The underlying causes of cicatricial entropion may broadly be classified into progressive and nonprogressive cicatrizing diseases, all having a common end point, which is relentless and irreversible scarring of the posterior lid lamellae, and ultimately the ocular surface.⁹ Progressive causes include infectious conditions like trachoma or leprosy, autoimmune processes like mucous membrane pemphigoid, Stevens-Johnson syndrome (SJS), and rarely thyroid eye disease. Nonprogressive conditions include trauma, surgery, chemical injuries, severe blepharitis, or severe meibomian gland dysfunction.⁷

Trachoma usually starts with repeated childhood infections with Chlamydia trachomatis serovars A-C (see Chapter 121).⁶,¹¹ Recurrent episodes of infection eventually result in entropion and trichiasis many years later, and ultimately corneal opacification and blindness.⁶ Mucous membrane pemphigoid is a life-threatening and occasionally blinding autoimmune disorder,⁹,¹²,¹³,¹⁴,¹⁵,¹⁶,¹⁷,¹⁸,¹⁹ and if the clinical manifestations primarily involve the eyes, the term ocular cicatricial pemphigoid (OCP) is often used (see Chapter 98).¹⁴,¹⁵ SJS is a drug-induced acute blistering disease of the skin and at least two mucosal surfaces, which may be complicated with high mortality or significant long-term ocular morbidity (see Chapter 117).²⁰,²¹,²²,²³,²⁴,²⁵,²⁶,²⁷,²⁸,²⁹ It is important not to overlook other less frequent causes of cicatricial entropion like penetrating trauma, chemical injuries, prior surgical trauma, linear IgA disease, atopic keratoconjunctivitis, graft-versus-host disease, IgG4-related disease, and dysthyroid ophthalmopathy.³⁰,³¹

Patients are usually aware of their condition, and the main presenting complaint is “lashes rubbing against the eye.”⁶ Additionally, they may complain of general symptoms like foreign body sensation, tearing, pain, or photophobia, but in rare situations, they may be completely asymptomatic.⁶ Patients should specifically be asked about a history of self-epilation, which would mislead the surgeon toward underrating the severity of entropion. They should also be questioned about previous eyelid surgery, which could undermine forthcoming surgical efforts.⁶ An inquiry should also be made about a history of drug intake, penetrating trauma, or chemical injuries, or a history of orbital floor fracture repair. Cicatricial entropion may involve the upper or lower eyelids and may affect male or female patients at any age, but there are specific demographic patterns related to certain diseases. Palpebral deformities in trachoma predominantly involve the upper eyelid and are more common in females in endemic areas of the world. TT is virtually absent in the Western Hemisphere except among migrants.⁴,⁷ Lower eyelid TT is less common than its upper eyelid counterpart, but it is not as rare as was previously thought,⁶ occurring in
up to 11% of TT patients.² Cicatrizing disorders from cutaneous drug reactions, as with SJS, can occur at any age,²⁰ and OCP is a disease more common in the elderly.¹⁵,¹⁹

When evaluating patients with cicatricial entropion, it is not only the eyelid position or the number of lashes in contact with the cornea that should be examined, but the tarsal conjunctiva, the lacrimal system, and the cornea should be examined as well.⁶ The palpebral manifestations embody a full spectrum of disease ranging at one end from simple trichiasis, where no entropion is observed (see Chapter 22), to frank entropion, where the entire eyelid margin rolls inward and may potentially be blinding (Figure 19.1).⁴,⁷ The location of the eyelashes rubbing against the cornea may be a general indicator for the later development of corneal blindness, with more remote eyelashes away from the center posing a lesser grave danger (Figure 19.2).⁶ However, it is important to remember that the eye is not a static structure and as the globe moves, contact between the eyelashes and the cornea will repeatedly take place.⁴ There is also “rounding” or loss of the sharp boundary of the posterior eyelid margin in milder cases, but this posterior lid margin may completely disappear from view in more severe cases.²,⁶ The eyelids may appear structurally deformed with loss of normal architecture, which may be accompanied by conjunctivalization or keratinization of the mucocutaneous junction or lagophthalmos in severe or recurrent cases.²