oral involvement, but others have extensive lesions on both skin and mucous membranes.

Learning objective: After completing this article, readers shall be familiar with the clinical presentations, histologic findings, immunopathology  of classical pemphigus and its  variants. It discusses the  desmoglein compensation theory of pathogenesis. along with the management of pemphigus.

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Published
2016-09-28
How to Cite
1.
Siddiqui S, Haroon MA, Hasan S ul, Khalid A. Desmoglein Compensation Theory: An explanations for Early Appearance of Oral lesions as Compared to Skin Lesions in Pemphigus Vulgaris. Int Arch BioMed Clin Res [Internet]. 2016Sep.28 [cited 2019Dec.14];2(3):13-7. Available from: https://iabcr.org/index.php/iabcr/article/view/155