Early diagnosis may permit successful treatment with only low levels of medication. Consult a dermatologist if there are any persistent skin or mouth lesions. Because they are so rare, pemphigus and pemphigoid are often the last disease considered during diagnosis.
For a definite diagnosis, doctors should consider:
- Clinical presentation — visual examination of skin lesions.
- Lesion biopsy — A sample of the blistered skin is removed and examined under the microscope. Additionally, the layer of skin in which cell-to-cell separation occurs can be determined.
- Direct immunoflourescence — The skin sample is treated to detect desmoglein antibodies in the skin. The presence of these antibodies indicates pemphigus.
- Indirect Immunofluorescence or antibody titer test. This measures desmoglein autoantibodies in the blood serum. It may be used to obtain a more complete understanding of the course of the disease.
- ELISA A serum assay for desmoglein antibodies, known as ELISA, is also available. Although in many cases there is a correlation between ELISA and disease activity it is not so in every case.
Please note that immunoflourescent testing (testing for circulating antibodies) for MMP is highly unreliable and is negative in the majority of cases. It is important to obtain positive direct immunoflourescence results (biopsies), even if it requires repeat biopsies because it can mimic other diseases such as lichen planus.
If the mouth and nose are the only involvement, treatment should be limited to topical steroids, intralesional steroid injections, or occasional short bursts of oral corticosteroids.
If only the gums are involved, topical therapy applied with flexible dental trays (similar to the disposable molds used to deliver fluoride treatments to the teeth).
A different situation exists if the eyes, esophagus or larynx are involved. In such cases, involvement could include blindness and asphyxiation (due to scarring), and aggressive systemic therapy is warranted. Systemic steroids may not adequately control progression; most other therapies only slow the scarring process. The treatment of choice is oral cyclophosphamide (Cytoxan). About 3/4 of the patients treated with this regimen tolerate the drug. Most of these patients will have complete clinical remission after all drugs are withdrawn.
Occasionally, patients respond to oral dapsone. Those that respond well do so quickly, but unfortunately, these patients are in the minority. Azathioprine and Mycophenolate Mofetil is an alternative for patients who cannot tolerate Cytoxan. Cyclosporine is not recommended for use in MMP/CP. Recently, the biologics have been noted as being an alternative treatment for MMP/CP – specifically rituximab, etanercept, and infliximab.
