Stevens-Johnson Syndrome: Ocular Emergency

Loran B. Morgan, M.D.
Torrington, Wyoming

Diplomate, American Board of Ophthalmology

Chief Consultant in Ophthalmology
Veterans Administration Center, Hot Springs, South Dakota

ABSTRACT: Two cases of chronic Stevens-Johnson’s disease are presented: one treated by the older method of blepharoplasty, the other treated by rehydration of the cornea with the Morgan ® Therapeutic [medi-FLOW®] Lens and a vinyl headband.

One acute case is presented with complete recovery following treatment with the Morgan therapeutic Lens.

Stevens-Johnson Syndrome-Ocular Emergency

First of all we should have a definition of Stevens-Johnson disease. This is a term applied to what A. M. Stevens and S. C. Johnson is 1922 described as a “new” disease, “consisting of an eruptive fever with stomatitis and ophthalmia.” Although the term “Stevens-Johnson syndrome” has been widely accepted, it is unfortunate, in that the cases described are really severe erythema multiforme with ocular complications.

To obtain a clear understanding of what we are speaking about today, let us go back to the basic classification of: “KERATO-CONJUNCTIVITIS ASSOCIATED WITH DISEASES OF THE SKIN AND MUCOUS MEMBRANES.”(l) Under this general heading we have MUCO-CUTANEOUS ERUPTIONS.’ Histologically, these are divided into two groups. In one group the changes are specific and essentially epidermal, so that the bullae are formed within the epithelium layer by a process whereby the epithelial cells lose their coherrence with one another (acatholysis), while in the other the histology is non-specific, changes are essentially subepithelial, and bullae are formed with the stratum detaching the epithelial layer as a whole. This gives us a rather clear cut classification of:

A. True Pemphigus, which is a lethal disease characterized by acantholysis.

B. Pemphigoid Conditions, a group of diseases, benign with regard to life although not necessarily to vision, with a non-specific pathology affecting primarily the subepithelial tissues. Under this heading we include:

(1) Benign mucosal pemphigoid.

(2) Bullous dermatitis herpitiformis.

(3) Epidermolysis bullosa.

*(4) Erythema multiforme.

(5) Beiter’s disease.

The condition which I wish to speak about today is erythema multiforme, and specifically, the rather malignant type, which involves the conjunctiva, and has been labeled by many of us as “Stevens-Johnson syndrome”.

A word about the etiology before I get into the treatment. David B. Coursen (2) in an article in JAMA in October, 1966, brings up the possibility of Stevens-Johnson syndrome being a non-specific parasensitivity reaction. As you review both the dermatolical and ophthalmological literature, it becomes quite obvious that this approach is probably quite rational. Many of us have made rash statements that Stevens-Johnson syndrome is due to a drug reaction and as you will see in a moment, all of the cases I present today were labeled in this manner. The first was labeled as a reaction to sulfonainide following a kidney infection, the second was attributed to penicillin following an acute tonsillitis, and. the third due to phenobarbital. Probably a more logical explanation is that erythema multiforme is a disease which frequently starts with a nonspecific condition. This involves a typical upper respiratory tract infection, characterized by fever, malaise, cough, coryza, sore throat, chest pain, vomiting, muscular aches, and arthralgia in varying combinations of severity. As in many similar conditions a sulfonainide product or an antibiotic is given in this pro-dromal stage and the disease, probably coincidentally, goes on to the more serious complication of mucous membrane involvement, which we have loosely termed Stevens-Johnson syndrome. From the multiplicity of drugs and various other agents, including deep x-ray therapy, vaccination, neoplasms, Collegen Diseases, deodorants, cosmetics, various food, vitamins, pregnancy, contact dermatitis, and even poison ivy, it makes it rather obvious that there are too many illogical agents to justify these conclusions. In fact, the literature contains over one hundred and fifty reported cases in which no drugs were given and these have been as severe and there have been equal numbers of fatalities in this group.

With this introduction I would like to proceed to the presentation of three cases, two chronic and one acute.

The first patient is a twenty-five year old female who has symblepharon of both eyes and near ankyloblepharon since being treated with heavy doses of sulfonamide for a urinary infection in 1947. She was admitted to my hospital in November of 1960, thirteen years later, for a mucous membrane graft to the right eye. On the 7th of November, 1960, blepharoplasty was done with a mucous membrane graft from the lower lip. The graft was from the six o’clock position, three fourths of the circumference of the globe of the right eye. An acrylic ring, with an opening for the cornea, which I call a “symblepharon lens”, was left in place.

She was admitted to the hospital again in March of 1961, for dissection of the graft from the cornea, from the nine to the three o’clock position. There had been an over growth of the graft so it was freed from the cornea and cauterized. Recovery was again uneventful.

The process was done again in April of 1963, for the same problem. The pseudo-pterygium of the right eye was encroaching upon the cornea, causing a marked astigmatism.

It is an interesting note that during the surgeries the patient had to be left on the bedpan because she has the same type of adhesions in her urinary bladder with a bladder capacity of about 60 cc’s. In August of 1964, release of symblepharon on the left eye was done with a symblepharon acrylic lens left in place. Recovery was also uneventful. In January of 1967 the pseudo-pterygium of the right eye was re-operated, since there had been an increase in growth of the previous graft.

Since this time the patient has done exceptionally well with the wearing of a scleral lens in the right eye and a spectacle correction in the left eye.

The second patient is an eleven year old girl who was first seen by me four years after a severe generalized rash and ocular involvement with bullae and purulent conjunctivitis, following an injection of penicillin for tonsillitis. There were dense corneal scars in both eyes and the Shirmer test revealed a complete absence of tears in the right eye and 2 mm of Shirmer test in the left eye. There was severe symblepharon on the right eye and the best vision was counting fingers at one foot in both eyes. The patient had been under intensive care throughout California, including Serum Albumen drops for one year. When first seen by me the patient was in extreme discomfort and had been so for four years. The father said she spent the last four years sitting in the dark with her eyes closed.

She was admitted to my hospital where the Morgan Therapeutic Lens was used in an attempt to hydrate the corneas. A hypodermoclysis set was used with a Morgan Therapeutic Lens in each eye. Lactated Ringer’s solution was started at l00 ml/hr. A Holter pump was used for this procedure in the hope that some definite information concerning dosage could be obtained. Within two days the patient was able to recognize pictures of automobiles, cigarettes, money and so forth.

The Morgan Therapeutic Lens dosage was then cut to about 20ml/hr and continued for a total of nine days. At the end of this time the patient was using coloring books and watching television. She was dilated with atropine since vision improved as she could see around the corneal opacities. She was fitted with +5.00 spherical lenses which gave her good near vision. The Morgan Therapeutic Lenses were discontinued and she was placed on lactated Ringer’s drops, two in each eye, every hour, day and night. Upon dismissal from the hospital she was instructed to put a drop of lactated Ringer’s in each eye every hour, during waking hours, and gradually reduced at night to every three hours.

She is now wearing a vinyl headband at bedtime which contains lactated Ringer’s and gives a continuous medication at night. She has actively returned to school and is living a normal life.

The third case is an eight year old boy who was placed on phenobarbital for a single convulsive episode. On the 18th of October, 1971, he was admitted to the hospital acutely ill with a temperature of 104 degrees. In two days the diagnosis of erythema multiforme was established with a diffuse hemorrhagic erythrodema with numerous bullous lesions and severe stomatitis and pharyngitis. The conjunctiva of both eyes was injected.

On the 25th of October the infection of both eyes was worse and two days later symblepharon formation started. Within a few hours these were extensive and had to be broken three times a day. The Morgan Therapeutic Lens was started at 6:00 a.m. on the next day with sheets of symblepharon. By 7:00 p.m. the same evening all symblepharon were gone and the patient continued to improve. The right eye was treated four days and the left five.

The patient has made a complete recovery and has remained so to this date (January, 1972).

A comparison of these three cases indicates that prompt treatment of the acute case with the Morgan Therapeutic Lens can prevent the complications of symblepharon, keratoconjunctivitis sicca, and so forth, thus saving the patient the life of an optic cripple.

Stevens-Johnson syndrome is a self-limited disease, and if hydration and separation of the inflammed surfaces can be maintained during this period, ocular complications can be eliminated.

REFERENCES

1. Duke-Elder, Stewart: System of Ophthalmology, St. Louis, Mosby, 1965, Vol. 8, Part 1, pp. 496-522.
2. Coursin, David B.: Stevens-Johnson Syndrome: Non-specific Parasensitivity Reaction? JAMA, 198, No. 2, 113-116, October 10, 1966.
3. Morgan, L. B.: JAMA, February 16, 1970, vol.211, p. 1089.
4. Morgan, L. B.: Plastic Scleral Lens Helps to End Pain in Injured Eyes and Promotes Healing, vol.214, No. 5, JAMA, pp. 835-814, November 2, 1970.
5. Morgan, L. B.: A New Therapeutic Scleral Lens, Vol. 68, No. 7, Rocky Mountain Medical Journal, pp. 26-28, July, 1971.
6. Morgan L. B.: A New Drug Delivery System for the Eye, vol. 40, No. 6, Industrial Medicine and Surgery, pp. 11-13, September, 1971.