Blistering Skin Disorders

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Overview

Pemphigus of the skin and mucous membranes is a chronic blistering disease most commonly found in middle-aged persons.

There are three major types of pemphigus:

• Vulgaris (literally meaning common)
  
• Foliaceus (featuring superficial blisters)
  
• Paraneoplastic (associated with some cancers)
  

Pemphigus is an autoimmune disorder in which the skin cells are attacked by the patient's own antibodies (immune proteins). This causes a separation between the layers of skin in which fluid subsequently accumulates, producing blisters. The introduction of corticosteroids in recent decades has turned what often had been a fatal disease into one that can be managed quite well.

Causes

The etiology of pemphigus is largely unknown, although genetic predisposition is thought to be the cause. An endemic form of pemphigus foliaceus has been identified in certain rural areas of Brazil and may be transmitted by the bite of a black fly. In rare cases, a medication can induce an attack. Such cases almost always resolve after the offending drug is discontinued. In paraneoplastic pemphigus, the skin problem is caused by cancer. The underlying cancer usually is lymphoid (lymphoma or leukemia) in origin. Paraneoplastic pemphigus typically resolves after the cancer has been treated successfully. It also can be associated with benign lymph tumors.

Signs and Symptoms

The primary lesion of pemphigus is a flaccid blister that can occur anywhere on the skin, typically on the chest, groin, face, scalp, neck, and axillae (armpits). The blisters are fragile, and denuded or raw areas often are seen. This is especially so with mucosal (mouth and genitalia) lesions, where the blisters rupture soon after formation. Patients commonly present with painful oral lesions several months before other areas of the skin are affected.

In pemphigus foliaceus, blisters are rarely seen. Instead, scaly, crusted sores form, especially on the upper trunk, face, and scalp.

Paraneoplastic pemphigus usually presents with severe mouth (oral) and eye (ocular) lesions. The eruption typically resolves with the resolution of the cancer.

Diagnosis

While the clinical presentation is helpful, a skin biopsy usually confirms a diagnosis of pemphigus. In most cases, two biopsies are taken one for routine evaluation and another for immunofluorescence. This identifies which of the patient's antibodies is present and is helpful in distinguishing pemphigus from other blistering diseases.

Treatment

High-dose oral corticosteroids are the mainstay in pemphigus treatment. Doses often are increased until remission begins, at which point the amount of steroid gradually is tapered. Adverse reactions associated with prolonged high-dose steroid therapy must be monitored and managed.

People with severe disease that cannot be controlled with corticosteroids or who cannot tolerate high-dose therapy usually are prescribed a combination of immunosuppressive drugs (drugs that suppress the immune system) that help control the disease with the least amount of oral steroids.

Prognosis

Once cleared, pemphigus can recur; however, there can be long remissions and even cures.

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