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First Reported Case of atypical Cogan's Syndrome in Central America.

Arch Soc Esp Oftalmol (Engl Ed)

December 2024

Departamento de Oftalmología, Hospital de la Familia, Nuevo Progreso, San Marcos, Guatemala. Electronic address:

Case Report: Case report of a 51 year old patient diagnosed with atypical Cogan's syndrome. The patient exhibited interstitial keratitis, anterior uveitis, and long-standing profound deafness. The treatment was based on topical and systemic steroids, resulting in a satisfactory evolution and currently in clinical remission.

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Background: Cogan syndrome is a rare autoimmune systemic vasculitis presenting with interstitial keratitis and audiovestibular symptoms. The atypical form, characterized by more extensive ocular lesions with audiovestibular symptoms appearing with a longer delay and more frequent systemic features, is usually underdiagnosed, delaying treatment.

Case Presentation: We report the case of a 30-year-old Mediterranean female who presented recurrent left red and painful eye.

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French protocol for diagnosis and management of Cogan's syndrome.

Rev Med Interne

October 2024

Service de médecine interne, hôpital Saint-Antoine, AP-HP, Sorbonne université, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France. Electronic address:

Article Synopsis
  • * It typically affects Caucasians equally across genders and can also be associated with other autoimmune diseases like polyarteritis nodosa.
  • * While ocular symptoms often have a good prognosis with potential recovery of vision, cochleo-vestibular issues can lead to serious and irreversible hearing loss, and treatment options are not well-established due to the condition's rarity.
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Article Synopsis
  • Primary atopic disorders (PAD) are rare genetic conditions caused by specific gene variants that affect skin and immune function, making diagnosis challenging among common allergic disease cases.
  • Identifying PAD requires recognizing clinical red flags like family history and unusual infections, as conventional lab tests are inadequate for definitive diagnosis.
  • Whole-genome sequencing (WGS) enhances diagnostic efficiency and accuracy, but requires careful interpretation and collaboration among specialists to effectively manage PAD cases.
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Cogan syndrome (CS) is a rare chronic inflammatory disease characterized by ocular and inner ear inflammation. Well-known ocular manifestations include non-syphilitic interstitial keratitis (IK); however, some cases are not associated with IK. Inner ear symptoms include sensorineural hearing loss, rotatory vertigo, and tinnitus, which can become irreversible without timely treatment.

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