Treatment of pemphigus vulgaris with protein A immunoadsorption
ZHANG Chenxi
SHEN Jing
ZHANG Xiaoying
GUO Liheng
CHEN Jialong
WANG Qi
ZENG Kang
LI Changxing
Abstract:A 25-year-old woman presented with a four-year history of widespread erythema,blisters,and erosions,accompanied by pain that had worsened over the past week.Four years ago,she was diagnosed with pemphigus vulgaris(PV)based on multiple oral ulcers,widespread erythema,and thin-walled blisters.Despite treatment with corticosteroids,immunosuppressants,and biologics such as rituximab,her condition remained poorly controlled with recurrent flares.A general examination revealed cushingoid features,a buffalo hump,and a Body Mass Index of 28.08.The dermatological examination showed extensive erythema,flaccid bullae,erosions,and crusting on the scalp,trunk,and extremities,with profuse serous exudate.Erythematous bullous,verrucous plaques were also noted in the axillary and genital areas.The Pemphigus Disease Area Index score was 49.Laboratory tests revealed anti-Dsg1 antibodies at 237.8 U/mL and anti-Dsg3 antibodies at 174.5 U/mL.Skin secretion cultures grew multiple pathogenic organisms.The final diagnoses were pemphigus vulgaris,cutaneous infection,and drug-induced Cushing's syndrome,and others.Following multidisciplinary consultation and informed consent,the patient received corticosteroids,methotrexate,and protein A immunoadsorption(PA-IA)therapy for a total of 10 sessions.After discharge,her autoantibody titers decreased,and skin lesions improved significant-ly.During a 6-month follow-up period,the patient remained lesion-free,with a sustained decline in pemphigus-related autoantibody titers.
Keywords:pemphigus vulgarissevere pemphigusimmunoadsorption therapycutane-ous infection
Publication Date:2026-01-28
Online Publishing Date:2026-03-11(First online date of this platform, not the publication date of the document)
Pages:7( 35-41 )