A retrospective study of immunoglobulin A pemphigus_West China Medical Journal

Authors：

WEI Mintong , FENG Xun , LI Jishu , ZOU Min , WANG Mi ,  LI Wei

Department of Dermatology and Venereology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding?author：

LI Wei, Email: liwei@wchscu.edu.cn

Keywords：

Immunoglobulin A pemphigus; immunofluorescence; immunoblotting; histopathology; treatment

DOI：

10.7507/1002-0179.202511259

Video：

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Objective To investigate the clinical features, pathological and immunological manifestations, as well as treatment outcomes in patients with immunoglobulin (Ig) A pemphigus. Methods The clinical, pathological, immunological, and follow-up data of IgA pemphigus patients diagnosed in the Department of Dermatology and Venereology, West China Hospital of Sichuan University between January 1, 2014 and December 31, 2024 were retrospectively analyzed. Results Ultimately, 8 patients were included, including 6 females and 2 males, with an average age of onset of 42.4 years and an average age of medical consultation of 45.3 years. All 8 patients presented with erythema and papules, including 6 cases with yellow white pustules and 2 cases with blisters. The affected areas of the skin lesions included the trunk, limbs, head and neck, and palms and soles, with 4 cases diagnosed with subcorneal pustular dermatosis type IgA pemphigus, 1 case diagnosed with intraepidermal neutrophilic dermatosis type, and the remaining 3 cases had atypical histopathological changes. The 7 successfully followed up patients achieved disease activity control, and during the follow-up period, 5 patients experienced at least 1 recurrence. A total of 6 patients completed itching assessment before and after treatment (3 severe itching and 1 moderate itching), and after treatment, the itching in all patients decreased to mild. Conclusions IgA pemphigus patients often present with blisters and pustules on the trunk and limbs, with a higher incidence of itching. Although most patients have a good response to treatment, recurrence is not uncommon during follow-up.

Citation： WEI Mintong, FENG Xun, LI Jishu, ZOU Min, WANG Mi, LI Wei. A retrospective study of immunoglobulin A pemphigus. West China Medical Journal, 2026, 41(1): 42-46. doi: 10.7507/1002-0179.202511259 Copy

1.	Kridin K, Patel PM, Jones VA, et al. IgA pemphigus: a systematic review. J Am Acad Dermatol, 2020, 82(6): 1386-1392.
2.	Tsuruta D, Ishii N, Hamada T, et al. IgA pemphigus. Clin Dermatol, 2011, 29(4): 437-442.
3.	Porro AM, Caetano Lde V, Maehara Lde S, et al. Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus. An Bras Dermatol, 2014, 89(1): 96-106.
4.	Anouar I, Oukabli M, Hjira N, et al. IgA pemphigus. Presse Med, 2019, 48(4): 461-464.
5.	Wallach D. Vintage descriptions of IgA pemphigus. J Eur Acad Dermatol Venereol, 2022, 36(12): e1012.
6.	Geller S, Gat A, Zeeli T, et al. The expanding spectrum of IgA pemphigus: a case report and review of the literature. Br J Dermatol, 2014, 171(3): 650-656.
7.	Solimani F, Meier K, Zimmer CL, et al. Immune serological diagnosis of pemphigus. Ital J Dermatol Venerol, 2021, 156(2): 151-160.
8.	Feng X, Zheng H, Wang M, et al. Autoimmune bullous diseases: pathogenesis and clinical management. Mol Biomed, 2025, 6(1): 30.
9.	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, 2017, 176(1): 168-175.
10.	Hegazy S, Bouchouicha S, Khaled A, et al. IgA pemphigus showing IgA antibodies to desmoglein 1 and 3. Dermatol Pract Concept, 2016, 6(4): 31-33.
11.	Hashimoto T. Immunopathology of IgA pemphigus. Clin Dermatol, 2001, 19(6): 683-689.
12.	中國醫師協會皮膚科醫師分會, 中華醫學會皮膚性病學分會治療學組, 中國醫療保健國際交流促進會皮膚醫學分會, 等. 中國天皰瘡診療指南(2024 版). 中華皮膚科雜志, 2024, 57(10): 873-886.
13.	Moreno AC, Santi CG, Gabbi TV, et al. IgA pemphigus: case series with emphasis on therapeutic response. J Am Acad Dermatol, 2014, 70(1): 200-201.
14.	詹同英, 陳澤镕, 蘭小夢, 等. 多重自身免疫綜合征一例并文獻復習. 中國麻風皮膚病雜志, 2020, 36(2): 94-96.
15.	Zhou Y, Xiao Y, Wang Y, et al. Refractory atypical IgA pemphigus successfully treated with apremilast. J Dermatol, 2024, 51(3): e86-e87.
16.	Koga H, Tsutsumi M, Teye K, et al. Subcorneal pustular dermatosis-type IgA pemphigus associated with multiple myeloma: a case report and literature review. J Dermatol, 2023, 50(2): 234-238.
17.	van Beek N, Holtsche MM, Atefi I, et al. State-of-the-art diagnosis of autoimmune blistering diseases. Front Immunol, 2024, 15: 1363032.
18.	Li J, Feng X, Wang M, et al. Demographic characteristics and disease severity associated with IgA/IgG deposition patterns in autoimmune bullous diseases: a cohort study based on a registry database. Front Immunol, 2025, 16: 1565073.
19.	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, 1989, 20(1): 89-97.
20.	Geng RSQ, Sood S, Bestavros S, et al. Treatment outcomes in IgA pemphigus: a systematic review. J Cutan Med Surg, 2025, 29(4): 420-421.

1. Kridin K, Patel PM, Jones VA, et al. IgA pemphigus: a systematic review. J Am Acad Dermatol, 2020, 82(6): 1386-1392.
2. Tsuruta D, Ishii N, Hamada T, et al. IgA pemphigus. Clin Dermatol, 2011, 29(4): 437-442.
3. Porro AM, Caetano Lde V, Maehara Lde S, et al. Non-classical forms of pemphigus: pemphigus herpetiformis, IgA pemphigus, paraneoplastic pemphigus and IgG/IgA pemphigus. An Bras Dermatol, 2014, 89(1): 96-106.
4. Anouar I, Oukabli M, Hjira N, et al. IgA pemphigus. Presse Med, 2019, 48(4): 461-464.
5. Wallach D. Vintage descriptions of IgA pemphigus. J Eur Acad Dermatol Venereol, 2022, 36(12): e1012.
6. Geller S, Gat A, Zeeli T, et al. The expanding spectrum of IgA pemphigus: a case report and review of the literature. Br J Dermatol, 2014, 171(3): 650-656.
7. Solimani F, Meier K, Zimmer CL, et al. Immune serological diagnosis of pemphigus. Ital J Dermatol Venerol, 2021, 156(2): 151-160.
8. Feng X, Zheng H, Wang M, et al. Autoimmune bullous diseases: pathogenesis and clinical management. Mol Biomed, 2025, 6(1): 30.
9. 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, 2017, 176(1): 168-175.
10. Hegazy S, Bouchouicha S, Khaled A, et al. IgA pemphigus showing IgA antibodies to desmoglein 1 and 3. Dermatol Pract Concept, 2016, 6(4): 31-33.
11. Hashimoto T. Immunopathology of IgA pemphigus. Clin Dermatol, 2001, 19(6): 683-689.
12. 中國醫師協會皮膚科醫師分會, 中華醫學會皮膚性病學分會治療學組, 中國醫療保健國際交流促進會皮膚醫學分會, 等. 中國天皰瘡診療指南(2024 版). 中華皮膚科雜志, 2024, 57(10): 873-886.
13. Moreno AC, Santi CG, Gabbi TV, et al. IgA pemphigus: case series with emphasis on therapeutic response. J Am Acad Dermatol, 2014, 70(1): 200-201.
14. 詹同英, 陳澤镕, 蘭小夢, 等. 多重自身免疫綜合征一例并文獻復習. 中國麻風皮膚病雜志, 2020, 36(2): 94-96.
15. Zhou Y, Xiao Y, Wang Y, et al. Refractory atypical IgA pemphigus successfully treated with apremilast. J Dermatol, 2024, 51(3): e86-e87.
16. Koga H, Tsutsumi M, Teye K, et al. Subcorneal pustular dermatosis-type IgA pemphigus associated with multiple myeloma: a case report and literature review. J Dermatol, 2023, 50(2): 234-238.
17. van Beek N, Holtsche MM, Atefi I, et al. State-of-the-art diagnosis of autoimmune blistering diseases. Front Immunol, 2024, 15: 1363032.
18. Li J, Feng X, Wang M, et al. Demographic characteristics and disease severity associated with IgA/IgG deposition patterns in autoimmune bullous diseases: a cohort study based on a registry database. Front Immunol, 2025, 16: 1565073.
19. 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, 1989, 20(1): 89-97.
20. Geng RSQ, Sood S, Bestavros S, et al. Treatment outcomes in IgA pemphigus: a systematic review. J Cutan Med Surg, 2025, 29(4): 420-421.

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