Pemphigus Vulgaris

Pemphigus is a group of rare autoimmune blistering diseases of the skin and/or mucous membranes. Pemphigus is chronic and progressive and characterised by blisters and ulcers on the skin and mucous membranes.

The oral mucosa is often the first site of involvement. Typically patients will have had multiple oral ulcers (broken blisters) that persist for weeks to months. Pemphigus Vulgaris affects the oral mucosa in nearly all cases (>80%) and affects the oral mucosa first in the majority of cases (c), and may in fact be the only site in patients.
A minority will present only with cutaneous erosions. Skin involved is typically the upper chest, back, scalp, and face, but lesions can occur on any part of the body. Note that the skin dominant form manifests the Dsg1 autoantibody profile (as in pemphigus foliaceus), whereas the mucosally dominant form manifests the Dsg3 autoantibody profile. A mixed pattern of skin and mucosa manifests a mixed Dsg1 and Dsg3 autoantibody profile. The condition progresses over weeks to months.

Sites often overlooked include around the nails (manifest as painful, red, and swollen), the pharynx and larynx (pain on swallowing and hoarseness), and the nasal cavity (nasal congestion and a bloody mucous discharge, particularly noticeable upon blowing the nose in the morning). As many as 49% of patients were shown to have laryngeal and nasal involvement (a).

Antibodies that attack one’s own cells are called autoantibodies. The part of the cells that are attacked in pemphigus are proteins (desmogleins) which act as the glue that attaches adjacent skin cells, keeping the skin intact. When autoantibodies attack desmogleins, the cells become separated from each other. The skin virtually becomes unglued. This causes burn-like lesions or blisters that do not heal naturally. In some cases, these blisters can cover a significant area of the skin.
The disease appears to affect people across racial and cultural lines, so it’s not possible to predict who may get pemphigus. Although fairly common in adults, pemphigus is rare in children. In children, it is most often caused by placental transmission of IgG autoantibodies in utero from a woman who has pemphigus.

If left untreated, the erosions and bullae of pemphigus vulgaris gradually spread, involving an increasing surface area, and can become complicated by severe infections and metabolic disturbances.
Before the advent of corticosteroids, pemphigus had a high fatality rate, with approximately 70+% of patients dying within a year (q, p).

With treatment, lesions can heal normally without scarring and the hyperpigmentation associated with pemphigus often resolves after several months. Most patients treated for pemphigus will enter a partial remission within 2 to 5 years. (In a longitudinal study of outcome in 40 patients with pemphigus vulgaris, 45% entered a complete and long-term remission after 5 years and 71% after 10 years.

Pemphigus Vulgaris (PV)

  • The term “vulgar” means “common,” and PV is the most commonly diagnosed form of pemphigus.
  • Sores and blisters almost always start in the mouth. The blisters can go as far down as the vocal cords, but no further.
  • The lesions are painful. Sometimes there is the effect that when just touching the skin can cause it to tear.
  • PV does not cause permanent scaring unless there is infection associated with the sore.
  • Today, with current therapies, the mortality rate is between 5% to 15%.
The information above was obtained from The International Pemphigus Pemphigoid Foundation.  To learn more, visit