Pemphigus Vulgaris

Overview

Pemphigus vulgaris is a rare, serious autoimmune blistering disease in which the immune system produces antibodies against desmoglein proteins that hold skin cells together. This causes painful, flaccid blisters and erosions on the skin and mucous membranes. Without treatment, pemphigus vulgaris can be life-threatening due to widespread skin loss and secondary infection.

Key Symptoms

Painful oral erosions and ulcers, often the first sign
Flaccid, fragile blisters on the skin that rupture easily
Nikolsky sign positive — gentle lateral pressure causes skin to shear off
Widespread, painful erosions and crusting where blisters have ruptured
Blisters arise on normal-appearing skin without surrounding redness
May affect other mucous membranes including throat, nose, and genitals

Affected Areas

Oral mucosa (most common initial site), Trunk, Scalp, Face, Groin, Any skin surface

Care Instructions

Seek urgent evaluation by a dermatologist for persistent oral ulcers or unexplained blistering.
Systemic corticosteroids are typically the first-line treatment to control the disease.
Steroid-sparing immunosuppressants (such as mycophenolate or azathioprine) are usually added.
Rituximab has become an important treatment option for moderate-to-severe disease.
Wound care for open erosions is essential to prevent secondary infection.
Regular monitoring of blood counts, liver function, and infection risk during immunosuppressive therapy.

Medical Disclaimer: This information is for educational purposes only and does not replace professional medical advice. Always consult a qualified dermatologist for diagnosis and treatment.

Prevalence: Rare; approximately 1-5 cases per million people per year