[Terrien's disease, apropos of 6 cases. Ultrastructural study]

Terrien's disease is characterized by slowly progressive thinning of the pre-limbal cornea in middle-aged patients. It usually leads to peripheral ectasia with marked astigmatism. It is rarely accompagnied by inflammation and its cause is unknown. Very few histopathologic examinations have been published, in particular those using electron microscopy We present detailed results concerning 6 patients in whom perforating keratoplasty permitted histopathologic and electron microscopic examination. Clinical and histological features reveal marked degeneration of the peripheral corneal stroma, with lipid accumulation unaccompagnied by significant inflammatory cellular infiltration in each case. Of interest is the association in one patient with keratoconus. The generally accepted pathogenic theories are fully discussed. The wealth of electron microscopic data provides a complete review of stromal degenerative changes.