(1)
Misdiagnosis Association & Society, Seattle, WA, USA
Keywords
Pemphigus vulgarisMucocutaneous variantMucosal dominant variantDesmogleinAutoantibodiesMusocal lesionsCutaneous-only diseaseEarly endpointsLate endpointsRelapseFlareTreatment failureClinical Variants of Pemphigus Vulgaris
There are two main different variants of pemphigus vulgaris: one is the mucocutaneous variant and the other is the mucosal dominant variant [1]. The mucosal dominant variant of pemphigus vulgaris involves autoantibodies against only desmoglein 3, whereas the mucocutaneous variant involves autoantibodies against both desmoglein 1 and 3. Some patients, however, report never having musocal lesions and are classified as having cutaneous-only disease .
Pemphigus vegetans is also a clinical variant of pemphigus vulgaris that involves erosions that evolve into fungoid or papillomatous growths [1]. These fungoid or vegetative growths are mainly seen on the scalp or face. This clinical variant is fairly rare and involves two subtypes: mild Hallopeau and severe Neumann types.
Classification of Pemphigus Disease Activity
According to the “Consensus Statement on Definitions of Disease Endpoints and Therapeutic Response for Pemphigus,” all pemphigus diseases can be classified according to the level of disease activity [2]. Knowing the disease activity level of pemphigus vulgaris can aid physicians in their decisions about which treatment to start and when in the course of the disease.