The aim of this review is to demonstrate the spectrum of conditions en
compassed by the term 'chronic progressive cicatrising conjunctivitis'
, to discuss mechanisms of conjunctival scar tissue formation and to d
escribe the sequelae and therapeutic options in this potentially blind
ing condition. Chronic progressive cicatrising conjunctivitis is found
in association with some mucocutaneous disorders (cicatricial pemphig
oid, linear IgA disease), as part of paraneoplastic syndromes and afte
r long-term treatment with certain systemic and topical medications (p
seudo-pemphigoid). Recent studies on the conjunctiva of pemphigoid pat
ients indicate that macrophages may play a pivotal role in chronic pro
gressive conjunctival cicatrisation. They mediate the transition from
inflammation to scar tissue by secretion of fibrogenic cytokines. Ther
e is evidence that similar mechanisms are involved in the other fibros
ing conjunctival disorders. Sequelae of chronic conjunctival cicatrisa
tion include the obstruction of lacrimal and meibomian glands, tear fi
lm alterations, trichiasis, keratopathy and blindness. Present possibi
lities and future options for the treatment of this condition are disc
ussed.