[Chronic keratitis and autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome - a case report].

Wannke E, Wannke B, Zierhut M

Klinische Monatsblatter fur Augenheilkunde

Dec 1, 2001

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Abstract

BACKGROUND: The autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome (APECED) is a rare disease which can be associated with severe keratitis leading to blindness. Besides conjunctivitis and keratitis uveitis anterior, cataract, retinitis pigmentosa, atrophy of the optical nerve, loss of lashes and eye-brows have been described. CASE REPORT: We report on an 11-year-old girl with the history of APECED syndrom. For 6.5 years she has suffered from chronic keratoconjunctivitis including corneal infiltrates, peripheral epithelial and subepithelial scarring and peripheral neovascularisation of the cornea. Most of all she complained of recurrent conjunctivitis, photophobia and epiphora. The best therapy consisted in local application of dexamethasone and gentamycine three times daily. Under this therapy the corneal infiltration remained stable and the patient felt most comfortable. No side effects appeared by now. CONCLUSIONS: Inspite of the bad prognosis of the keratitis we managed to keep the cornea infiltrates stable. Nevertheless the search for a more effective treatment with less side effects like secondary glaucoma or cataracts will go on.