IgA Pemphigus Treatment & Management

Updated: Apr 18, 2017
  • Author: Blanca Anais Estupiñan; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Medical therapy for IgA pemphigus should be directed towards reducing inflammation, because IgA pemphigus represents a group of autoimmune blistering skin diseases manifested clinically as chronic inflammation. Because IgA pemphigus is uncommon, therapy is based on the mechanism of disease and anecdotal reports.

Generally, corticosteroids are the mainstay of treatment for IgA pemphigus. To minimize adverse effects, slow tapering of corticosteroids is advised in order to identify the lowest efficacious dose. Dapsone also may be helpful in IgA pemphigus because of its antineutrophilic effects. Rapid response to treatment with adalimumab and mycophenolate mofetil was reported in 2005. [32] One case report described lesion regression after the addition of azithromycin to a local steroid and keratolytic agent, while another case report described rapid response in SPD-type IgA pemphigus with oral isotretinoin treatment. [33, 34]

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Activity

Usually, no restrictions are placed on patient activities; however, advise IgA pemphigus patients to avoid contact sports during the active disease state.

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Complications

Infection may occur secondary to open wounds from the blistering process of IgA pemphigus or secondary to medications used to treat IgA pemphigus.

Malignancies may occur secondary to the chronic inflammatory process or secondary to medications.

Growth retardation is possible secondary to medications used to treat IgA pemphigus during childhood.

Adrenal insufficiency may result secondary to long-term prednisone treatment. Osteoporosis is a complication secondary to long-term prednisone treatment.

Other complications due to long-term therapy, specifically with corticosteroids, include anemia, hypertension, diabetes, and weight gain. Infections with oral candidal organisms and herpes simplex virus may occur.

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