Genitourinary
Genitourinary sequelae in SJS/TEN include adhesions, particularly in the female urethra and vaginal opening; vaginal adenosis; vulvovaginal endometriosis; and persistent genital ulcerations most commonly reported in females.22 Prompt inpatient gynecologic or urologic consultation is critical to reduce these potentially permanent outcomes. Topical corticosteroid therapy is recommended in the acute phase.22
Psychologic
Posttraumatic stress disorder may occur in patients with SJS/TEN. One study showed that 23% (7/30) of patients had posttraumatic stress disorder 6 months after hospitalization for SJS/TEN. The investigators recommended routine psychiatric assessment in the acute disease period and for at least 1 year after discharge.25
Pulmonary, Gastrointestinal, and Renal
Interstitial pneumonia and obliterative bronchitis/bronchiolitis can be caused by SJS/TEN. Interstitial pneumonia tends to occur during the acute course, while obliterative airway disease manifests after resolution of SJS/TEN.21,22 Abnormal pulmonary function testing can be seen in more than half of SJS/TEN patients 2 months after the ADR.22 Gastrointestinal sequelae include esophageal strictures, intestinal ulceration, and cholestasis.22 Renal sequelae include acute kidney injury and glomerulonephritis, which may be secondary to the volume loss seen in SJS/TEN but may be irreversible.21
Special Populations
A correlation with infertility in women has been documented in patients with SJS/TEN; thus, follow-up with obstetrics and gynecology is recommended in women of child-bearing potential. The most considerable risk in pregnant women with SJS/TEN is premature birth, and mucosal necrosis of SJS/TEN can impair vaginal delivery.26 Antiretrovirals can be a cause of SJS/TEN in the human immunodeficiency virus–positive population.27 In those cases, it is best to discontinue the medication and find an alternative.
Risk factors for children can be different and can include viral and febrile illnesses as well as mycoplasma infection.28 Children also can be at an increased risk for poor ocular outcomes, such as permanent deficiency in visual acuity and blindness.29
Follow-up Recommendations
Patients should be counseled regarding sequelae and the multisystem nature of SJS/TEN. Inpatient referrals should be given as needed. It is important to watch for ocular symptoms for 1 year after SJS/TEN resolution. When ocular involvement is present, follow-up with ophthalmology is recommended within 1 month of discharge and then at the discretion of the ophthalmologist. Pulmonary function should be monitored for 1 year after SJS/TEN, starting 1 month after discharge and then at the discretion of the pulmonologist. Patients also should be screened for psychologic sequelae for at least 1 year after discharge.
FINAL THOUGHTS
Adverse drug reactions are notable causes of inpatient hospitalization and may lead to considerable sequelae. These ADRs range in severity from more common and benign maculopapular exanthems to severe multiorgan ADRs such as DRESS syndrome and SJS/TEN.
In AGEP, it is important to monitor patients with preexisting dermatologic diseases and to screen for visceral involvement. DRESS syndrome has the potential to cause immune dysregulation and variable long-term adverse sequelae, both from the disease itself and from corticosteroid therapy. Mucocutaneous sequelae of SJS/TEN can potentially affect a patient’s cutaneous, ocular, genitourinary, mental, pulmonary, gastrointestinal, and renal health.
The baseline recommendations provided here warrant more frequent monitoring if the findings and symptoms are severe. In all of these cases, if a causative medication is identified, it should be added to the patient’s allergy list and the patient should be counseled extensively to avoid this medication and other medications in the same class. If a single agent cannot be identified, referrals for patch testing may be of some utility, particularly in AGEP and DRESS syndrome.30,31