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.
