Laryngeal pemphigus without skin manifestations and review of the literature

Eur Arch Otorhinolaryngol. 2007 May;264(5):509-12. doi: 10.1007/s00405-006-0208-6. Epub 2006 Nov 17.

Abstract

Pemphigus is an uncommon chronic disease with dermatologic and mucosal manifestations. Primary laryngeal involvement without skin lesions is extremely rare. The present paper describes a 72-year old man who presented with a 2-month history of hoarseness, haemoptisis and dysphagia. Clinical examination revealed an erythematous oral mucosa without ulcerations. Indirect laryngoscopy revealed supraglottic ulcerations mainly in the laryngeal surface of the epiglottis and in the right arytenoid. The lesions had characteristic gray color membranes. The patient underwent microlaryngoscopy under general anesthesia and biopsies were taken for histology that revealed inflammatory and granular lesions with necrosis. The diagnosis of pemphigus was based on immunohistopathology and the clinical examination. The patient underwent intravenous treatment with high doses of corticosteroids (prezolon 75 mg/24 h) for 10 days and gradually the dose was reduced to 10 mg/24 h. The patient had a very good response to the treatment and after a week approximately 80% of the lesions disappeared. However, the dose of 10 mg prednisolone per day was sustained for 3 months because any attempt of prednisolone discontinuation was related with reappearance of the clinical symptoms. After 3 months, finally the treatment was discontinued without problems. Now, 15 months later, the patient is well and without symptoms. He is under long-term follow-up. ENT surgeons should be aware of pemphigus as primary laryngeal manifestation in order to investigate and manage patients accordingly.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Anti-Inflammatory Agents / therapeutic use
  • Drug Administration Schedule
  • Humans
  • Laryngeal Diseases / diagnosis*
  • Laryngeal Diseases / drug therapy
  • Laryngoscopy
  • Male
  • Pemphigus / diagnosis*
  • Pemphigus / drug therapy
  • Prednisolone / therapeutic use
  • Skin Physiological Phenomena*

Substances

  • Anti-Inflammatory Agents
  • Prednisolone