Therapeutic Options for IgA Pemphigus in Patients Refractory to Dapsone

ABSTRACT IgA pemphigus is a rare autoimmune blistering disorder with limited therapeutic guidance available due to its rarity and complex presentation. This comprehensive analysis evaluates the therapeutic outcomes of 67 published patients, emphasising the significant role of dapsone as the first‐line treatment. Among the IgA pemphigus cases analysed, 63% of patients achieved complete remission with dapsone monotherapy, further highlighting its overall efficacy and reliability. For patients with insufficient response to dapsone alternative therapies such as adalimumab consistently showed favourable outcomes, with most achieving complete remission. Of note, three patients with therapy‐refractory IgA pemphigus in the cohort were diagnosed with concurrent cancer, highlighting a screening for malignancies in refractory cases. Evaluating IgG/IgA pemphigus and IgA pemphigus with monoclonal gammopathy of undetermined significance (MGUS) or multiple myeloma (MM) as distinct subsets revealed differences in therapeutic responses, underlying pathologies and comorbidities in this group of patients. Thus, our analyses confirm that dapsone is a highly effective first‐line therapy in IgA pemphigus, however, emerging treatments to improve outcomes, especially in refractory and complex cases, should be recognised.