title: Pemphigus: An Ophthalmological Review.
abstract: OBJECTIVE
  To describe the epidemiology, etiology, pathogenesis, clinical characteristics, and management of pemphigus, with an emphasis on ocular involvement.
  
  
  METHODS
  Literature review.
  
  
  RESULTS
  Pemphigus is an autoimmune epithelial blistering disease of the skin and mucous membranes. The typical pathological finding is acantholysis of the epidermis that leads to blister formation. Immunofluorescence techniques show autoantibody deposition on the epidermal intercellular substance. Although a genetic background is necessary, environmental factors are crucial for the onset and perpetuation of the disease. Exposure to some drugs, toxic agents, and foods and associations with other autoimmune diseases and lymphoproliferative conditions should be assessed. Generally, the skin is the most commonly affected tissue. Ocular involvement might be present and exhibit a clinical course that is independent of skin compromise. Visual function may be affected depending on the severity of the presentation. In untreated cases, mortality is high because of bacterial sepsis and hydroelectrolyte imbalance. A multidisciplinary approach should be used involving a dermatologist, ophthalmologist, and immunologist. Immunosuppressive agents are the mainstay of treatment; corticosteroids typically with azathioprine or mycophenolate mofetil are the drugs of choice. Surgical treatment of trichiasis and malposition of the eyelids and tectonic procedures for corneal perforation are sometimes required in very severe and recalcitrant cases.
  
  
  CONCLUSIONS
  Pemphigus is a potential life- and sight-threatening disease. Understanding the disease facilitates the adequate assessment of the modifiable factors and the prompt initiation of immunotherapy. Ocular involvement can develop in patients with pemphigus. Adequate ophthalmological care is needed, in particular, prevention of infections, scarring, and corneal perforation.
authors.affiliation: Cornea and Refractive Surgery Department (P.C.-S., A.N., A.R.-M., E.O.G.-H.), Institute of Ophthalmology "Fundación, de Asistencia Privada Conde de Valenciana," Mexico City, Mexico; and Immunology Lab (M.C. J.-M.), Department of Biochemistry, Faculty of Medicine National, Autonomous University of Mexico, Mexico City, Mexico.
authors.forename: Patricia
authors.initials: P
authors.lastname: Chirinos-Saldaña
authors.forename: Alejandro
authors.initials: A
authors.lastname: Navas
authors.forename: Arturo
authors.initials: A
authors.lastname: Ramírez-Miranda
authors.forename: María Carmen
authors.initials: MC
authors.lastname: Jiménez-Martínez
authors.forename: Enrique O
authors.initials: EO
authors.lastname: Graue-Hernández
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D003229:Conjunctival Diseases / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D005141:Eyelid Diseases / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010392:Pemphigus / Q000150:complications* / Q000473:pathology / Q000628:therapy; D012307:Risk Factors; D014786:Vision Disorders / Q000209:etiology / Q000628:therapy
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents