Systemic retinoids for treatment of recalcitrant IgA pemphigus

Franziska Schauer,
Johannes Steffen Kern and
Dimitra KiritsiEmail author View ORCID ID profile

Orphanet Journal of Rare Diseases201813:163

https://doi.org/10.1186/s13023-018-0899-y

Received: 28 February 2018
Accepted: 24 August 2018
Published: 18 September 2018

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.