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.^[40]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 subcorneal pustular dermatosis (SPD)–type IgA pemphigus with oral isotretinoin treatment.^{[41, 42]}

Activity

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

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 restriction 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.

Medication

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Media Gallery

Histopathologic examination of a blister lesion obtained from a patient with immunoglobulin A pemphigus shows a suprabasal blistering process, acantholysis, and an inflammatory cell infiltrate containing numerous neutrophils (hematoxylin and eosin, original magnification X50).
Indirect immunofluorescence microscopy performed on monkey esophagus substrate (with serum from a patient with immunoglobulin A pemphigus) detects the immunoglobulin A1 subclass of circulating autoantibodies that label the epithelial cell surfaces of the substrate.
Histopathologic examination of a blister lesion obtained from a punch biopsy shows prominent subcorneal and intraepidermal pustule formation with numerous neutrophils (hematoxylin and eosin, X40).
Histopathologic examination of a blister lesion obtained from a punch biopsy shows prominent subcorneal and intraepidermal pustule formation with numerous neutrophils (hematoxylin and eosin, X40).

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Author

Jose A Plaza, MD Director of Dermatopathology, Professor of Pathology and Dermatology, The Ohio State University Wexner Medical Center

Jose A Plaza, MD is a member of the following medical societies: American Medical Association, American Society for Clinical Pathology, American Society of Dermatopathology, International Society of Dermatopathology

Disclosure: Nothing to disclose.

Coauthor(s)

Jesse D Sheldon, DO Resident Physician in Anatomic and Clinical Pathology, Department of Pathology, The Ohio State University Wexner Medical Center

Jesse D Sheldon, DO is a member of the following medical societies: American Medical Association, American Society for Clinical Pathology, American Society of Dermatopathology, College of American Pathologists

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Edward F Chan, MD Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine

Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Neil Sandhu, MD, FAAD Dermatologist (Medical/Cosmetic) and Mohs Surgeon, Gulf Coast Dermatology

Neil Sandhu, MD, FAAD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery

Disclosure: Nothing to disclose.

Blanca Anais Estupiñan University of Central Florida College of Medicine

Blanca Anais Estupiñan is a member of the following medical societies: American College of Physicians, American Medical Association, American Psychiatric Association

Disclosure: Nothing to disclose.

Acknowledgements

Lawrence S Chan, MD Dr Orville J Stone Professor of Dermatology, Head, Department of Dermatology, University of Illinois College of Medicine

Lawrence S Chan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Dermatological Association, American Medical Association, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Illinois State Medical Society, Microcirculatory Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Takeji Nishikawa, MD Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan, Inc

Disclosure: Nothing to disclose.

IgA Pemphigus Treatment & Management

Medical Care

Activity

Complications

References

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