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Case Reports
. 2017 Aug 11:2017:bcr2017220887.
doi: 10.1136/bcr-2017-220887.

Paraneoplastic mucous membrane pemphigoid with ocular and laryngeal involvement

Silvia Lambiel  1 , Pavel Dulguerov  1 , Emmanuel Laffitte  2 , Igor Leuchter  1
Affiliations
Case Reports

Paraneoplastic mucous membrane pemphigoid with ocular and laryngeal involvement

Silvia Lambiel et al. BMJ Case Rep. .

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.

Keywords: dentistry and oral medicine; dermatology; ear, nose and throat/otolaryngology; oncology; ophthalmology.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Intra-oral view of a right sublingual erosion covered with fibrin (black arrow) and a left healing ulcer (yellow arrow). Tongue frenulum (red arrow).
Figure 2
Figure 2
View of the oropharynx showing an ulcer with fibrin deposit of the right soft palate (yellow arrow); uvula (black arrow).
Figure 3
Figure 3
Laryngoscopy showing diffuse whitish pseudomembranes covering the epiglottis and both aryepiglottic folds and arytenoids.
Figure 4
Figure 4
Haematoxylin and eosin stain: subepithelial split on a perilesional skin biopsy.
Figure 5
Figure 5
Direct immunofluorescence of a bullous skin lesion. Linear IgG deposition at the basement membrane.
Figure 6
Figure 6
Direct immunofluorescence of a bullous skin lesion. Linear C3 deposition at the basement membrane.
Figure 7
Figure 7
Laryngoscopy showing interarytenoid synechia (arrow).
See this image and copyright information in PMC

References

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