Systemic retinoids for treatment of recalcitrant IgA pemphigus

Orphanet Journal of Rare Diseases

Dimitra Kiritsi¹  Johannes Steffen Kern¹  Franziska Schauer¹ 

[1] Department of Dermatology, Medical Center- University of Freiburg, Faculty of Medicine, University of Freiburg;

关键词: Autoimmune skin blistering;    Alitretoin;    Acitretin;    Desmosomes;    Desmocollin;   

DOI  :  10.1186/s13023-018-0899-y

来源: DOAJ

【 摘 要 】

Abstract IgA pemphigus is an exceedingly rare autoimmune blistering disorder, caused by IgA autoantibodies against desmosomal proteins. No treatment option has been found to be universally effective. The disease is often recalcitrant to oral steroids and immunosuppressants. Here, we describe the use of systemic retinoids for the treatment of recalcitrant IgA pemphigus in 3 cases. Although the use of acitretin has been reported before, we present for the first time the positive effects of alitretinoin in treatment of 2 patients with IgA pemphigus. Besides hyperlipoproteinaemia requiring use of hypolipidemic agents in one case, alitretinoin was well-tolerated and has generally a more favorable side effect spectrum than immunosuppressants.

【 授权许可】

Unknown