Pemphigus

Group of autoimmune blistering diseases. Pemphigus foliaceus (PF) is confined to the skin. Pemphigus vulgaris (PV) and paraneoplastic pemphigus (PNP) can involve the skin and mucosal surfaces of the eyes, mouth, nasopharynx, and esophagus.

Diagnosis is based on clinical findings of epidermal loss, erosions, and superficial blisters of the skin, mucosa, or both.

Direct immunofluorescence staining of the skin shows immunoglobulin and C3 deposits on the keratinocyte surface, and histology reveals keratinocyte acantholysis.

Treatment aims to reduce or eliminate pathologic autoantibodies and consists of long-term immunosuppressive therapy.

Outlook for patients with PV and PF is good if the disease is adequately controlled. PNP is associated with malignancy, particularly non-Hodgkin lymphoma, and mortality can approach 90% due to respiratory failure from bronchiolitis obliterans.

Pemphigus describes a group of autoimmune blistering diseases that involve the epidermal surfaces of the skin, mucosa, or both. There are 3 broad categories: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP). Pemphigus is mediated by an autoantibody that binds a specific component of the desmosomal plaque. The desmosomal plaque holds the keratinocytes of the skin and mucosa together.