Paraneoplastic mucous membrane pemphigoid with ocular and laryngeal involvement

BMJ Case Rep. 2017 Aug 11;2017:bcr2017220887. doi: 10.1136/bcr-2017-220887.


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.

Publication types

  • Case Reports

MeSH terms

  • Adenocarcinoma / drug therapy
  • Adenocarcinoma / secondary*
  • Aged
  • Antineoplastic Agents, Immunological / therapeutic use*
  • Breast Neoplasms / drug therapy
  • Breast Neoplasms / secondary*
  • Conjunctivitis / drug therapy
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use*
  • Laryngitis / drug therapy
  • Larynx / pathology*
  • Pemphigoid, Benign Mucous Membrane / diagnosis*
  • Pemphigoid, Benign Mucous Membrane / therapy
  • Positron Emission Tomography Computed Tomography
  • Rituximab / therapeutic use*
  • Treatment Outcome


  • Antineoplastic Agents, Immunological
  • Immunoglobulins, Intravenous
  • Rituximab