Corneal pathology and outcome of keratoplasty in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED).
Disabling, chronic, persistent keratoconjunctivitis is an essential ocular manifestation in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED). Because of the paucity of previous studies our aim is to describe the main histopathological features of the keratopathy and to report the long-term outcome of corneal grafting. Four corneal buttons obtained at keratoplasties. Two patients with clinical follow-up data of 30 years. The corneal epithelium showed a severe atrophy with even areas of incipient epidermalization. The Bowman's membrane was destroyed. The anterior corneal stroma was replaced by vascularized scar tissue with areas of chronic inflammatory cell infiltration consisting mainly of lymphocytes and plasma cells. The posterior corneal stroma, the Descemet's membrane and the endothelium were normal. Rejection occurred after each keratoplasty. The last visual acuity of the first patient was finger counting of the right eye and 0.3 of the left eye and of the second patient 0.08 of both eyes. In chronic keratopathy of APECED the anterior corneal layers, the epithelium, the Bowman's membrane and the anterior corneal stroma are affected while the posterior cornea appears normal. As after keratoplasty rejection may be expected, its prevention and management need intensive attention.