Pemphigus Vulgaris:A Closer Look

1553 Words4 Pages

Pemphigus Vulgaris: A Closer Look Pemphigus vulgaris is a serious but rare autoimmune disorder of the epithelial cells and mucosal lining of the skin. The first cases were recorded by McBride in 1771 and by Whichman in 1979. McBride was to describe the first to casualties as being caused by “bloody ichor” and “putrid ulcers”. (Jordon, 2013) However, Whichman was the one who would originally name the disease as pemphigus. The word “pemphigus” is of Greek origin which means blister or bubble. (Jordon, 2013) As the name suggests, this disease causes painful blisters or bullous erosions of the squamous epithelia and mucous membranes. It can be sub-classified based on the location of the lesions in the epidermis. Pemphigus vulgaris is considered to be one of the deep forms of the disease. The lesions appear deep into the epidermis of the skin right above the basal lamina. (Zeina, 2013) Pathology The autoimmune process that causes pathogenicity is the formation and activation of antibodies targeting the keratinocyte desmosomal cadherins or for simplicity desmosomes. The latter is responsible for the adherence of keratinocytes, and when it is targeted by these antibodies the desmosomes lose their ability to maintain keratinocytes together and the cells become detached. “Ultrastructural studies of pemphigus lesions have suggested that dissolution of desmosomes may result in the formation of blisters. This is a phenomenon known as acantholysis.” (Loannides, Lazaridou, Rigopolous, 2008) Studies have shown that the antigens responsible for this autoimmune attack have components of the actual desmosome. Two transmembrane glycoproteins have been identified. One being desmoglein ( Dsg) and desmocollin (Dsc). Both of these have three isofor... ... middle of paper ... ...glubulin isotypes correlate with disease activity, morphology, duration, and hla association in pemphigus vulgaris. Autoimmunity, 45(7), 516-526. DOI:10.3109/08916934.2012.702811 IPPF. (2013). International pemphigoid and pemphigus foundation. Retrieved from http://www.pemphigus.org/research/clinically-speaking/treatments/ Jordon, R. (2003, January 31)Pemphigus. Pemphigus:A historical perspective.Retrieved from http://www.pemphigus.org/pemphigus-a-historical-prescpective-2/ Loannides, D; Lazaridou, E; Rogopolous, D. (2008). Pemphigus. European academy of dermatology and venereology,22, 1478-1496. DOI: 10.1111/jdv.2008.22.issue-12 Lowe, S. (2007). A patient's journey: Pemphigus vulgaris. BMJ, 335, 1152. DOI: http://dx.doi.org/10.1136/bmj.39374.504884.BE Pemphigus Vulgaris Network (2014). What is pemphigus vulgaris? Retrieved from http://www.pemphigus.org.uk/

More about Pemphigus Vulgaris:A Closer Look

Open Document