Sunday, November 25, 2018

Pemphigus vulgaris vs Paraneoplastic Pemphigus vulgaris (PNP)

Hello friends, today let's talk about subtle differences between pemphigus Vulgaris and Paraneoplastic Pemphigus Vulgaris

Pemphigus Vulgaris usually involves buccal and labial mucosa.
PNP causes severe stomatitis as well as targetoid lesions on palms and soles much like erythema multiforme.

In Pemphigus Vulgaris antibodies are directed against intercellular adhesion molecules desmoglein-1 and desmoglein-3.
However, in PNP apart from desmoglein-1 and desmoglein-3 antibodies are also directed against envoplakin, plectin, desmoplakin, periplakin, and BPAG-1. 

In Pemphigus Vulgaris typical chicken-wire pattern is seen due to the intercellular deposition of IgG and C3
In PNP, that is not the case as although there is IgG deposition in all layers but not intercellularly and furthermore C3 is deposited along basement membrane as in Bullous pemphigoid.

In Pemphigus Vulgaris it is rare while in PNP often mucosa of the esophagus, stomach, duodenum, intestines, and the pulmonary epithelium is seen.

Prognosis is quite poor in PNP with bronchiolitis obliterans and sepsis being chief complications.
Mostly seen in Non- Hodgkin's lymphoma and CLL.

- Kirtan Patolia

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