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
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.[24, 25]
Prednisone (Deltasone)
Effective treatment for IgA pemphigus. Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. Consult the pediatrician before prescribing medication in children.
Dapsone (Avlosulfon)
Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to sulfonamides in which competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Used alone or in conjunction with other anti-inflammatory medications for treating IgA pemphigus. Consult the pediatrician before prescribing medication in children.
Acitretin (Soriatane)
Retinoic acid analog. Mechanism of action is unknown. Used effectively in several reported cases of IgA pemphigus.
Amagai M, Klaus-Kovtun V, Stanley JR. Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion. Cell. Nov 29 1991;67(5):869-77. [Medline].
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. Feb 1996;106(2):351-5. [Medline].
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. Apr 1 1996;183(4):1579-86. [Medline].
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. [Medline].
Nanda A, Dvorak R, Al-Saeed K, Al-Sabah H, Alsaleh QA. Spectrum of autoimmune bullous diseases in Kuwait. Int J Dermatol. Dec 2004;43(12):876-81. [Medline].
Suzuki M, Karube S, Kobori Y, et al. IgA pemphigus occurring in a 1-month-old infant. J Am Acad Dermatol. Feb 2003;48(2 Suppl):S22-4. [Medline].
Chorzelski TP, Beutner EH, Kowalewski C, et al. IgA pemphigus foliaceus with a clinical presentation of pemphigus herpetiformis. J Am Acad Dermatol. May 1991;24(5 Pt 2):839-44. [Medline].
Erdag G, Qureshi HS, Greer KE, Patterson JW. Immunoglobulin A pemphigus involving the perianal skin and oral mucosa: an unusual presentation. Cutis. Sep 2007;80(3):218-20. [Medline].
Ebihara T, Hashimoto T, Iwatsuki K, et al. Autoantigens for IgA anti-intercellular antibodies of intercellular IgA vesiculopustular dermatosis. J Invest Dermatol. Oct 1991;97(4):742-5. [Medline].
Hashimoto T, Ebihara T, Nishikawa T. Studies of autoantigens recognized by IgA anti-keratinocyte cell surface antibodies. J Dermatol Sci. Apr 1996;12(1):10-7. [Medline].
Hashimoto T, Inamoto N, Nakamura K, Nishikawa T. Intercellular IgA dermatosis with clinical features of subcorneal pustular dermatosis. Arch Dermatol. Aug 1987;123(8):1062-5. [Medline].
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. Aug 1997;109(2):127-31. [Medline].
Ishii N, Ishida-Yamamoto A, Hashimoto T. Immunolocalization of target autoantigens in IgA pemphigus. Clin Exp Dermatol. Jan 2004;29(1):62-6. [Medline].
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. Jun 2000;9(3):224-8. [Medline].
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. Jun 1997;150(6):1901-7. [Medline].
Fujihashi K, McGhee JR, Kweon MN, et al. gamma/delta T cell-deficient mice have impaired mucosal immunoglobulin A responses. J Exp Med. Apr 1 1996;183(4):1929-35. [Medline].
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. Jun 2008;35(6):341-5. [Medline].
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. Jul-Aug 2005;22(4):321-7. [Medline].
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. Oct 2009;145(10):1159-62. [Medline].
Muller R, Heber B, Hashimoto T, et al. Autoantibodies against desmocollins in European patients with pemphigus. Clin Exp Dermatol. Dec 2009;34(8):898-903. [Medline].
Chan LS, Vanderlugt CJ, Hashimoto T, et al. Epitope spreading: lessons from autoimmune skin diseases. J Invest Dermatol. Feb 1998;110(2):103-9. [Medline].
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. Jun 2001;137(6):735-8. [Medline].
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. Sep 2005;53(3):541-3. [Medline].
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. Nov 2000;43(5 Pt 2):923-6. [Medline].
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. Nov 2002;147(5):1040-2. [Medline].
Amagai M, Tsunoda K, Suzuki H, Nishifuji K, Koyasu S, Nishikawa T. Use of autoantigen-knockout mice in developing an active autoimmune disease model for pemphigus. J Clin Invest. Mar 2000;105(5):625-31. [Medline].
Beutner EH, Chorzelski TP, Wilson RM, et al. IgA pemphigus foliaceus. Report of two cases and a review of the literature. J Am Acad Dermatol. Jan 1989;20(1):89-97. [Medline].
Coffman RL, Shrader B, Carty J, Mosmann TR, Bond MW. A mouse T cell product that preferentially enhances IgA production. I. Biologic characterization. J Immunol. Dec 1 1987;139(11):3685-90. [Medline].
de Oliveira JP, Gabbi TV, Hashimoto T, et al. Two Brazilian cases of IgA pemphigus. J Dermatol. Dec 2003;30(12):886-91. [Medline].
Hall RP, Lawley TJ. Characterization of circulating and cutaneous IgA immune complexes in patients with dermatitis herpetiformis. J Immunol. Sep 1985;135(3):1760-5. [Medline].
Hodak E, David M, Ingber A, et al. The clinical and histopathological spectrum of IgA-pemphigus--report of two cases. Clin Exp Dermatol. Nov 1990;15(6):433-7. [Medline].
Huff JC, Golitz LE, Kunke KS. Intraepidermal neutrophilic IgA dermatosis. N Engl J Med. Dec 26 1985;313(26):1643-5. [Medline].
Robinson ND, Hashimoto T, Amagai M, Chan LS. The new pemphigus variants. J Am Acad Dermatol. May 1999;40(5 Pt 1):649-71; quiz 672-3. [Medline].
Tagami H, Iwatsuki K, Iwase Y, Yamada M. Subcorneal pustular dermatosis with vesiculo-bullous eruption. Demonstration of subcorneal IgA deposits and a leukocyte chemotactic factor. Br J Dermatol. Nov 1983;109(5):581-7. [Medline].
Wallach D, Janssen F, Vignon-Pennamen MD, Lemarchand-Venencie F, Cottenot F. Atypical neutrophilic dermatosis with subcorneal IgA deposits. Arch Dermatol. Jun 1987;123(6):790-5. [Medline].
Weisbart RH, Kacena A, Schuh A, Golde DW. GM-CSF induces human neutrophil IgA-mediated phagocytosis by an IgA Fc receptor activation mechanism. Nature. Apr 14 1988;332(6165):647-8. [Medline].
Zillikens D, Miller K, Hartmann AA, Burg G. IgA pemphigus foliaceus: a case report. Dermatologica. 1990;181(4):304-7. [Medline].

