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01.12.2018 | Letter to the Editor | Ausgabe 1/2018 Open Access

Orphanet Journal of Rare Diseases 1/2018

Systemic retinoids for treatment of recalcitrant IgA pemphigus

Orphanet Journal of Rare Diseases > Ausgabe 1/2018
Franziska Schauer, Johannes Steffen Kern, Dimitra Kiritsi
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Electronic supplementary material

The online version of this article (https://​doi.​org/​10.​1186/​s13023-018-0899-y) contains supplementary material, which is available to authorized users.


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.
Additional file 1: Figure S1. Haematoxylin-eosin stainings of patients’ biopsies, as well as direct immunofluorescence staining pictures with IgA for diagnostics are shown. Both the histological and the immunofluorescence findings are similar in the 3 cases. The histology shows spongiosis and intraepidermal blisters, as well as infiltrates of neutrophilic granulocytes (hematoxylin-eosin, original magnification × 100). Direct immunofluorescence microscopy revealed IgA deposits at the upper part of the epidermis (original magnification × 200). (TIF 548 kb)
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