Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With Rituximab
Mucous membrane pemphigoid (MMP) is a rare autoimmune bullous disease of the mucous membranes, which can cause irreversible scarring and is discussed to be associated with cancer, if laminin-332-autoantibodies are present. MMP with severe ocular and laryngeal involvement is difficult to treat and ca...
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doaj-af394899ca914936b93f3c06f195da4e2020-11-24T21:17:01ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2019-01-01610.3389/fmed.2019.00008422661Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With RituximabMarcel WittenbergMargitta WormMucous membrane pemphigoid (MMP) is a rare autoimmune bullous disease of the mucous membranes, which can cause irreversible scarring and is discussed to be associated with cancer, if laminin-332-autoantibodies are present. MMP with severe ocular and laryngeal involvement is difficult to treat and can be treatment-refractory to conventional immunosuppressant therapy. A 67-year-old man with a history of prostate cancer presented to our clinic with sore throat, intraoral bullae, odynophagia, dysphonia, exertional dyspnea, and erosions of the glans penis. Clinical examination confirmed a laryngo-pharyngitis with involvement of the epiglottis and bilateral symblepharon. Diagnostics comprising multiple biopsies, direct and indirect immunofluorescence, serology analysis, and immunoblotting confirmed the diagnosis of a paraneoplastic MMP by showing a subepithelial split in histology and the presence of anti-laminin-332-antibodies. Despite combined systemic treatment with prednisolone and either dapsone or azathioprine, a progress of the disease occurred leading to severe ocular and laryngeal complications. Two month after rituximab treatment, complete disease control was achieved. This case report shows a severe ocular and life threatening laryngeal involvement of therapy-refractory paraneoplastic MMP highlighting the importance of interdisciplinary management and difficulty of diagnosing MMP despite repeated diagnostic testing.https://www.frontiersin.org/article/10.3389/fmed.2019.00008/fullmucous membrane pemphigoidrituxmabparaneoplasticlaminin 332therapy refractoriness |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Marcel Wittenberg Margitta Worm |
spellingShingle |
Marcel Wittenberg Margitta Worm Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With Rituximab Frontiers in Medicine mucous membrane pemphigoid rituxmab paraneoplastic laminin 332 therapy refractoriness |
author_facet |
Marcel Wittenberg Margitta Worm |
author_sort |
Marcel Wittenberg |
title |
Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With Rituximab |
title_short |
Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With Rituximab |
title_full |
Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With Rituximab |
title_fullStr |
Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With Rituximab |
title_full_unstemmed |
Severe Refractory Paraneoplastic Mucous Membrane Pemphigoid Successfully Treated With Rituximab |
title_sort |
severe refractory paraneoplastic mucous membrane pemphigoid successfully treated with rituximab |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Medicine |
issn |
2296-858X |
publishDate |
2019-01-01 |
description |
Mucous membrane pemphigoid (MMP) is a rare autoimmune bullous disease of the mucous membranes, which can cause irreversible scarring and is discussed to be associated with cancer, if laminin-332-autoantibodies are present. MMP with severe ocular and laryngeal involvement is difficult to treat and can be treatment-refractory to conventional immunosuppressant therapy. A 67-year-old man with a history of prostate cancer presented to our clinic with sore throat, intraoral bullae, odynophagia, dysphonia, exertional dyspnea, and erosions of the glans penis. Clinical examination confirmed a laryngo-pharyngitis with involvement of the epiglottis and bilateral symblepharon. Diagnostics comprising multiple biopsies, direct and indirect immunofluorescence, serology analysis, and immunoblotting confirmed the diagnosis of a paraneoplastic MMP by showing a subepithelial split in histology and the presence of anti-laminin-332-antibodies. Despite combined systemic treatment with prednisolone and either dapsone or azathioprine, a progress of the disease occurred leading to severe ocular and laryngeal complications. Two month after rituximab treatment, complete disease control was achieved. This case report shows a severe ocular and life threatening laryngeal involvement of therapy-refractory paraneoplastic MMP highlighting the importance of interdisciplinary management and difficulty of diagnosing MMP despite repeated diagnostic testing. |
topic |
mucous membrane pemphigoid rituxmab paraneoplastic laminin 332 therapy refractoriness |
url |
https://www.frontiersin.org/article/10.3389/fmed.2019.00008/full |
work_keys_str_mv |
AT marcelwittenberg severerefractoryparaneoplasticmucousmembranepemphigoidsuccessfullytreatedwithrituximab AT margittaworm severerefractoryparaneoplasticmucousmembranepemphigoidsuccessfullytreatedwithrituximab |
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