Clinical Profiles of Japanese Patients with Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Collected by a Nationwide System from 2006 to 2023.

Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are potentially life-threatening severe cutaneous adverse drug reactions. These diseases are rare, and their onset is difficult to predict because of their idiosyncratic reactivity. The Japan Severe Adverse Reactions Research Group, led by the National Institute of Health Sciences, has operated a nationwide to collect clinical information and genomic samples from patients with SJS/TEN since 2006. This study evaluated the associations of clinical symptoms with sequelae and specific causative drugs/drug groups in Japanese patients with SJS/TEN to identify clinical clues for SJS/TEN treatment and prognosis. Acetaminophen, antibiotics, and carbocisteine were linked to high frequencies of severe ocular symptoms and ocular sequelae (p < 0.05). For erythema and erosion areas, antipyretic analgesics had higher rates of skin symptom affecting <10% of the skin than the other drugs, suggesting narrower lesions (p < 0.004). Hepatic dysfunction, was common in both SJS and TEN, and antiepileptic drugs carried higher risks of hepatic dysfunction than the other drug groups (p = 0.0032). This study revealed that the clinical manifestations of SJS/TEN vary according to the causative drugs.

Drug eruptions and the vulva.

Drug reactions affecting the vulva are understudied and underreported, with some having the potential to cause serious morbidity through long-term sequelae. We conducted a literature review to investigate the current evidence about vulval drug eruptions. We aimed to establish the extent of drug reactions affecting the vulva, identify the common culprit drugs, and review current evidence and guidelines regarding their management. The vulval involvement seen in Steven-Johnson syndrome, toxic epidermal necrolysis and fixed drug eruption forms the focus of this review, but we also summarize the current evidence regarding less common reactions.

Anesthesia management in a child with mucopolysaccharidosis and toxic epidermal necrolysis: A case report.

Toxic epidermal necrolysis and mucopolysaccharidosis are both rare diseases that pose significant airway maintenance challenges to anesthesiologists. In this report, we describe the anesthesia management in a 4-year-old male with mucopolysaccharidosis type II who developed toxic epidermal necrolysis and presented for ophthalmic surgical procedures. Combined use of propofol and ketamine with the support of high-flow nasal oxygen enabled adequate analgesia and sedation while maintaining spontaneous ventilation and airway patency. The strategy presented in this report may contribute to enhancing the safety of sedation in spontaneously breathing children with abnormal airways.

Severe reactive infectious mucocutaneous eruption mimicking drug-induced epidermal necrolysis triggered by norovirus.

Reactive infectious mucocutaneous eruption (RIME) is an eruptive mucositis with varying degrees of cutaneous involvement presumed to be due to an immunologic response to various infectious pathogens. Most reported cases occur after a prodromal upper respiratory illness. We present a patient with a particularly severe case mimicking drug-induced epidermal necrolysis found to be triggered by asymptomatic norovirus infection, a virus not previously reported in association with RIME.

A Comprehensive Assessment of Long-Term Complications in Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis.

INTRODUCTION: Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and SJS/TEN overlap syndrome are rare severe hypersensitivity reactions that lead to epithelial sloughing. Studies investigating the chronic multisystem effects of these syndromes and assessing patients in terms of quality of life (QOL), depression, and anxiety in the pediatric population are limited. In this study, we aimed to investigate the long-term effects of these diseases from a multisystem perspective. METHOD: Sixteen pediatric patients diagnosed with SJS, TEN, and SJS/TEN overlap syndrome were evaluated between September 2020 and March 2021. Physical and eye examinations were performed. To evaluate QOL and psychological status, Children's Dermatology Life Quality Index (CDLQI), Screen for Child Anxiety-Related Emotional Disorders (SCARED), and Children's Depression Inventory (CDI) were conducted. The patients' general characteristics, symptoms, and examination findings at their first admission were retrospectively obtained from the hospital's electronic records. RESULTS: Nineteen percent of the patients were female (n = 3). There were 7 patients (44%) with the diagnosis of SJS, 5 patients (31%) with TEN, and 4 patients (25%) with SJS/TEN overlap. The median follow-up time of the subjects was 6.5 years. The most common sequelae in the chronic period were skin changes (n = 13, 81%). Hyperpigmentation was the most common skin change (n = 9, 56%). In the last evaluation, 9 cases had eye involvement. In two cases, eye examination was normal in the acute phase, while ocular involvement was present in the chronic period. In 4 (50%) patients, there was height and/or weight percentile loss. Three patients' SCARED scores and 2 patients' CDI scores were high. According to the CDLQI survey, SJS, TEN, or SJS/TEN overlap syndrome had a small to moderate effect on the QOL in the 43% (n = 6) of the patients. The ANA values of 3 patients (60%) were positive at the follow-up and negative at the first admission. CONCLUSION: SJS, TEN, and SJS/TEN overlap syndrome may cause sequelae even after a long time of the onset of the disease. Patients' QOL and psychological status can be affected negatively. Ocular symptoms may develop in the follow-up, even without involvement in the acute period. Patients with SJS, TEN, and SJS/TEN overlap syndrome should be followed up in the chronic period and approached multidisciplinary.

APOA4 as a novel predictor of prognosis in Stevens-Johnson syndrome/toxic epidermal necrolysis: A proteomics analysis from two prospective cohorts.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening adverse drug reactions. Conventional systemic therapies are of limited efficacy and often exhibit strong side effects. OBJECTIVE: To assess the efficacy and safety of the combination treatment with a tumor necrosis factor-α antagonist adalimumab and delineate the underlying mechanisms. METHODS: We evaluated the efficacy and safety of the combination therapy with adalimumab by comparing 2 treatment cohorts of SJS/TEN patients. Patient plasma samples were collected for proteomics analysis. RESULTS: The combination therapy with adalimumab significantly shortened the time to mucocutaneous re-epithelization and healing, with reduced side effects caused by corticosteroids. Plasma proteomic profiling showed that apolipoprotein A-IV (APOA4) was one of the most significant differentially expressed proteins. Multivariate regression analysis revealed that APOA4 level was significantly associated with prognosis parameter of SJS/TEN (P = .004), but not with disease severity score (severity-of-illness score for toxic epidermal necrolysis [SCORTEN]) (P = .118). Thus further research will be helpful to effectively incorporate APOA4 into current SCORTEN-driven protocols. LIMITATIONS: The cohort size is relatively small. Both cohorts had low overall SCORTEN scores. CONCLUSION: Adalimumab in combination with corticosteroids demonstrates significant clinical benefits over corticosteroids alone in SJS/TEN patients. Moreover, APOA4 may serve as a novel prognostic marker of SJS/TEN.

A case of eruptive lichen spinulosus after toxic epidermal necrolysis.

We report a case of a 13-year-old boy who presented with eruptive monomorphic white papules on the trunk and arms involving regions previously affected by toxic epidermal necrolysis (TEN). Biopsy revealed compact keratin involving the hair follicle and sparse mixed perivascular infiltrate, findings consistent with lichen spinulosus. Improvement was noted after treatment with ammonium lactate 12% lotion. While cutaneous dyschromia and xerosis are common after TEN, lichen spinulosus has not yet been described in the literature. It is important for providers to be aware of any potential cutaneous sequelae of TEN that can affect quality of life in order to best counsel their patients.

Long-Term Benefits of Tear Exchangeable Limbal-Rigid Contact Lens Wear Therapy in Stevens-Johnson Syndrome Cases.

OBJECTIVES: To evaluate the long-term benefits of tear-exchangeable, limbal-rigid contact lens (CL) wear therapy in patients with Stevens-Johnson syndrome (SJS)-associated ocular sequelae. METHODS: This retrospective study evaluated 50 eyes of 41 SJS patients (15 men and 26 women) who underwent limbal-rigid CL wear therapy for more than 2 years post fitting. Ocular sequelae (i.e., conjunctival hyperemia, corneal neovascularization, and upper tarsus scarring) before fitting and at 3 months, 6 months, 12 months, and annually after initiating CL wear therapy were evaluated and then graded on a severity score (range: 0-3, maximum score: 3). Moreover, visual acuity (VA) at immediately post initiating CL wear therapy was evaluated. RESULTS: The mean follow-up period was 4.3±1.1 years. Compared with before fitting, the mean conjunctival hyperemia score improved from 1.14 to 0.86 at 3 months of CL wear therapy ( P <0.01) and was maintained thereafter; the mean corneal neovascularization score improved from 2.10 to 1.98 at 3 months of CL wear therapy, with no deterioration of the score observed in all cases at the final follow-up examination, and mean VA (log of minimum angle of resolution) improved from 1.60 to 1.04 at immediately post initiating CL wear therapy ( P <0.01). CONCLUSIONS: Limbal-rigid CL wear therapy can provide long-term ocular surface stabilization and improved VA in SJS patients.

Sutureless Amniotic Membrane Transplantation Using Pediatric Nasogastric Tube for Patients With Acute Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis.

PURPOSE: Evaluation of the results of sutureless amniotic membrane transplantation (AMT) using a pediatric nasogastric tube (NGT) for patients with acute Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) was aimed. METHODS: Twenty-six eyes of 13 patients with SJS/TEN with ocular involvement at a single tertiary care hospital between May 2020 and January 2022 were included in the study. An amniotic membrane was implanted to the ocular surface and conjunctival fornix using a modified symblepharon ring (MR) created by inserting NGT end-to-end. Patients were followed for at least 6 months. The mean duration of the surgery was recorded. The presence of corneal epithelial defect and vascularization, meibomian gland dysfunction (MGD) grade, conjunctival scarring, eyelid margin keratinization, symblepharon, and trichiasis were recorded. RESULTS: The mean age of the patients was 38.4±17.6 years. Amniotic membrane transplantation was performed only once for eight patients and twice for five patients. The mean duration of the surgery was 3.2±0.4 min. The modified symblepharon ring was removed 28 days after its application. At the first examination, 20 eyes (76.9%) had a corneal epithelial defect and 6 (23.1%) had early symblepharon. At the postoperative 6 months, although seven eyes (26.9%) had conjunctival scarring and 5 (19.2%) had eyelid margin keratinization, all patients were without any corneal defect, corneal vascularization, or symblepharon. Only 11 eyes (42.3%) showed MGD, and 1 eye (3.8%) showed trichiasis. CONCLUSIONS: This study showed that sutureless AMT using pediatric NGT could be a potentially fast and inexpensive treatment option for the treatment of SJS/TEN with ocular involvement at the bedside without the need for general anesthesia.

Cicatricial Entropion in Chronic Cicatrizing Conjunctivitis: Potential Pathophysiologic Mechanisms and Long-Term Outcomes of a Modified Technique.

PURPOSE: The purpose of this study was to assess the long-term outcomes of severe cicatricial entropion repair with mucous membrane grafting in patients with chronic cicatrizing conjunctivitis and report histopathological changes in the eyelid margin area. METHODS: Prospective interventional study included 19 patients with severe cicatricial entropion with trichiasis (N = 20 eyelids; 19 upper and 1 lower eyelid) who underwent anterior lamellar recession (with back cuts) and mucous membrane grafting cover for bare anterior tarsus, lid margin, and 2 mm of marginal tarsus, and had a minimum 6 months of follow-up. The anterior lamella and metaplastic eyelid margins were sent for routine Haematoxylin and Eosin and special staining with Masson trichrome stain. RESULTS: The etiologies were chronic Stevens-Johnson syndrome (N = 6), chemical injury (N = 11), and drug-induced pseudopemphigoid (N = 2). Five eyes had undergone entropion correction in the past, and 9 had electroepilation for trichiasis. Entropion was well corrected (without residual trichiasis) in 85% of eyelids with primary surgery. The etiology-wise success rates were 100% for Stevens-Johnson syndrome, 72.7% for chemical injury, and 100% for drug-induced pseudopemphigoid. Three eyelids with failure belonged to chemical injury, and trichiasis in these eyes could be managed with subsequent interventions except in 1 case. All eyelids had no entropion at a mean follow-up of 10.8 months (range, 6-18). Histopathological evaluation of anterior lamella (N = 10) and eyelid margins revealed significant fibrosis in subepithelial, perimysium (muscle of Riolan), and perifollicular areas. CONCLUSION: Anterior lamellar recession combined with mucous membrane grafting achieves good cicatricial entropion correction except in eyes with chemical injury. The eyelid margins in these eyes have persistent inflammation, and fibrosis involving lash follicles.

Stevens-Johnson syndrome and toxic epidermal necrolysis from pathologist's point of view.

Stevens-Johnson syndrome and toxic epidermal necrolysis (Lyell syndrome) are rare diseases characterized by rapid blistering followed by extensive skin and mucosal exfoliation and constitutional symptoms. In most cases, drugs are the main triggers, but the etiopathogenesis of the diseases is not fully understood. Lyell syndrome is associated with a high mortality rate, reported to be around 35%. Therefore, early diagnosis requiring close interdisciplinary cooperation is essential. The diagnosis based on the clinical picture and a detailed pharmacological history should be confirmed by histopathological examination of the skin specimen, including analysis by direct immunofluorescence.

Chronic ocular sequelae in Stevens-Johnson syndrome: a genetic association study.

Purpose: This study sought to investigate the association of molecular markers with chronic ocular sequelae in Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). Methods: One hundred SJS/TEN patients (200 eyes) with confirmed diagnosis were enrolled between July 2011 and July 2015 from a tertiary eye-care hospital, and their clinical histories were noted. Each eye was scored for severity of manifestation on a scale of 0-5. Peripheral blood samples were collected for DNA followed by screening for interleukin (IL-4, IL-13, IL-4R) polymorphisms, HLA-A locus allele typing, and sera to detect levels of the apoptotic markers granulysin and sFas L. Results: Of the 100 enrolled patients (53 males/47 females; age range: 6-58 years), the incriminating drugs were non-steroidal anti-inflammatory (52%), antibiotics (10%), sulphonamides (8%), anti-epileptics (6%), and unknown (24%). Significant differences in the frequencies of IL-4R polymorphism, HLA-A*3301, HLA-A*02, and HLA-A*2402 alleles, and elevated levels of granulysin and sFas L were observed in patients compared to controls. The ocular complications of conjunctival keratinization (p=0.004) showed an association with IL-13 promoter region (IL-13a) genotypes. Conclusions: The study highlights the possible association of interleukin-13 with severity-graded chronic sequelae and the role of HLA-A alleles- HLA-A*3301, HLA-A*02, and HLA-A*2402 in SJS/TEN causation and manifestation. Screening of these alleles may help caregivers to identify markers associated with severe and lifelong ocular complications, and help in appropriate treatment and management of the condition.

Toxic epidermal necrolysis-like presentation of toxic erythema of chemotherapy.

Toxic erythema of chemotherapy is an umbrella term encompassing a range of reactions characterized by symmetric erythematous to dusky patches, which can develop oedema, desquamation and/or purpura. We describe an elderly patient with prostate cancer who developed this complication while receiving docetaxel chemotherapy, presenting with prominent mucosal and periorificial involvement, along with epidermal necrosis, closely mimicking toxic epidermal necrolysis.

Matrix metalloproteinase 9 is associated with conjunctival microbiota culture positivity in Korean patients with chronic Stevens-Johnson syndrome.

BACKGROUND: Stevens-Johnson syndrome (SJS) is an abnormal immune-response causing extensive exfoliation of the mucocutaneous tissue including conjunctiva. While several factors are associated with the alteration of conjunctival microbiota, the conjunctiva of SJS patients are found to harbor a different microbiota compared to healthy subjects. We investigated the conjunctival microbiota of Korean SJS patients, and identified factors associated with the conjunctival microbiota and its positive culture. METHODS: Medical records were retrospectively reviewed in 30 chronic SJS patients who had undergone conjunctival swab culture sampling. Demographic factors, chronic ocular surface complications score (COCS), tear break-up time (TBUT), tear secretion, tear matrix metalloproteinase 9 (MMP9), and results of conjunctival swab culture were assessed. RESULTS: Positive culture was seen in 58.1%. Gram positive bacteria was most commonly isolated, among which Coagulase-negative Staphylococci (45.5%) and Corynebacterium species (40.9%) were predominantly observed. Tear MMP9 positivity was observed significantly more in the positive culture group (100%) compared to the negative culture group (70%) (P = 0.041). Topical cyclosporine and corticosteroid were not associated with repetitive positive cultures. No significant differences in COCS, TBUT, and tear secretion were found between culture-positive and culture-negative groups. CONCLUSION: Our study suggests that tear MMP9 positivity may be related with the presence of an abnormal ocular surface microbiota in chronic SJS patients.

Severe cutaneous adverse drug reactions: diagnostic approach and genetic study in a Brazilian case series.

Summary: Background. Severe cutaneous adverse reactions (SCAR) are potentially fatal reactions. Genetic predisposition is involved in their pathogenesis related to drugs and ethnicities, however in a mixed population these relationships are still unknown. The aim of this study was to describe phenotypes, suspect drugs and HLA-alleles related to SCAR, identified by a systematized approach in a Brazilian case series. Methods. Patients who were diagnosed with SCAR between March 2011 and July 2019 at our university hospital were included. European Network for Drug Allergy (ENDA) questionnaire was used to collect clinical and laboratory data and algorithms for assessment of drug causality were applied. Socio-demographic variables included age, gender and skin color/ethnicity. Drug patch tests (DPT) and HLA-A, -B, -DRB1 typing were carried out. Results. A total of 74 patients were included: 36 (48.64%) with SJS/TEN, 32 (43.24%) DRESS/DIHS, 3 (4.05%) AGEP, 2 (2.70%) overlap(DRESS/SJS and DRESS/AGEP) and 1 (1.35%) GBFDE. The median age was31.5 years (IQR = 14-52.25), most were female (n = 44/59.46%) and brown (n = 38/51.35%). Anticonvulsants (n = 32/43.24%) were the largest group involved and antibiotics (n = 26/35.13%) were the second most common. Two patients with DRESS died during the acute phase. Positive DPT were shown only in anticonvulsant associated DRESS. HLA related to abacavir, allopurinol and carbamazepine were identified. Conclusions. A systematized approach allowed the phenotypic characterization of SCAR. The HLA-A*31:01, B*57:01 and B*58:01 alleles were identified, reinforcing the causality in SCAR by CBZ, ABC and ALLO in the Brazilian population.

[A case of Stevens-Johnson syndrome with binocular corneal perforation and multiple corneal transplantation which was difficult to control].

The 31-year-old female patient was admitted to the General Hospital of the Chinese People's Liberation Army for 3 days after the corneal transplantation of her right eye for 5 months.Four years ago, the patient developed red eyes, pain, dryness and photophobia after intravenous drip of cefuroxime sodium and metronidazole due to pelvic inflammation, accompanied by high fever, systemic rash and epidermal exfoliation, fingernail peeling, and mucosal ulceration in the eyes and mouth.Later, the patient received systemic hormone shock and point eye treatment in a local hospital, and the dry eyes gradually worsened. Despite continuous artificial tears and bandage mirror treatment, the corneal ulcer perforation in both eyes still occurred successively. After several penetrating keratografts and drug therapy, the ulcer and dissolution could not be prevented. He was admitted to our hospital due to corneal perforation in both eyes.Ophthalmic examination: visual acuity manual/15 cm in the right eye, intraocular pressure T-2, conjunctival sac stenosis, extensive corneal opacity and edema, ulcer about 8 mm, corneal perforation near the corneal limbus about 2 mm×5 mm below.The left eye had no light perception, a central corneal ulcer of about 8 mm, bulge of the posterior elastic layer, no anterior chamber, and atrophy of the eyeball.B-ultrasound showed choroidal detachment of the right eye.On the second day, the patient received right eye intraocular exploration, vitrectomy, ecotopic keratoscleral carrier Boston Ⅱ artificial keratoplasty, glaucoma valve implantation, autogenous ear cartilage implantation, conjunctival occlusion, and left eye lamellar keratoplasty, conjunctival occlusion.Postoperative visual acuity of right eye was -6.50 DS=0.12, intraocular pressure TN, ocular surface was stable.The left eye has no light perception and the ocular surface is stable.

Stevens-Johnson Syndrome in Children: Consider Monitoring for Bronchiolitis Obliterans.

We reviewed patients with Stevens-Johnson syndrome (SJS) evaluated at Children's Hospital Colorado and investigated the occurrence of bronchiolitis obliterans (BO). Approximately 9% of patients with SJS developed BO. Pediatricians should consider monitoring patients with SJS for BO, especially those with recurrent SJS and patients treated with mechanical ventilation.

Vulvovaginal manifestations in Stevens-Johnson syndrome and toxic epidermal necrolysis: Prevention and treatment.

The prevalence of acute vulvovaginal involvement in toxic epidermal necrolysis can be as high as 70%; up to 28% of female patients will also develop chronic vulvovaginal sequelae. There is little consensus regarding prevention and treatment of the gynecologic sequelae of both Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). We review acute and chronic sequelae, including erosions, scar formation, chronic skin changes, urethral complications, adenosis, malignant transformation, vulvodynia, and dyspareunia. We provide comprehensive recommendations for acute and long-term vulvovaginal care in adult and pediatric SJS/TEN patients. Treatment should include an ultrapotent topical steroid, followed by a nonirritating barrier cream applied to vulvar and perineal lesions. A steroid should be used intravaginally along with vaginal dilation in all adults (but should be avoided in prepubertal adolescents) with vaginal involvement. Menstrual suppression should be considered in all reproductive age patients until vulvovaginal lesions have healed. Last, referrals for pelvic floor physical therapy and to surgical subspecialties should be offered on a case-by-case basis. This guide summarizes the current available literature combined with expert opinion of both dermatologists and gynecologists who treat a high volume of SJS/TEN patients.

Disseminated intravascular coagulation in Stevens-Johnson syndrome and toxic epidermal necrolysis.

BACKGROUND: Patients with Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN) have high mortality rates. Disseminated intravascular coagulation has been reported in SJS/TEN patients. The influence of this lethal complication in patients with SJS/TEN is not well known. OBJECTIVE: This study aimed to investigate the risk and outcomes of disseminated intravascular coagulation in patients with SJS/TEN. METHODS: We analyzed the disseminated intravascular coagulation profiles of patients receiving a diagnosis of SJS/TEN between 2010 and 2019. RESULTS: We analyzed 150 patients with SJS/TEN (75 with SJS, 22 with overlapping SJS/TEN, and 53 with TEN) and their complete disseminated intravascular coagulation profiles. Disseminated intravascular coagulation was diagnosed in 32 patients (21.3%), primarily those with TEN. It was significantly associated with systemic complications, including gastrointestinal bleeding, respiratory failure, renal failure, liver failure, infection, and bacteremia. Additionally, SJS/TEN patients with disseminated intravascular coagulation had elevated procalcitonin levels. Among patients with SJS/TEN, disseminated intravascular coagulation was associated with a greater than 10-fold increase in mortality (78.1% vs 7%). LIMITATIONS: The study limitations include small sample size and a single hospital system. CONCLUSION: Disseminated intravascular coagulation is a potential complication of SJS/TEN and associated with higher mortality. Early recognition and appropriate management of this critical complication are important for patients with SJS/TEN.

Acute pancreatic injuries: A complication of Stevens-Johnson syndrome/toxic epidermal necrolysis associated with cytotoxic immunocell activation.

BACKGROUND: Complications involving internal organs are usually present in Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, pancreatic complications are rarely reported and studied. OBJECTIVE: To summarize clinical characteristics of SJS/TEN-associated acute pancreatic injuries and to investigate underlying inflammatory mechanisms. METHODS: Clinical records of 124 inpatients with SJS/TEN were reviewed. Serum levels of tumor necrosis factor α, interleukin (IL) 6, IL-18, IL-15, IL-12p70, and soluble CD56 were determined in 18 healthy donors and 17 patients with SJS/TEN, including 3 with acute pancreatic injuries. RESULTS: Acute pancreatic injury was diagnosed in 7.3% of patients (9/124) in the SJS/TEN cohort. Elevation of serum transaminase level and hypoalbuminemia occurred more frequently in patients with acute pancreatic injuries compared with those without pancreatic symptoms (P = .004 and <.001, respectively). Although acute pancreatic injury did not alter mortality rate of SJS/TEN, it was associated with longer hospitalization stays (P = .008). Within the serum cytokines whose levels were elevated in SJS/TEN, only IL-18 was found to be selectively increased in patients with acute pancreatic injuries compared with those without them (P = .03). LIMITATIONS: Cohort was small. CONCLUSION: Acute pancreatic injury is a gastrointestinal complication of SJS/TEN in which hepatotoxicity is more likely to occur. Overexpression of IL-18 might be involved in this unique entity.