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Paraneoplastic mucous membrane pemphigoid with ocular and laryngeal involvement

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Published in BMJ case reports. 2017, vol. 2017
Abstract A 73-year-old woman was treated 8 years previously for synchronous breast and uterine neoplasms. She presented with a severe sore throat, odynophagia, dysphonia, dyspnoea, ocular irritation and weight loss over the last 3 months. Physical examination revealed ulcerations in the oral cavity, posterior pharyngeal wall and supraglottic larynx, nasal crusting, bilateral conjunctivitis and three cutaneous blisters. A diagnosis of anti-laminin 5 mucous membrane pemphigoid was retained, based on skin biopsy, direct immunofluorescence and immunoprecipitation. A positron emission tomography (PET)-CT detected multiple adenopathies. Cytology revealed adenocarcinoma with an immunocytology compatible with a breast origin and this was considered as a late metastatic recurrence of her previous breast cancer. A treatment of prednisone, dapsone and hormonotherapy was introduced, but intravenous immunoglobulin and rituximab were added due to new mucosal lesions. Despite treatment, a posterior laryngeal scar and bilateral symblepharon were developed. After 3 years, the patient is still alive and reports a satisfactory quality of life.
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PMID: 28801511
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Article (Published version) (1.6 MB) - public document Free access
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Research group Groupe Dulguerov Pavel (ORL et chirurgie cervico-faciale) (145)
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LAMBIEL, Silvia et al. Paraneoplastic mucous membrane pemphigoid with ocular and laryngeal involvement. In: BMJ Case Reports, 2017, vol. 2017. doi: 10.1136/bcr-2017-220887 https://archive-ouverte.unige.ch/unige:99636

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Deposited on : 2017-11-24

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