Pemphigus vulgaris usually occurs in middle-aged or elderly patients and is rare in children. One variant, paraneoplastic pemphigus, occurs in older patients with malignancy, outcome is poor.

Causes

The disease is characterized by the presence of auto or self antibodies directed against intercellular adhesion molecules desmoglein-1 and desmoglein-3 in the epidermis of the skin

These antibodies binding to the skin cause separation of the cells of the top layer of the skin. This separation reaction is known as acantholysis

These auto antibodies are present in both serum and skin during active disease

Symptoms and Signs

Pemphigus is characterized by blisters on your skin and mucous membranes. The blisters rupture easily, leaving open sores, which may ooze and become infected

• Pemphigus vulgaris- the most common form, pemphigus vulgaris usually begins with blisters in your mouth, which then erupt on your skin. Blisters can also break out on the mucous membranes of your genitals. The blisters typically are painful, but don't itch
• Pemphigus foliaceus- his type doesn't usually affect mucous membranes. The blisters, which usually begin on your face and scalp and later erupt on your chest and back, usually aren't painful. They tend to be crusty and itchy
• Lateral pressure on skin adjacent to a blister causes epidermal detachment (Nikolsky's sign).
• Pressure on a blister can cause the blister to extend to adjacent skin (Asboe-Hansen sign).

Tests and diagnosis

• Skin biopsy- Biopsy of the edge of a fresh lesion and of a nearby area of normal skin is required
• Sometimes titers of antibodies against desmoglein-3 or desmoglein-1

Complications

Infection of skin and spread of infection through your bloodstream (sepsis)

Systemic infection can be fatal

Prognosis

Before systemic corticosteroids were used, pemphigus vulgaris was usually fatal; most patients died within 5 yr of disease onset.

Even with treatment, pemphigus vulgaris is a serious disease with an inconsistent and unpredictable response to treatment, a prolonged course, and virtually inevitable adverse drug effects.

Lifestyle and home remedies

• Minimize trauma to your skin
• Taking good care of your wounds can help prevent infection and scarring
• Generously sprinkling talcum powder on your sheets may help keep oozing skin from sticking

Treatment of mild pemphigus

• Corticosteroids- oral prednisone or IV methyl prednisolone
• Immunosuppressants- methotrexate, cyclophoshamide, azathioprine, rituximab
• Antibiotics and antifungal medications to prevent infections

Treatment of severe pemphigus

• Intravenous fluids with electrolytes- sodium, potassium, and calcium
• Anesthetic mouth lozenges- these can help control pain of mild to moderate mouth sores.
• Plasmapheresis and IV immunoglobulin (IVIG).