Cone Beam Computed Tomographic Cone beam computed tomographic characterization of short root anomaly in chloroquine-induced Stevens-Johnson Syndrome - a case report.

Stevens-Johnson syndrome (SJS) is a rare medical condition with severe mucocutaneous reaction due to infection or adverse drug reaction. The present case reports the impact of chloroquine-induced SJS on the tooth root development. A 20-year-old Indian male reported to conservative dentistry and endodontics speciality clinic with the chief complaint of food lodgement and sensitivity in maxillary and mandibular posterior teeth. He had a past medical history of severe cutaneous reaction after taking Tab. Lariago (chloroquine) for treatment of malarial fever at the age of 8 years. The acute inflammatory immune response was managed by hospitalization and administration of steroids and anti-inflammatory drugs. Clinical examination revealed dry mucosa, carious teeth with adequate oral hygiene. Panoramic X-ray showed multiple teeth with short roots. A detailed cone beam computed tomographic scan (CBCT) demonstrated a healthy bone trabecular pattern with the absence of any periapical radiolucency. Short, blunt roots with immature apex were seen in many teeth. Based upon the measurement of root to the crown ratio on the CBCT scan and correlating the development status of teeth with the medical history, a diagnosis of short root anomaly (SRA) after chloroquine-induced SJS was made. This is the first report to describe the three-dimensional features of teeth with SRA in a patient with SJS. Diagnostic, restorative, and endodontic implications of SJS are highlighted.

Clinical and histopathologic characteristics of submandibular gland in Stevens-Johnson syndrome: A comparative study.

OBJECTIVE: The objective of this study was to investigate the clinical manifestations and pathologic appearances of the submandibular gland (SMG) in Stevens-Johnson syndrome (SJS). STUDY DESIGN: Patients with autologous transplantation of SMG for treatment of severe dry eye between March 1998 and May 2018 were divided into the SJS group (70 cases) and non-SJS group (50 cases) according to the history of SJS. The SMG weight and computed tomography volume and salivary flow rate were measured. The concentration index and secretion index were estimated using scintigraphy with technetium-99m-pertechnetate. Histopathology studies of SMG tissues were conducted, and the acini parameters were measured using a digital image analyzer. RESULTS: A decreased computed tomography volume and weight was observed in 48.57% the SJS group and 2% in the non-SJS group (P < .01). The rest whole, acid-stimulated whole, and SMG rest salivary flow rates decreased in the SJS group (P < .05). The normal SMG concentration index (37.5% vs 96.67%, P < .001) and secretion index (35% vs 96.67%, P < .001) rates were lower in the SJS group than in the non-SJS group. The glandular parenchyma was reduced, the acinar space was widened, and the fat content was increased in the SJS group. CONCLUSION: SMG atrophic and degenerative changes occurred in the SJS group, with a decrease in salivary secretion function in more than half of the patients.

Cohort study of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis in China: evaluation of risk models and new predictor of pulmonary consolidation on computed tomography.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe diseases. This study aimed to validate the predictive ability of risk models in patients with SJS/TEN and propose possible refinement in China. Patients in the Department of Dermatology of Huashan Hospital from January 2008 to January 2019 were included. Results showed that the severity-of-illness score for TEN (SCORTEN) had a good discrimination (area under the receiver operating characteristic curve (AUC), 0.78), and it was superior to auxiliary score (AS) and ABCD-10, which indicates age, bicarbonate level, cancer, dialysis, and 10% involved body surface area (AUC, 0.69 and 0.68, respectively). The calibration of SCORTEN (Hosmer-Lemeshow goodness-of-fit test, P = 0.69) was also better than that of AS (P = 0.25) and ABCD-10 (P = 0.55). SCORTEN and ABCD-10 were similar (Brier score (BS), 0.04 and 0.04) in terms of accuracy of predictions. In addition, the imaging appearance of pulmonary consolidation on computed tomography was associated with high mortality. Refined models were formed using the variables and this imaging appearance. The refined AS and ABCD-10 models were similar in discrimination compared with the original SCORTEN (0.74 vs. 0.78, P = 0.23; 0.74 vs. 0.78, P = 0.30, respectively). Therefore, SCORTEN showed good discrimination performance, calibration, and accuracy, and refined AS or ABCD-10 model may be an option when SCORTEN variables are not available.

Cohort study of patients with Stevens-Johnson syndrome and toxic epidermal necrolysis in China: evaluation of risk models and new predictor of pulmonary consolidation on computed tomography / 医学前沿

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe diseases. This study aimed to validate the predictive ability of risk models in patients with SJS/TEN and propose possible refinement in China. Patients in the Department of Dermatology of Huashan Hospital from January 2008 to January 2019 were included. Results showed that the severity-of-illness score for TEN (SCORTEN) had a good discrimination (area under the receiver operating characteristic curve (AUC), 0.78), and it was superior to auxiliary score (AS) and ABCD-10, which indicates age, bicarbonate level, cancer, dialysis, and 10% involved body surface area (AUC, 0.69 and 0.68, respectively). The calibration of SCORTEN (Hosmer-Lemeshow goodness-of-fit test, P = 0.69) was also better than that of AS (P = 0.25) and ABCD-10 (P = 0.55). SCORTEN and ABCD-10 were similar (Brier score (BS), 0.04 and 0.04) in terms of accuracy of predictions. In addition, the imaging appearance of pulmonary consolidation on computed tomography was associated with high mortality. Refined models were formed using the variables and this imaging appearance. The refined AS and ABCD-10 models were similar in discrimination compared with the original SCORTEN (0.74 vs. 0.78, P = 0.23; 0.74 vs. 0.78, P = 0.30, respectively). Therefore, SCORTEN showed good discrimination performance, calibration, and accuracy, and refined AS or ABCD-10 model may be an option when SCORTEN variables are not available.

Asymptomatic Hyperamylasemia in Stevens-Johnson Syndrome Is Associated with Intestinal Barrier Dysfunction.

METHODS: A retrospective study on SJS patients was conducted at a tertiary medical center. All patients diagnosed as SJS, with available serum amylase index, were included. Clinical data of all subjects were retrospectively collected and analyzed. Colonic mucosal biopsies were obtained to measure tight junction protein expression. RESULTS: A total of nine patients were included in the present study for study analysis. The average serum amylase of the study cohort was 228.78 ± 204.18 U/L. Among which, five patients had a positive fecal occult blood test (FOBT). Colonic mucosal biopsies were obtained and stained with occludin and zonula occludens-1 (ZO-1). The expression of occludin and ZO-1 was significantly downregulated in SJS patients (p < 0.01), which was indicative of intestinal barrier dysfunction. CONCLUSION: Hyperamylasemia often extends beyond pancreatic diseases. Clinical awareness of asymptomatic hyperamylasemia secondary to other systemic diseases can help avoid unnecessary overexamination and overtreatment.

Evaluating Dry Eye and Meibomian Gland Dysfunction With Meibography in Patients With Stevens-Johnson Syndrome.

PURPOSE: To investigate ocular surface and meibomian gland characteristics using infrared meibography in patients with Stevens-Johnson syndrome (SJS). METHODS: This is a single-center, prospective, noncontrolled, observational study. Thirty-two Thai patients (64 eyes) with SJS for 1 year or longer (1-44 years) were enrolled in the study. All participants underwent a comprehensive ophthalmic examination including slit-lamp biomicroscopy, tear meniscus height, fluorescein tear breakup time, ocular surface fluorescein staining, eyelid morphology, Schirmer 1 test, meibomian gland expressibility, and upper and lower eyelid meibography using a noncontact infrared meibograph mounted on a slit-lamp biomicroscope. RESULTS: The mean age was 42.2 ± 17.7 years (range, 4-68 years). Twenty-nine patients (90.6%) had a history of severe ocular complications in the acute stage of the disease. Medications were the most common cause of SJS (93.8%). Meibum quality could not be assessed in 23 patients (71.9%) due to no glands expressible. Partial or complete loss of the meibomian glands in either the upper or lower eyelid was found in all patients. The degree of meibomian gland dropout significantly correlated with tear breakup time (P < 0.001), meibum quality (P < 0.001), meibum expressibility (P < 0.001), ocular surface staining (P < 0.001), and presence of long-term ocular sequelae including symblepharon (P = 0.027) and limbal stem cell deficiency (P = 0.003). CONCLUSIONS: SJS is associated with obstructive meibomian gland dysfunction. The severity of meibomian gland dropout has a relationship with abnormal dry eye tests, subjective meibomian gland evaluation, and other ocular sequelae of SJS.

Stevens-Johnson syndrome manifesting late in the course of pembrolizumab therapy.

Pembrolizumab is a humanized antibody that targets programmed cell death receptor-1. This agent is approved for use in the treatment of several malignancies. While pruritus and papulo-erythematous rash are not uncommon with its use, severe reactions such as Stevens-Johnson syndrome/toxic epidermal necrolysis are very rare. We present herein a case of Stevens-Johnson syndrome occurring in a patient who had previously tolerated pembrolizumab without significant side effects for seven months. Prompt recognition of Stevens-Johnson syndrome/toxic epidermal necrolysis and discontinuation of the offending agent are paramount to ensure a favorable outcome.

Cone-beam computed tomography for planning and assessing surgical outcomes of osteo-odonto-keratoprosthesis.

PURPOSE: The aim of this study was to investigate the feasibility and effectiveness of cone-beam computed tomography (CBCT) in the planning, assessment, and follow-up for osteo-odonto-keratoprosthesis (OOKP). METHODS: Six OOKP patients received a CBCT scan. CBCT scans were performed before and/or between ∼5 and 504 months after the primary OOKP intervention. Preoperative and postoperative results of the CBCT were assessed, regarding the available teeth and to assess the loss of bone in 1 patient, respectively. Resorption of the osteo-odonto-lamina was measured and graded. Five different measurements (I-V) were performed in the coronal and transversal views of CBCT. RESULTS: Four CBCT scans were performed preoperatively and 4 postoperatively. The follow-up time of the patients is between ∼1 to 528 months. Visualization of the potential donor teeth resulted in accurate 3-dimensional visualization of the tooth-lamina-bone complex. CBCT was found to help in the preoperative decision-making process (diameter of optical implant) and in enabling accurate postoperative evaluation of the bone volume and resorption zones of the OOKP. Loss of bone could be measured in a precise range and showed in the completed cases an average loss of 20.2%. CONCLUSIONS: The use of CBCT simplifies the preoperative decision making and ordering process. It also helps in determining the postoperative structure and resorption of the prosthesis.

[Severe Stevens-Johnson syndrome in 4 children]. / Syndrome de Stevens-Johnson sévère chez l'enfant: à propos de 4 observations.

Stevens-Johnson syndrome is an acute, self-limiting disease of the skin and mucous membranes. Erythema multiforme, Stevens-Johnson syndrome and toxic epidermal necrolysis are all part of a single spectrum illness. We report severe erythema multiform in 4 children aged from 6 to 15 years old. Erythema was mostly related to mycoplasma pneumoniae infection (3/4) and 1 case was attributed to drugs. Two children developed severe sequelae (obliterans bronchiolitis). No patient had recurrent disease. The early use of steroids is still debated, but in our experience it seems to benefit overall. A long term follow-up is necessary with the study of pulmonary function tests and chest X-rays ophtalmologic and dermatologic examination.

Vulvovaginal involvement in toxic epidermal necrolysis: a retrospective study of 40 cases.

OBJECTIVE: To determine the incidence, features, and surgical treatment of vulvovaginal lesions in toxic epidermal necrolysis. METHODS: Acute genital lesions were studied retrospectively in 40 women hospitalized for toxic epidermal necrolysis in a dermatologic intensive care unit. A questionnaire was sent to evaluate sequelae and their effects on sexual activity. Examination and surgical treatment were proposed to patients with symptomatic sequelae. RESULTS: Twenty-eight of the 40 patients reported genital lesions during the acute phase of toxic epidermal necrolysis. No specific treatment was carried out during the acute period. Sequelae were observed in five cases, of which three involved the lower genital tract and two the vulva exclusively. The two patients with exclusive vulval involvement did not attempt any sexual activity. The other three patients with both vulval and vaginal lesions were unable to have normal sexual intercourse. Two of the three patients were treated surgically. One patient succeeded in having intercourse, but surgery failed to relieve dyspareunia. CONCLUSION: Genital involvement is frequent during toxic epidermal necrolysis but rarely leads to symptomatic sequelae. Surgery for synechiae is sometimes necessary to recover sexual activity because the vulvovaginal canal is stenotic. Because of the partial effect on pain relief after surgery, a preventive approach should be tried.

[Multiple complications of a mycoplasma pneumoniae infection]. / Multiples complications d'une infection à mycoplasma pneumoniae.

We report the case of a patient who was admitted to hospital for a pneumonia in association with a Stevens-Johnson syndrome. The association of these conditions suggested a Mycoplasma pneumoniae infection, which was confirmed on serology. There were three other extra-respiratory complications discovered: cold agglutinins, a disturbance of liver function tests, and circulating anticoagulant; both the two latter and the Stevens-Johnson syndrome are rare complications.