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The objective of the current study was to characterize the epidemiology and resource use of U.S. children hospitalized with ophthalmologic disease secondary to erythema multiforme (EM), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). We studied children ages 5 to 19 years hospitalized in 2005 in 11 states, encompassing 38% of the U.S. pediatric population. Using International Classification of Diseases, Ninth Revision, Clinical Modification codes, we identified admissions of children with EM, SJS, or TEN and the presence of concurrent ophthalmologic disease, analyzed patient and hospitalization characteristics, and generated age- and sex-adjusted national estimates. We identified 460 children admitted with EM, SJS, or TEN, corresponding to 1,229 U.S. hospitalizations in 2005. Of the children with EM, SJS, or TEN, 60 (13.0%) had ophthalmologic disease, primarily (90.0%) disorders of the conjunctiva. Children with the highest proportions of ophthalmologic disease included those with mycoplasma pneumonia (26.7%), herpes simplex virus (15.6%), upper respiratory infection (13.9%), and lower respiratory infection (13.7%). Individuals with EM, SJS, or TEN and ophthalmologic disease were more likely than those without ophthalmologic disease to receive intensive care unit care (28.3% vs 17.0%, p = 0.03) and to be admitted to a children's hospital (63.3% vs 48.8%, p = 0.03). Ophthalmologic disease was also associated with a significantly longer median length of stay (6.0 days, interquartile range [IQR] 3-9 days vs 3.0 days, IQR 2-6 days, p < 0.001) and median hospital cost ($7,868, IQR $3,539-$17,440 vs $2,969, IQR $1,603-$8,656, p < 0.001). In children with EM, SJS, or TEN, ophthalmologic disease was most common in those with concurrent Mycoplasma pneumoniae and herpes simplex virus infections. Ophthalmologic disease was associated with considerably higher inpatient resource use in this population. Children with EM, SJS, or TEN should be screened and treated early for ophthalmologic disease to prevent morbidity and minimize long-term sequellae., (© 2013 Wiley Periodicals, Inc.)

Purpose: To study the acute ocular/cutaneous manifestations, causes, and management of the erythema multiforme (EM)/Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) disease spectrum., Methods: We retrospectively reviewed the medical records of all EM/SJS/TEN patients hospitalized at National Cheng Kung University Hospital in Taiwan between 1988 and 2004. Demographic data, medical/medication histories, ocular/mucocutaneous manifestations, management, sequelae, and recurrence were analyzed., Results: A total of 207 patients 2 months to 95 years of age were hospitalized with 213 episodes/attacks of EM/SJS/TEN. Medications were the most common cause of any condition: for SJS, carbamazepine was most common; for EM or TEN, allopurinol was most common. In 128 of the 213 attacks (60.1%; 126 patients), ocular manifestations were documented during hospitalization, occurring more often in those with SJS (81.3%) or TEN (66.7%) compared with those with EM (22.7%; P < 0.01). The most frequent ocular treatments were topical steroids, topical antibiotics, and lubricants. Overall, 24 (18.8%) of 128 acute attacks in 126 patients were followed by ocular sequelae, mostly dry eye. Five (2.4%) of the 207 patients sustained a total of 6 recurrent attacks, in 3 cases because of the same medication., Conclusions: Ocular manifestations occur in a high proportion of patients with EM/SJS/TEN. The most frequent causes were carbamazepine and allopurinol. A careful medication history should be obtained from these patients. Ophthalmic consultation, evaluation, and management are mandatory.

Purpose: To evaluate the epidemiology, possible etiologic factors, complications encountered, and treatment administered to a group of patients with ocular involvement in the erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum who were seen at two large tertiary referral centers over a 34-year period., Methods: Hospital records from 1960 to 1994 at the Massachusetts General Hospital and Shriners Hospital for Crippled Children were reviewed for patients with erythema multiforme, Stevens-Johnson syndrome, or toxic epidermal necrolysis. Only patients fulfilling specific clinical diagnostic criteria and those who received a diagnosis by a dermatologist were included in the review., Results: A total of 366 patients with erythema multiforme, Stevens-Johnson syndrome, or toxic epidermal necrolysis were identified. Drugs were the most commonly identified etiologic factor in all three conditions: sulfonamides were the most frequently identified agents. Eighty-nine patients (24%) had ocular manifestations at the time of their acute hospital stay. Ocular involvement was seen in 9% of patients with erythema multiforme, in 69% with Stevens-Johnson syndrome, and in 50% with toxic epidermal necrolysis. The ocular problems were more severe in patients with both Stevens-Johnson syndrome and toxic epidermal necrolysis. There was no significant difference between the number of patients who were treated with systemic steroids and those who were not (P = 0.42)., Conclusions: The erythema multiforme/Stevens-Johnson syndrome/toxic epidermal necrolysis disease spectrum remains an important cause of severe visual loss in a significant number of patients. Systemic steroids used during the acute phase of the disease appear to have no effect on the development of ocular manifestations. Studies on the acute immunopathogenic mechanisms occurring in these disease are warranted if more effective therapies are to be found.

Although they were once believed to be distinct diseases, many observers currently consider erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis to represent a mild-to-severe continuum of the same process. Each shares certain clinical, histologic, and etiologic characteristics; among the latter two are frequent ocular involvement. Because long-term ophthalmic sequelae may be catastrophic, early recognition and therapy of this acute oculocutaneous disease spectrum are critically important.

Cicatricial pemphigoid (CP) and erythema multiforme (EM) are bullous diseases that involve the skin and mucous membranes including the conjunctiva. Of all the bullous diseases, CP and EM not only involve the conjunctiva most frequently but also cause the most severe conjunctival disease. A chronic, progressive disease, CP is characterized by shrinkage of the conjunctiva, symblepharon, entropion, trichiasis, dry eye, and finally reduced vision from corneal opacification. It is primarily a disease of the elderly that affects more women than men and is characterized by blisters or bullae in a subepithelial location and immunoglobulins and complement bound to the basement membrane zone of skin and mucous membranes including the conjunctiva. Circulating antibodies to the basement membrane zone can be demonstrated occasionally. Treatment includes artificial tears, topical antibiotics, correction of entropion and trichiasis, therapeutic soft contact lenses, and systemic immunosuppressive therapy including corticosteroids. An acute, generally self-limited, inflammatory disorder of the skin and mucous membranes, EM occurs primarily in young, healthy individuals. The most frequent precipitating factors are (1) drugs and (2) infections caused by Mycoplasma pneumoniae and herpes simplex. Conjunctival involvement ranges from a mild catarrhal conjunctivitis which terminates without sequelae to membranous conjunctivitis which may heal leaving scarring, symblepharon, and even ankyloblepharon. Histopathologic findings include subepithelial bullae and perivascular mononuclear cell infiltrates. Patients with EM have circulating immune complexes and immunoreactant deposition in the blood vessel walls of the dermis. After the acute episode has subsided, they may require artificial tears, topical antibiotics, correction of entropion and trichiasis, therapeutic soft contact lenses, tarsorrhaphy, and mucous membrane grafts.

Acute ocular complications of erythema multiforme such as infection causing conjunctivitis, suppurative keratitis or endophthalmitis are well recognized and usually easily managed. Late complications include disorders to tear production and drainage, position of lids, abberant or metaplastic lashes and metaplasia of the conjunctiva. Early detection and active management of these abnormalities greatly improves the long term prognosis. The techniques employed and the indications for their use in a recent series of 34 patients are presented.