It usually manifests on the face and scalp, but also involves the back and chest. Lesions do not occur in the mouth. The blisters are more confined to the outermost surface and often itch. Topical steroids may be effective. The mortality rate associated with this form of pemphigus is lower than the other forms described.
Paraneoplastic pemphigus is very rare and generally occurs in people who have cancer .
Due to involvement of the airways, signs of respiratory disease may occur and can be life-threatening. Aggressive immunosuppressive therapy is used to treat the disease. If the cancer can be surgically removed, patients may show marked improvement.