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Skin Blistering Diseases

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Front. Med. | doi: 10.3389/fmed.2018.00268

Mucous membrane pemphigoid, bullous pemphigoid and anti-programmed-death-1/death-ligand-1: a case report of mucous membrane pemphigoid and review of the literature

 Coralie ZUMELZU1, 2,  Marina ALEXANDRE1, 2, Christelle LEROUX1, 2, Patricia WEBER1, 2, Alexis GUYOT1, 2, Annie LEVY1, 2, Françoise AUCOUTURIER3, Sabine MIGNOT-GROOTENBOER2, 4, Frédéric CAUX2, Eve MAUBEC2 and  Catherine PROST SQUARCIONI1, 2, 5, 6*
  • 1Centre de référence des maladies bulleuses auto-immunes (MALIBUL), France
  • 2Centre de référence des maladies bulleuses auto-immunes (MALIBUL), France
  • 3Department of Immunology, Centre de référence Maladies rares bulleuses, Hôpital St Louis, France
  • 4Centre de référence des maladies bulleuses auto-immunes (MALIBUL), France
  • 5Department of Pathology, Hôpital Avicenne, France
  • 6Université Paris 13, France

An 83-year-old patient developed erosions and a blister of the gingival mucous membrane, 6 months after discontinuation of the anti-programmed death-1 (anti PD-1) pembrolizumab therapy administered for 10 months for a metastatic melanoma. A diagnosis of mild mucous membrane pemphigoid (MMP) was made. Complete remission of MMP was rapidly obtained with minimal therapy (doxycycline). MMP remained in complete remission after a 3-month follow-up since discontinuation of the doxycycline therapy and no evidence of relapse of the melanoma was observed after a 14-month follow-up since discontinuation of the pembrolizumab therapy.
The widespread use of anti PD-1 and anti-programmed death-ligand-1 (PD-L1) in several malignancies reveals new adverse events. MMP describes a group of chronic, inflammatory, mucous membrane-predominant, subepithelial auto-immmune blistering diseases. It shares similarities with bullous pemphigoid, another autoimmune blistering disease, but is clinically and immunologically distinct. Twenty-nine cases of bullous pemphigoid associated with anti PD-1/PD-L1 have been reported in the literature and one of MMP. Here, we described the case of a MMP developed after pembrolizumab and discussed the accountability of anti PD-1/PD-L1 in our case and the previous reported bullous pemphigoid and MMP cases using the Begaud system scoring.

Keywords: Mucous membrane pemphigoid, ; bullous pemphigoid, ; melanoma, ; anti-programmed-death-1/death-ligand-1, ; immune checkpoints inhibitors, ; pembrolizumab, ; drug accountability study, Adverse Drug Reaction

Received: 23 Jun 2018; Accepted: 03 Sep 2018.

Edited by:

Cristina Has, Albert-Ludwigs-Universität Freiburg, Germany

Reviewed by:

Marian Dmochowski, Poznan University of Medical Sciences, Poland
Hiroshi Koga, Department of Dermatology, Kurume University School of Medicine, Japan  

Copyright: © 2018 ZUMELZU, ALEXANDRE, LEROUX, WEBER, GUYOT, LEVY, AUCOUTURIER, MIGNOT-GROOTENBOER, CAUX, MAUBEC and PROST SQUARCIONI. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Prof. Catherine PROST SQUARCIONI, Centre de référence des maladies bulleuses auto-immunes (MALIBUL), Paris, France,