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Paraneoplastic Mucous Membrane Pemphigoid with Ocular and Laryngeal Involvement Silvia Lambiel,1 Pavel Dulguerov,2 Emmanuel Laffitte,3 Igor Leuchter4

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Paraneoplastic Mucous Membrane Pemphigoid with Ocular and Laryngeal Involvement Silvia Lambiel,1 Pavel Dulguerov,2 Emmanuel Laffitte,3 Igor Leuchter4 BMJ Case Reports: first published as 10.1136/bcr-2017-220887 on 11 August 2017. Downloaded from Rare disease CASE REPORT Paraneoplastic mucous membrane pemphigoid with ocular and laryngeal involvement Silvia Lambiel,1 Pavel Dulguerov,2 Emmanuel Laffitte,3 Igor Leuchter4 1Département des SUMMARY as the challenging multidisciplinary management of Neurosciences Cliniques, A 73-year-old woman was treated 8 years previously paraneoplastic MMP. Hôpitaux Universitaires de for synchronous breast and uterine neoplasms. She Genève, Geneva, Switzerland 2 presented with a severe sore throat, odynophagia, CASE PRESENTATION Département des Neurosciences Cliniques, dysphonia, dyspnoea, ocular irritation and weight A 73-year-old Caucasian woman presented to the Hôpitaux Universitaires de loss over the last 3 months. Physical examination ear–nose–throat clinic with a diffuse oral burning Genève, Geneva, Switzerland revealed ulcerations in the oral cavity, posterior sensation and disabling pharyngodynia over the last 3Département des Spécialités pharyngeal wall and supraglottic larynx, nasal crusting, 3 months. She reported weight loss with asthenia, de Médecine, Hôpitaux bilateral conjunctivitis and three cutaneous blisters. cough when lying down, hoarseness and dyspnoea. Universitaires de Genève, A diagnosis of anti-laminin 5 mucous membrane Previous treatments included topical and systemic Geneva, Switzerland pemphigoid was retained, based on skin biopsy, direct antifungal agents because of Candida-positive 4Département des immunofluorescence and immunoprecipitation. A oral swabs, antibiotics and antiviral therapy. Two Neurosciences Cliniques, positron emission tomography (PET)-CT detected multiple Hôpitaux Universitaires de previous oral biopsies revealed fibrin deposits with Genève, Geneva, Switzerland adenopathies. Cytology revealed adenocarcinoma with leucocytes without malignant cells. She had been an immunocytology compatible with a breast origin and treated during the previous month for bilateral Correspondence to this was considered as a late metastatic recurrence of ocular irritation and nail inflammation of the right Silvia Lambiel, her previous breast cancer. A treatment of prednisone, index finger by topical antibiotic drops and partial silvialambiel@ gmail. com dapsone and hormonotherapy was introduced, but nail avulsion, respectively. intravenous immunoglobulin and rituximab were added Her past medical history was marked by two Accepted 4 August 2017 due to new mucosal lesions. Despite treatment, a simultaneous cancers: a breast carcinoma and an posterior laryngeal scar and bilateral symblepharon were endometrial adenocarcinoma. These had been developed. After 3 years, the patient is still alive and treated successfully 8 years previously by right reports a satisfactory quality of life. mastectomy, axillary dissection, adjuvant radio- therapy and hormonotherapy, and by adnexectomy http://casereports.bmj.com/ and hysterectomy, respectively. At the last gynae- BACKGROUND cological examination, there was no sign of recur- Mucous membrane pemphigoid (MMP), also rence. referred to as cicatricial pemphigoid, is a rare Clinical examination revealed bilateral conjunc- bullous disease affecting predominantly mucosal tival inflammation and three blister-type skin sites and occasionally the skin in about 25% of lesions on the arms and legs. Inspection of the oral cases.1 Unfortunately, most specialists in oral cavity showed mucosal ulcerations of the ventral pathology or otolaryngology are unfamiliar with tongue (figure 1), cheeks, soft palate (figure 2), this disease. The most commonly affected sites uvula and posterior oropharyngeal wall. A flex- include the oral cavity and eye, followed by the ible transnasal endoscopy revealed crusts on the on 28 September 2021 by guest. Protected copyright. nasal, anogenital and pharyngeal mucosa and, right middle turbinate and nasopharynx, as well as less frequently, the oesophagus and larynx.2 The diffuse whitish pseudomembrane-covered erosions protean phenotypical manifestations are related to of almost all the supraglottic area and posterior the sites involved, age of the lesions and severity of hypopharyngeal wall (figure 3). the disease. The natural course of untreated lesions is irreversible scarring, which can result in airway INVESTIGATIONS obstruction and blindness in the case of laryngeal Full blood tests were unremarkable. A perilesional and ocular involvement, respectively. Some studies skin biopsy of a bullous lesion on the left leg revealed have evoked the possible association between MMP subepithelial cleavage with a rare eosinophilic infil- and malignant neoplasms when autoantibodies to trate on routine histopathological haematoxylin laminin 5 are present,3–7 arguing that anti-laminin 5 and eosin staining (figure 4). Direct immunofluo- MMP could be a paraneoplastic manifestation, but rescence analysis revealed a linear deposit of IgG T Lambiel S,o cite: this notion remains controversial.8 and C3 at the dermo-epidermal junction (figures 5 Dulguerov P, Laffitte E, et al. Herein, we report a rare case of MMP where and 6). Indirect immunofluorescence analysis was BMJ Case Rep Published negative. Immunoblotting studies demonstrated Online First: [please several mucosal sites were involved, leading to include Day Month Year]. cicatricial lesions despite adequate treatment. The circulating autoantibodies to bullous pemphigoid doi:10.1136/bcr-2017- association between anti-laminin 5 autoantibodies (BP)180 and laminin 5 (ELISA-BP 180 +: 26.99 U/ 220887 and metastatic breast carcinoma is discussed, as well mL; ELISA-BP 230: −). Lambiel S, et al. BMJ Case Rep 2017. doi:10.1136/bcr-2017-220887 1 BMJ Case Reports: first published as 10.1136/bcr-2017-220887 on 11 August 2017. Downloaded from Rare disease Figure 1 Intra-oral view of a right sublingual erosion covered with Figure 3 Laryngoscopy showing diffuse whitish pseudomembranes fibrin (black arrow) and a left healing ulcer (yellow arrow). Tongue covering the epiglottis and both aryepiglottic folds and arytenoids. frenulum (red arrow). between these entities relies on clinical criteria. Pemphigus A diagnosis of MMP on clinical and immunohistological disease is distinguished from MMP by clinically flaccid blisters criteria was made, including diffuse oral, ocular, nasal, cuta- and suprabasal acantholysis on histopathology. neous, pharyngeal and laryngeal involvement. Given the past In oral lichen planus, fibrinogen deposition is encountered in oncological history and the presence of anti-laminin 5 antibodies, the basement membrane zone, but neither IgG, IgA nor C3 is a PET-CT scan was performed and revealed a left supraclavicular present. and multiple retroperitoneal adenopathies. Ultrasound-guided and CT-guided fine-needle biopsies were compatible with meta- TREATMENT static adenocarcinoma of breast origin, were hormone depen- The patient was treated initially with intravenous dexametha- dent and were considered as a late metastatic recurrence of the sone 4 mg three times daily for 3 days, followed by oral pred- previous breast tumour, without locoregional relapse. nisone (30 mg/day) in conjunction with dapsone at increasing doses: 50 mg/day during the first week; 75 mg/day during the DIFFERENTIAL DIAGNOSIS second week; and 100 mg/day thereafter. Hormonal therapy Diagnosis of MMP is based on clinical features, routine histo- with letrozole 2.5 mg/day was also started. A nasogastric feeding pathological study (subepithelial split), immunohistological tube was initially necessary, but a rapid clinical improvement http://casereports.bmj.com/ examination (linear deposition of IgG, IgA or C3 along the base- in the pharynx and larynx allowed the patient to resume oral ment membrane zone on direct immunofluorescence) and serum intake. antibody analysis (indirect immunofluorescence; detection of autoantigens including BP antigen 1 (BPAg1 or BP230), BP OUTCOME AND FOLLOW-UP antigen 2 (BPAg2 or BP180), integrin subunits alpha-6/beta-4, The active oral lesions completely disappeared initially. laminin 5, laminin 6 and type VII collagen by immunoblotting However, after 2 months, a transnasal endoscopy revealed an and immunoprecipitation techniques).1 Although BP has similar direct immunofluorescence features, the skin is predominantly affected and the differential diagnosis on 28 September 2021 by guest. Protected copyright. F igure 2 View of the oropharynx showing an ulcer with fibrin deposit Figure 4 Haematoxylin and eosin stain: subepithelial split on a of the right soft palate (yellow arrow); uvula (black arrow). perilesional skin biopsy. 2 Lambiel S, et al. BMJ Case Rep 2017. doi:10.1136/bcr-2017-220887 BMJ Case Reports: first published as 10.1136/bcr-2017-220887 on 11 August 2017. Downloaded from Rare disease Figure 5 Direct immunofluorescence of a bullous skin lesion. Linear Figure 7 Laryngoscopy showing interarytenoid synechia (arrow). IgG deposition at the basement membrane. DISCUSSION interarytenoid synechia with a somewhat decreased laryngeal MMP is a rare autoimmune subepithelial bullous disease char- patency (figure 7), while the ophthalmic examination showed acterised by the linear deposition of IgG, IgA or C3 along the bilateral moderate symblepharon without active inflammation. epithelial basement membrane zone and affecting predominantly Due to a mild haemolysis and general ill feeling when taking mucous membranes.2 The true incidence remains unknown, but dapsone, intravenous immunoglobulin was introduced (every it has been estimated
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