Sections

IgA Pemphigus

Medication

Medication Summary

In general, corticosteroids are the mainstay of treatment for IgA pemphigus. Dapsone, a medication with antineutrophilic effects, also may be helpful. Anti-inflammatory agents theoretically block the inflammatory process and improve the disease conditions.^{[41, 43]}

Class Summary

IgA pemphigus is characterized histologically by inflammatory cell (neutrophil) infiltration in the upper epidermis. Anti-inflammatory agents theoretically block the inflammatory process and improve the disease conditions.

Prednisone (Deltasone)

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Prednisone is an effective treatment for IgA pemphigus. It is an immunosuppressant for the treatment of autoimmune disorders; it may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Prednisone stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. Consult a pediatrician before prescribing this medication in children.

Dapsone (Avlosulfon)

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Dapsone is bactericidal and bacteriostatic against mycobacteria; its mechanism of action is similar to sulfonamides, in which competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Dapsone is used alone or in conjunction with other anti-inflammatory medications for treating IgA pemphigus. Consult a pediatrician before prescribing medication in children.

Acitretin (Soriatane)

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Acitretin is a retinoic acid analog. Its mechanism of action is unknown. Acitretin has been used effectively in several reported cases of IgA pemphigus.

Tsuruta D, Ishii N, Hamada T, Ohyama B, Fukuda S, Koga H, et al. IgA pemphigus. Clin Dermatol. 2011 Jul-Aug. 29(4):437-42. [QxMD MEDLINE Link].
Sticherling M, Erfurt-Berge C. Autoimmune blistering diseases of the skin. Autoimmun Rev. 2012 Jan. 11(3):226-30. [QxMD MEDLINE Link].
Hashimoto T, Teye K, Ishii N. Clinical and immunological studies of 49 cases of various types of intercellular IgA dermatosis and 13 cases of classical subcorneal pustular dermatosis examined at Kurume University. Br J Dermatol. 2016 Jun 3. [QxMD MEDLINE Link].
Ali S, Kelly C, Challacombe SJ, Donaldson AN, Bhogal BS, Setterfield JF. Serum and salivary IgG and IgA antibodies to desmoglein 3 in mucosal pemphigus vulgaris. Br J Dermatol. 2016 Jul. 175 (1):113-21. [QxMD MEDLINE Link].
Aslanova M, Yarrarapu SNS, Zito PM. IgA Pemphigus. StatPearls [Internet]. 2021 Jan. [QxMD MEDLINE Link]. [Full Text].
Toosi S, Collins JW, Lohse CM, Wolz MM, Wieland CN, Camilleri MJ, et al. Clinicopathologic features of IgG/IgA pemphigus in comparison with classic (IgG) and IgA pemphigus. Int J Dermatol. 2016 Apr. 55 (4):e184-90. [QxMD MEDLINE Link].
Kridin K, Patel PM, Jones VA, Cordova A, Amber KT. IgA pemphigus: A systematic review. J Am Acad Dermatol. 2020 Jun. 82 (6):1386-1392. [QxMD MEDLINE Link].
Criscito MC, Cohen JM, Toosi S, Penn LA, Ross CL, Park J, et al. A retrospective study on the clinicopathologic features of IgG/IgA pemphigus. J Am Acad Dermatol. 2021 Jul. 85 (1):237-240. [QxMD MEDLINE Link].
Santiago-et-Sánchez-Mateos D, Juárez Martín A, González De Arriba A, Delgado Jiménez Y, Fraga J, Hashimoto T, et al. IgG/IgA pemphigus with IgA and IgG antidesmoglein 1 antibodies detected by enzyme-linked immunosorbent assay: presentation of two cases. J Eur Acad Dermatol Venereol. 2011 Jan. 25 (1):110-2. [QxMD MEDLINE Link].
Hashimoto T, Komai A, Futei Y, Nishikawa T, Amagai M. Detection of IgA autoantibodies to desmogleins by an enzyme-linked immunosorbent assay: the presence of new minor subtypes of IgA pemphigus. Arch Dermatol. 2001 Jun. 137(6):735-8. [QxMD MEDLINE Link].
Geller S, Gat A, Zeeli T, Hafner A, Eming R, Hertl M, et al. The expanding spectrum of IgA pemphigus: a case report and review of the literature. Br J Dermatol. 2014 Sep. 171 (3):650-6. [QxMD MEDLINE Link].
Geller S, Sprecher E. IgA pemphigus: lumping or splitting?. Br J Dermatol. 2017 Aug. 177 (2):581-582. [QxMD MEDLINE Link].
Ishii N, Ishida-Yamamoto A, Hashimoto T. Immunolocalization of target autoantigens in IgA pemphigus. Clin Exp Dermatol. 2004 Jan. 29(1):62-6. [QxMD MEDLINE Link].
Amagai M, Klaus-Kovtun V, Stanley JR. Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion. Cell. 1991 Nov 29. 67(5):869-77. [QxMD MEDLINE Link].
Amagai M, Koch PJ, Nishikawa T, Stanley JR. Pemphigus vulgaris antigen (desmoglein 3) is localized in the lower epidermis, the site of blister formation in patients. J Invest Dermatol. 1996 Feb. 106(2):351-5. [QxMD MEDLINE Link].
Carayannopoulos L, Hexham JM, Capra JD. Localization of the binding site for the monocyte immunoglobulin (Ig) A-Fc receptor (CD89) to the domain boundary between Calpha2 and Calpha3 in human IgA1. J Exp Med. 1996 Apr 1. 183(4):1579-86. [QxMD MEDLINE Link].
Ebihara T, Hashimoto T, Iwatsuki K, et al. Autoantigens for IgA anti-intercellular antibodies of intercellular IgA vesiculopustular dermatosis. J Invest Dermatol. 1991 Oct. 97(4):742-5. [QxMD MEDLINE Link].
Hashimoto T, Ebihara T, Nishikawa T. Studies of autoantigens recognized by IgA anti-keratinocyte cell surface antibodies. J Dermatol Sci. 1996 Apr. 12(1):10-7. [QxMD MEDLINE Link].
Hashimoto T, Inamoto N, Nakamura K, Nishikawa T. Intercellular IgA dermatosis with clinical features of subcorneal pustular dermatosis. Arch Dermatol. 1987 Aug. 123(8):1062-5. [QxMD MEDLINE Link].
Hashimoto T, Kiyokawa C, Mori O, et al. Human desmocollin 1 (Dsc1) is an autoantigen for the subcorneal pustular dermatosis type of IgA pemphigus. J Invest Dermatol. 1997 Aug. 109(2):127-31. [QxMD MEDLINE Link].
Karpati S, Amagai M, Liu WL, Dmochowski M, Hashimoto T, Horvath A. Identification of desmoglein 1 as autoantigen in a patient with intraepidermal neutrophilic IgA dermatosis type of IgA pemphigus. Exp Dermatol. 2000 Jun. 9(3):224-8. [QxMD MEDLINE Link].
Wang J, Kwon J, Ding X, Fairley JA, Woodley DT, Chan LS. Nonsecretory IgA1 autoantibodies targeting desmosomal component desmoglein 3 in intraepidermal neutrophilic IgA dermatosis. Am J Pathol. 1997 Jun. 150(6):1901-7. [QxMD MEDLINE Link].
Fujihashi K, McGhee JR, Kweon MN, et al. gamma/delta T cell-deficient mice have impaired mucosal immunoglobulin A responses. J Exp Med. 1996 Apr 1. 183(4):1929-35. [QxMD MEDLINE Link].
Petropoulou H, Politis G, Panagakis P, Hatziolou E, Aroni K, Kontochristopoulos G. Immunoglobulin A pemphigus associated with immunoglobulin A gammopathy and lung cancer. J Dermatol. 2008 Jun. 35(6):341-5. [QxMD MEDLINE Link].
Chen L, Yang B, Fan J, Yang K, Liu H, Wu G. Peripheral T-cell lymphoma complicated by immunoglobulin A pemphigus: A case report and literature review. Oncol Lett. 2014 Jul. 8(1):62-66. [QxMD MEDLINE Link]. [Full Text].
Bruckner AL, Fitzpatrick JE, Hashimoto T, Weston WL, Morelli JG. Atypical IgA/IgG pemphigus involving the skin, oral mucosa, and colon in a child: a novel variant of IgA pemphigus?. Pediatr Dermatol. 2005 Jul-Aug. 22(4):321-7. [QxMD MEDLINE Link].
Duker I, Schaller J, Rose C, Zillikens D, Hashimoto T, Kunze J. Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3. Arch Dermatol. 2009 Oct. 145(10):1159-62. [QxMD MEDLINE Link].
Muller R, Heber B, Hashimoto T, et al. Autoantibodies against desmocollins in European patients with pemphigus. Clin Exp Dermatol. 2009 Dec. 34(8):898-903. [QxMD MEDLINE Link].
Chan LS, Vanderlugt CJ, Hashimoto T, et al. Epitope spreading: lessons from autoimmune skin diseases. J Invest Dermatol. 1998 Feb. 110(2):103-9. [QxMD MEDLINE Link].
Chowdhury J, Datta PK, Chowdhury SN, Das NK. A Clinicopathological Study of Pemphigus in Eastern India with Special Reference to Direct Immunofluorescence. Indian J Dermatol. 2016 May-Jun. 61 (3):288-94. [QxMD MEDLINE Link].
de Oliveira JP, Gabbi TV, Hashimoto T, et al. Two Brazilian cases of IgA pemphigus. J Dermatol. 2003 Dec. 30(12):886-91. [QxMD MEDLINE Link].
Gniadecki R, Bygum A, Clemmensen O, Svejgaard E, Ullman S. IgA pemphigus: the first two Scandinavian cases. Acta Derm Venereol. 2002. 82(6):441-5. [QxMD MEDLINE Link].
Nanda A, Dvorak R, Al-Saeed K, Al-Sabah H, Alsaleh QA. Spectrum of autoimmune bullous diseases in Kuwait. Int J Dermatol. 2004 Dec. 43(12):876-81. [QxMD MEDLINE Link].
Tsuruta D, Ishii N, Hamada T, Ohyama B, Fukuda S, Koga H, et al. IgA pemphigus. Clin Dermatol. 2011 Jul-Aug. 29 (4):437-42. [QxMD MEDLINE Link].
Kridin K. Pemphigus group: overview, epidemiology, mortality, and comorbidities. Immunol Res. 2018 Apr. 66 (2):255-270. [QxMD MEDLINE Link].
Chorzelski TP, Beutner EH, Kowalewski C, et al. IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis. J Am Acad Dermatol. 1991 May. 24(5 Pt 2):839-44. [QxMD MEDLINE Link].
Erdag G, Qureshi HS, Greer KE, Patterson JW. Immunoglobulin A pemphigus involving the perianal skin and oral mucosa: an unusual presentation. Cutis. 2007 Sep. 80(3):218-20. [QxMD MEDLINE Link].
Neethu KC, Rao R, Balachandran C, Pai S. Juvenile IgA pemphigus: A case report and review of literature. Indian J Dermatol Venereol Leprol. 2016 Jul-Aug. 82 (4):439-42. [QxMD MEDLINE Link].
Porro AM, Caetano Lde V, Maehara Lde S, Enokihara MM. Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus. An Bras Dermatol. 2014 Jan-Feb. 89 (1):96-106. [QxMD MEDLINE Link].
Howell SM, Bessinger GT, Altman CE, Belnap CM. Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. J Am Acad Dermatol. 2005 Sep. 53(3):541-3. [QxMD MEDLINE Link].
Gruss C, Zillikens D, Hashimoto T, et al. Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin. J Am Acad Dermatol. 2000 Nov. 43(5 Pt 2):923-6. [QxMD MEDLINE Link].
Bliziotis I, Rafailidis P, Vergidis P, Falagas ME. Regression of subcorneal pustular dermatosis type of IgA pemphigus lesions with azithromycin. J Infect. 2005 Aug. 51 (2):E31-4. [QxMD MEDLINE Link].
Ruiz-Genao DP, Hernandez-Nunez A, Hashimoto T, Amagai M, Fernandez-Herrera J, Garcia-Diez A. A case of IgA pemphigus successfully treated with acitretin. Br J Dermatol. 2002 Nov. 147(5):1040-2. [QxMD MEDLINE Link].

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 Medication

Medication Summary

Anti-inflammatory agents

Class Summary

Prednisone (Deltasone)

Prednisone (Deltasone)

Dapsone (Avlosulfon)

Dapsone (Avlosulfon)

Acitretin (Soriatane)

Acitretin (Soriatane)

References

Tables

Contributor Information and Disclosures

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