By Edward Tenner M.D.
Hoffman Estates, Il.
Each type of pemphigus and relate diseases has differing kinds and differing percentages of ocular involvement. Also, the treatments for these diseases have many ocular side effects. Therefore it is very important that an Ophthalmologist examines the eyes of patients with pemphigus . This is especially true at the beginning of symptoms and treatments so any eye problem can be promptly handled.
Ocular involvement in pemphigus vulgaris is uncommon according to the medical literature. However, a group of patients have been described with eye irritation, excessive tearing, and foreign body sensation where the eye symptoms preceded the appearance of mouth and skin lesions.
Blisters can involve the skin surface of the eyelids. Since the skin around the eyes is very thin this area must be cared for gently, usually with warm compresses and a mild steroid cream. Blisters may also form on the eyelid margins, but are less common on the bulbar conjunctiva (covers the white part of the eye).
These rarely cause scarring. More common is a purulent or catarrhal (lots of mucus) conjunctivitis with some redness of the conjuntiva. Since this is usually not infectious only warm compresses are needed. Blepharitis (crusted matter along eyelid margins) can occur. Treatment entails warm compresses and eye lid scrubs. Dry eye irritation can be a problem which is treated with preservative-free artificial tears.
Eye involvement in pemphigus foliaceus is more common. The skin around the eyes and eyebrows is often affected. A quarter of all patients lose part or all of their eyebrows. Pemphigus foliaceus often affects the skin of the eyelid and eyelid margins. If severe the eyelid can be scarred causing entropion (the eyelid turns inward) and trichiasis (eyelashes grow in abnormal directions, can rub against eye). While blisters on the palpebral (inside of eyelid) or bulbar conjunctiva are rare, conjunctivitis with redness and mucous secretion is often noted. Blisters of the cornea (clear "windshield" front of eye) usually involve the middle or lower part of it. They can lead to inflammatory changes and pannus (scarring) formation of the cornea. The iris (colored part of eye) can be involved, with a degenerative reddish lesion on the surface of the iris. Cataract (cloudiness of the clear natural lens of the eye) is also commonly noted with variable size, shape, and color in the anterior lens cortex (soft outer part of lens.
Paraneoplastic pemphigus (a distinct type of pemphigus associated with an underlying cancer) commonly has eye involvement. A marked conjunctivitis with erosions was present in a majority of patients described. In one article this was treated with eyelid scrubs, antibiotic ointment, and steroid drops. Conjunctival biopsy has been used to help confirm this diagnosis.
Bullous pemphigoid is an autoimmune blistering disorder sharing some common features with pemphigus. Occasional involvement of the conjunctiva and eyelid margins with inflammation and scarring has been reported.
Cicatricial pemphigoid is a related disorder with common involvement of mouth and eyes. In the two thirds of cases when the eye is affected, it is also called ocular cicatrical pemphigoid. Both eyes are usually affected with a chronic conjunctivitis with thick stringy discharge, and burning and tearing. Fibrosis (scarring) beneath the conjunctival epithelium (surface cellular layer) is typical in this disease. As the scarring progresses symblepharons (fibrotic bridges between the conjunctiva of the eyelid and the eyeball) are characteristic. Also dry eye symptoms are created by destruction of tear component forming cells and tear duct openings. As the scarring gets worse the cornea is involved with new blood vessels growing in the previous clear tissue. Eventually the eyelids and eyeball fuse together (ankyloblepharon).
With the eye not able to close, the cornea becomes opaque and blindness ensued in almost one third of patients before there were effective treatments. Immunosuppressive drugs e.g. Cytoxan, Prednisone, and Dapsone are usually effective in controlling the disease along with aggressive use of lubricating drops and ointments. Any eye surgery must be undertaken with care because it can reactivate the disease.
As to side-effects from medications, Prednisone is the major source. Its two major eye side-effects being cataract and glaucoma. The cataracts are a special type called posterior subcapsular cataract. They refer to a cloudiness of the back part of the lens. They tend to grow slowly, usually after using Prednisone for along time at high levels.
If the Prednisone is stopped, the cataract remains stable, and as long as it is not decreasing vision, can be left alone. If the vision is impaired the cataract is removed surgically by a procedure called phacoemulsification where the cataract is sucked out of the eye. Then a plastic intraocular lens is put in its place. Glaucoma can occur with oral Prednisone or Prednisone drops. The people whose eye pressure increases are called steroid responders. The increased eye pressure usually develops within a few weeks to months. If the Prednisone has to be continued, treatment with glaucoma drops is necessary to prevent damage to the vision. Prednisone can bring out diabetic tendencies and diabetics need to be checked for eye complications. Also because Prednisone suppresses the immune system, people who have had herpes involving the eye can get recurrences and others may be more susceptible to new eye infection.
Fortunately, advances in medical care have helped to minimize the extent and impairment all of these problems can cause.