Blistering Diseases – Pemphigus Vulgaris

Pemphigus Vulgaris is a blistering disease that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices.  Clinically, the lesions of Pemphigus Vulgaris are very fragile blisters (bullae) that rupture easily and leave erosions and ulcers on the skin.  The oral cavity is the most common location for Pemphigus Vulgaris to arise and is usually the first anatomic site a patient develops this disorder.  However, blisters can form anywhere on the body including the face, arms, legs, neck, trunk and groin.  There are two clinical diagnostic tests that can be performed: The Nikolsky sign and The Asboe-Hansen sign.  The Nikolsky sign involves applying pressure to normal appearing skin to elicit the formation of a blister.  The Asboe-Hansen sign involves putting vertical pressure on an intact blister and observing the subsequent lateral spread of the blister.  Pemphigus Vulgaris is very rare in young people and usually occurs in the elderly.  The cause of Pemphigus Vulgaris is an autoimmune attack with antibodies directed towards certain structural areas holding the epidermis and dermis intact.  Thus, without a strong connection these two layers split forming a blister and ultimately an ulcer.  Many medicines have been implicated in inducing Pemphigus Vulgaris especially the ACE inhibitors used for blood pressure control.  Pemphigus Vulgaris can be life threatening due to severe electrolyte imbalances from fluid loss as well as infections leading to sepsis.  Treatment involves removing any culprit medications and immunosuppressive medication.