Amikacin induced Stevens Johnson syndrome and toxic epidermal necrolysis overlap: a rare case report

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the most severe adverse drug reactions. They are characterized by necrosis and epidermal release in vesicobulosa skin, mucous orifice, and eyes, with more severe general symptoms. SJS/TEN-overlap syndrome is the term used to characterize situations where 10–30% of the body skin area is detached. TEN or SJS is one of the deadliest dermatological catastrophes. Despite being a rare condition, it frequently has a high death rate. Different types of purpuric macules or rounded patches with mucosal lesions are characteristic features of these type 3 hypersensitivity reactions. We are presenting a case of 50-year-old female patient brought to hospital on account of multiple black to red coloured raised lesions over body in the last 8 days. Patient was asymptomatic in the last 8 days after that patient noted red coloured lesions first over face followed by lower back, upper limb, lower limb followed by itching and Jiburning sensation all over the body. The patient had symptoms such as redness of eyes in the last 3-4 days, burning sensation and ulcers in mouth in the last 3-4 days. These symptoms developed day after she received amikacin. She was diagnosed with SJS-TEN overlap. This case highlights the precipitant of antibiotics for SJS. She was recovered completely after stopping the causative drug and treatment with Immunoglobulin with other symptomatic measures. The causative drug was found to be Amikacin and medicine taken for comorbid conditions like epilepsy, hypothyroidism and hypertension. Rarely, amikacin has been linked to a range of drug hypersensitivity reactions, including toxic epidermal necrolysis, Stevens-Johnson syndrome, and overlap between the two conditions.

Necrólisis epidérmica tóxica debido a un fármaco a base de caléndula más acetilcisteína: presentación de caso / Toxic Epidermal Necrolysis Induced by a Drug Combination of Calendula and Acetylcysteine: A Case Report / Necrólise epidermal tóxica por medicamento à base de calêndula mais acetilcisteína: relato de caso

Introducción: la hipersensibilidad por fármacos es inducida por una respuesta aberrante del sistema inmune; por lo general, impredecible, dosis independiente y amenazante para la vida del paciente. Las reacciones de hipersensibilidad por fármacos con compromiso mucocutáneo se dan en alrededor del 2 %-3 % de los pacientes hospitalizados. Objetivo: describir un caso de necrólisis tóxica epidérmica por un fármaco a base de caléndula y acetilcisteína como los presuntos desencadenantes. Presentación del caso: hombre de 39 años, quien asistió a emergencias, por malestar general, artralgias, mialgias, astenia y adinamia de un día de evolución. Tres días después refirió la aparición de lesiones purpúricas progre-sivas que se extendieron por cara, extremidades y glúteos; delimitadas; no dolorosas, y sin desaparecer a la digitopresión, asociado con disnea, inestabilidad hemodinámica y cardiovascular. Al sexto día de hos-pitalización, se interrogó al paciente, quien se había automedicado un fármaco de origen desconocido, presuntamente de extracto herbal a base de caléndula más acetilcisteína para artralgias, osteomialgias y disfagia. A la mañana siguiente, presentó lesiones purpúricas y ulcerativas extensas. Se observaron lesiones purpúricas progresivas y ulcerativas en cara extremidades y glúteos. Mediante la sospecha clínica y la evaluación histopatológica, se confirmó la necrólisis tóxica epidérmica. Conclusiones: las reacciones adversas medicamentosas severas que amenazan la vida del paciente son relativamente raras, pero representan un verdadero desafío diagnóstico y terapéutico. Es necesario profundizar en la investigación para esclarecer la causa de este tipo de reacción medicamentosa.

Introduction: Drug hypersensitivity is induced by an aberrant response from the immune system. It is usually unpredictable, dose-independent, and can be life-threatening to the patient. Drug-induced hypersensitivity reactions with mucocutaneous involvement occur in approximately 2­3% of hospitalized patients. However, Toxic Epidermal Necrolysis is a rare case. Aim: To describe a case of drug-induced toxic epidermal necrolysis triggered by Calendula officinalis and acetylcysteine. Case report: A 39-year-old male was pre-sented to the emergency room with malaise, arthralgia, myalgia, asthenia, and adynamia since day one. Three days later, he developed progressive purpuric lesions that spread to the face, extremities, and buttocks. These lesions were painless, not delineate, and did not blanch under pressure. They were asso-ciated with dyspnea and hemodynamic and cardiovascular instability. On the sixth day of hospitaliza-tion, the patient admitted to self-medicating with an unknown drug, presumably a herbal extract based on calendula and acetylcysteine, for arthralgia, myalgia, and dysphagia. The morning after the drug consumption, the patient developed extensive purpuric and ulcerative lesions. A diagnosis of toxic epi-dermal necrolysis was made based on clinical suspicion and histopathological confirmation. Conclusion:Severe adverse drug reactions that threaten a patient's life are relatively rare. However, they represent a real diagnostic and therapeutic challenge when they do occur

Introdução: a hipersensibilidade a drogas é induzida por uma resposta aberrante do sistema imunoló-gico; geralmente imprevisível, independente da dose e com risco de vida para o paciente. As reações de hipersensibilidade a drogas com comprometimento mucocutâneo estão em torno dos 2-3% dos pacien-tes hospitalizados. Objetivo: descrever um caso de necrólise epidérmica tóxica causada por uma droga à base de Calendula officinalis e acetilcisteína como os supostos desencadeantes. Apresentação do caso:homem, 39 anos, deu entrada no pronto-socorro manifestando mal-estar, artralgia, mialgia, astenia e adinamia há um dia. Posteriormente, três dias depois, relatou o aparecimento de lesões purpúricas pro-gressivas que se espalharam para a face, extremidades e nádegas; que são delimitadas, não dolorosas e não desaparecem com a acupressão associada a dispnéia, instabilidade hemodinâmica e cardiovascular. No sexto dia de internação, o paciente foi questionado, y disse que tinha se automedicado com uma droga de origem desconhecida, presumivelmente extrato de ervas à base de calêndula mais acetilcis-teína para artralgia, osteomialgia e disfagia. Na manhã seguinte, apresentou extensas lesões purpúricas e ulcerativas. Observam-se lesões purpúricas progressivas e ulcerativas que se estendem à face, extre-midades e nádegas. Pela suspeita clínica e confirmação histopatológica, confirma-se a necrólise epidér-mica tóxica. Conclusões: reações adversas graves a medicamentos que ameaçam a vida do paciente são relativamente raras, mas quando ocorrem representam um verdadeiro desafio a nível diagnóstico e terapêutico. São precisas mais pesquisas para esclarecer a causa desse tipo de reação medicamentosa.

Retrospective study on cutaneous adverse drug reactions in a tertiary care center

Background: Cutaneous adverse drug reactions (CADRs) range from mild-to-severe types and occasionally can become fatal. Hence, these incur additional financial burden both to patients and community. Aim and Objective: The aim of the study was to describe the characteristics of CADRs reported to ADR monitoring center (AMC) of a tertiary care center. Materials and Methods: CADRs reported to the AMC over a period of 2 ½ years were retrospectively studied. This study mainly focused on affected age group, gender, various pattern of CADRs, the group and name of drugs causing CADRs, and severity and causality assessment. Results: CADRs contributed 31.6% of the total ADRs reported to the AMC. Among these, 51.7% were females and 40% were of 51–60 years age group. About 37.9% of CADRs were pruritus. Antibacterial drugs were the most common cause of CADRs and beta-lactam antibiotics were responsible for 30% of CADRs. Stevens Johnson syndrome (SJS) constituted 4.9% of CADRs and 20% of this was due to Paracetamol. Drugs were withdrawn in 89% of cases and 85% cases recovered. On causality assessment, 94% were of probable category. Conclusion: Pruritus was the most commonly observed CADR and antibacterial drugs were the most common cause. Beta lactam antibiotic was the most frequent antibacterial drug to cause CADRs. The most common serious CADR was SJS and Paracetamol was the most frequent culprit drug.

5-Fluorouracil as a targeted lacrimal gland therapy for chronic Stevens-Johnson syndrome: A pilot study

Purpose: The surgical technique of periglandular 5?fluorouracil (5?FU) injection and its effects on the morphology and function of the main lacrimal gland of patients with severe dry eye disease due to Stevens–Johnson syndrome (SJS) are reported. Methods: 5?FU, as a potential antifibrotic agent, is given in the dose of 0.1 ml (50 mg/ml), subconjunctivally into the periglandular fibrosed area of the palpebral lobe of the main lacrimal gland. The injection is given using 30G needle into the subconjunctival plane and not into the substance of palpebral lobe. Results: Eight eyes (eight lobes) of seven chronic SJS patients (mean age, 32.5 years, <5 mm Schirmer) received the injection. All eight lobes demonstrated a visible reduction in the conjunctival congestion and scarring over the lobar area. The mean OSDI scoring improved from 65.3 to 51.1. Three patients with mean pre?injection Schirmer I values of 4 mm showed a mean change of 1 mm at four weeks following a single injection. The tear flow rate per lobe for the above three patients improved from 0.22, 0.12, and 0.16 ?l/min to 0.31, 0.12, and 0.21 ?l/min, respectively. Another patient with pre?injection Schirmer of 4 mm showed no change in tear flow. Three eyes with zero baseline Schirmer values (no visible secretory opening) had no improvement in tearing or ocular surface staining. Conclusion: Local 5?FU injection alters morphology of the conjunctiva overlying the palpebral lobe in SJS patients, but fails to show any significant effect on tear secretion.

Optimization and evaluation of tear protein elution from Schirmer’s strips in dry eye disease

Purpose: Extraction of tear protein from Schirmer’s strip is a prerequisite for the proper identification and screening of biomarkers in dry eye disease. The study compares different methods of extraction of tear proteins from the Schirmer’s strip. Methods: Reflex tear was collected from healthy controls (HC; n = 12), Stevens–Johnson syndrome (SJS; n = 3) and dry eye disease (DED; n = 3) patients using capillary tube. This tear was used to measure the volume absorbed by Schirmer’s strip per microliter. Different buffers (6) were used to compare the protein yield from the Schirmer’s strip in four different conditions. The tear proteins extracted using the highest protein yield buffer were analyzed by mass spectrometry. Results: A linear relationship between the tear volume and wetting length was observed (r = 0.0.997, n = 6). The highest yield was observed after incubation of the Schirmer’s strip in 100 mM ammonium bicarbonate (ABC) with 0.25% Nonidet P?40(NP?40) at 4°C for an hour (P < 0.00005). The in?solution digestion of tear eluted in the above condition 100 Mm ABC + 0.25% NP?40 with one?hour incubation yielded a total of 2119 proteins in HC, SJS, and DED. The unique protein observed in SJS and DED was 0.6% and 17.9%, respectively. The significantly expressed proteins are associated with innate immune response, proteolysis, wound healing, and defense response. Conclusion: A method for extraction of protein from Schirmer’s strip was optimized for increase in protein yield from the tear sample. SJS and DED tear samples have unique protein signature. The study will aid in better design of tear protein?based experimental study.

A case report on ofloxacin-induced Stevens–Johnson syndrome: Scorten-based approach

Stevens–Johnson syndrome (SJS) is a rare immune-mediated severe cutaneous adverse reaction with an incidence rate of 0.05–2 persons/million population/month. Drugs are the most commonly implicated in 95% of cases. In our report, a 52-year-old male patient presented with chief complaints of skin rashes over the body and was having a history of using a tab. ofloxacin for gastroenteritis. The severity of SJS was assessed using SCORTEN (=1). The drug can be considered as a probable/likely cause of adverse drug reaction as per causality assessment of the suspected adverse drug reactions. Early diagnosis helps the clinician to elude secondary infection and subsequent complications. It highlights the mandatory reporting of the offending drug and the necessity of pharmacovigilance in different countries.

Oral involvement by Stevens-Johnson Syndrome associated with carbamazepine in an immunosuppressed patient: Case report

Introduction: Stevens-Johnson syndrome (SJS) is caused by a delayed immune response triggered by the association of genetic and environmental factors. This reaction can be mediated mainly by some drugs. Objective: The purpose of this article is to report the case of a patient with SJS associated with carbamazepine. Case report: A man was hospitalized complaining of odynophagia, whitish plaques in the mouth and swelling of the lips. Clinical examination revealed ulcerated and erosive lesions involving the buccal mucosa, lips, tongue and hard palate. The lips were swollen and with hemorrhagic crusts. Papular and purplish-colored lesions were observed on the skin. The patient reported that the lesions started 10 days ago, coinciding with the start of carbamazepine use. The diagnosis of SJS was established based on clinical information and the patient treated with support therapy. Conclusion: SJS is a clinical condition that affects the oral mucosa and can be triggered by the use of carbamazepine. (AU)

Pharmaceutical analysis for a patient with rebamipide-induced Stevens-Johnson syndrome by clinical pharmacists / 中国药房

OBJECTIVE To provide reference for the diagnosis and treatment of Stevens-Johnson syndrome caused by rebamipide， and to explore the predisposing factors of Stevens-Johnson syndrome. METHODS Clinical pharmacists analyzed the treatment process of a patient with gastrointestinal diseases and evaluated the correlation between the drug used and adverse reactions， in order to determine the suspected allergenic drug causing Stevens-Johnson syndrome. The predisposing factors of patients with Stevens-Johnson syndrome were explored. RESULTS & CONCLUSIONS The suspected allergenic drugs that caused the patient to develop Stevens-Johnson syndrome included Ilaprazole enteric-coated tablets， Rebamipide tablets and Kangfuxin liquid. In summary， the suspect drug was identified as Rebamipide tablets according to the causality evaluation method of the National Center for Adverse Drug Reaction Monitoring， Naranjo’s scoring method and the algorithm of drug causality for epidermal necrolysis scoring criteria. Hypoproteinemia， competitive binding of plasma proteins between drugs， advanced age， bacterial and viral infections were the predisposing factors of Stevens-Johnson syndrome. Therefore， before using rebamipide in clinical practice， it is necessary to inquire about the patient’s allergy history in detail. During the use process， it is necessary to strengthen the patient’s medication monitoring and be alert to the occurrence of serious adverse reactions. If any abnormalities are found， the medication should be stopped immediately and symptomatic treatment should be given as soon as possible to ensure the safety and effectiveness of the patient’s medication.

Role of systematic wound management in the treatment of Stevens-Johnson syndrome/toxic epidermal necrolysis in children / 中华皮肤科杂志

Objective:To explore the role of systematic wound management in the treatment of Stevens-Johnson syndrome (SJS) /toxic epidermal necrolysis (TEN) in children.Methods:Clinical data were collected from 5 children with SJS/TEN, who were admitted to the Department of Dermatology, Tianjin Children′s Hospital from October 2019 to August 2021, and retrospectively analyzed to investigate the role of systematic management of the skin and mucosa wound besides drug therapy in the treatment of SJS/TEN.Results:The 5 patients, including 1 case of SJS, 1 case of SJS/TEN overlap syndrome, and 3 cases of TEN, were aged 2 to 13 years, and were cured and discharged after the treatment with intravenous methylprednisolone and systematic wound management. Among the 5 children, the severity of TEN scores (STENS) were 18.6 ± 1.9 points at admission, and 13.8 ± 1.9, 4.4 ± 0.5, and 2.0 ± 0.7 points on days 7, 10, and 14 during hospitalization, respectively; the time to complete re-epithelialization was 10.0 ± 2.1 days, and the length of hospital stay was 14.4 ± 4.2 days. During 4-month multidisciplinary follow-up, no scars or complications of the eyes, mouth, genitalia, or lungs occurred.Conclusion:Systematic wound management is helpful to control the progress of SJS/TEN, accelerate wound healing, and improve patients′ quality of life.

Stevens-Johnson syndrome secondary to massive inflammatory hyperplasia of bilateral lingual margins: a case report and literature review / 华西口腔医学杂志

Stevens-Johnson syndrome (SJS), also known as the multifactorial erythematous drug eruption, is a class of adverse reactions of the skin and mucous membranes primarily caused by drug allergy often involving the oral cavity, eyes, and external genital mucosa, generally accompanied by fever, and can be life-threatening in severe cases. In February 2022, the Department of Stomatology, the First Affiliated Hospital of Zhengzhou University admitted a patient with huge inflammatory hyperplasia of bilateral lingual margins secondary to SJS. Upon admission, no other obvious symptoms were observed except for tongue hyperplasia. The patient suffered from a severe adverse drug reaction caused by acetaminophen 2 months ago and was complicated by liver dysfunction and pulmonary infection. After 1 month of treatment and rehabilitation, he developed a secondary tongue mass and was subsequently admitted to Dept. of Oral and Maxillofacial Surgery Ward 2, the First Affiliated Hospital of Zhengzhou University. After completing the examination, the tongue mass was surgically removed. After a follow-up of 11 months, the patient's condition was satisfactory and no temporary discomfort was observed. The case of tongue mass secondary to SJS is extremely rare. If a stomatologist encounters a similar case, we should carefully inquire about the drug allergy history and recent medication history, and be alert to whether or not they had adverse drug reactions recently.

Eritema multiforme, una rara presentación de exantema en población pediátrica: a propósito de un caso / Erythema multiforme, a rare occurrence of exanthema in the pediatric population: on the subject of a case

El Eritema multiforme (EM) o eritema polimorfo es una enfermedad aguda de la piel de naturaleza inmunológica con o sin compromiso de mucosas, que puede comportarse como crónica recurrente. Se presenta con lesiones cutáneas en diana distintivas, a menudo acompañado de úlceras o bullas en mucosas (oral, genital u ocular). Entre sus formas clínicas se distingue: una forma menor caracterizado por un síndrome cutáneo leve y su forma mayor que se manifiesta como una afectación cutánea con daño mucoso marcado. Entre sus principales diagnósticos diferenciales se encuentran el Síndrome de Stevens-Johnson (SSJ) y Síndrome de Lyell (Necrólisis epidérmica tóxica (NET)). Tiene una incidencia estimada < 1%, siendo su forma mayor levemente más frecuente que su forma menor (0.8-6 por millón/año). Puede darse a cualquier edad, presentando un peak de incidencia entre los 20 y 30 años, predominando ligeramente el sexo masculino con una proporción 3:2, sin predilección racial. Su presentación en edad pediátrica es rara, más aún en la primera infancia. En esta población es más frecuente el EM menor recurrente. En el presente texto se reporta un caso de EM en población pediátrica como una rara forma de presentación exantemática, abordado en el Servicio de Pediatría del Complejo Asistencial Dr Victor Rios Ruiz (CAVRR)en la ciudad de Los Ángeles, Chile en el presente año.

Erythema multiforme (EM) also known as polymorph erythema is an acute skin disease of immunological nature with or without mucous membrane involvement, which may behave as chronic recurrent. It presents with distinctive targets like skin lesions, often together with ulcers or bullae in mucous membranes (oral, genital or ocular). Among its clinical forms are: a minor form characterized by a mild skin syndrome and its major form that manifests as a skin disease with marked mucosal damage. Among its main differential diagnoses are Stevens-Johnson Syndrome (SJS) and Lyell Syndrome (Toxic Epidermal Necrolysis (TEC)). It has an estimated incidence < 1%, with its major form being slightly more frequent than its minor form (0. 8-6 per million/year). It can occur at any age, presenting a peak incidence at the age between 20 and 30 years, with a slight predominance of males with a 3:2 ratio, without racial predilection. Its presentation in pediatric age is rare, even more so in early childhood. Minor recurrent EM is more common in this population. This paper reports a case of EM in the pediatric population as a rare form of exanthematic presentation, addressed at the Department of Pediatrics of the Complejo Asistencial Victor Rios Ruiz (CAVRR) in the city of Los Angeles, Chile this year.

Síndrome de stevens johnson, a propósito de un caso / Stevens Johnson syndrome: about a case

El síndrome de Stevens Jonson y la Necrolisis Epidérmica Toxica forman parte de un mismo espectro clínico caracterizado por lesiones cutáneas y mucosas con necrosis y desprendimiento de la piel, se diferencian por la extensión y la gravedad de las lesiones, aunque existen casos traslapados como en este caso, donde la paciente presentaba lesiones en más del 30% del cuerpo pero, solo fueron lesiones cutáneas y no así mucosas, lo que favoreció a su recuperación exitosa sin necesidad de ingresar a un servicio de terapia intensiva.

The Stevens Johnson syndrome and Toxic Epidermal Necrolysis are part of the same clinical spectrum characterized by cutaneous lesions and mucous membranes with necrosis and detachment of the skin. They differ by the extent and severity of the lesions. Although, there are overlapping cases as in this case, where the patient had lesions in more than 30% of the body. But they were only skin lesions and not mucous membranes, which favored her successful recovery without the need to enter and ITS (intensive therapy service).

Severe cicatricial entropion repair using mucous membrane graft in Stevens–Johnson syndrome

Background: Severe cicatricial entropion in Stevens–Johnson syndrome (SJS) patients is difficult to treat and is associated with a higher recurrence rate. Also, entropion in the presence of lid margin mucous membrane graft (MMG) further complicates the surgical anatomy and approach. Purpose: To report a modified surgical technique of repairing severe upper eyelid cicatricial entropion in an SJS patient with history of lid margin MMG. Synopsis: Cicatricial entropion in patients with SJS is entirely different from trachomatous cicatricial entropion. The involvement of the lid margin with keratinization, tarsal scarring, persistent conjunctival inflammation, and unhealthy ocular surface affects the surgical approach and outcomes. Conjunctiva?sparing surgery with reconstruction of the lid margin using MMG, flattening and repositioning the anterior lamella, and covering the bare tarsus with MMG rather than leaving it raw are the necessary modifications in this technique from conventional anterior lamellar recession. The video demonstrates the surgical technique for harvesting and preparation of a labial MMG, the splitting of the anterior and posterior lamella of the lid margin, scar tissue release between the lash line and the tarsus, repositioning of the anterior lamella, and anchoring of the labial MMG. Highlights: Anterior lamellar recession combined with MMG wrapping the lid margin and bare tarsus offers good cicatricial entropion repair outcomes. Removal of fat and submucosa from the mucosal graft should be done for better cosmesis. Adequate separation of the scar tissues from the lash line and the tarsus is essential.

Drug-induced Stevens-Johnson syndrome: A case series

Stevens-Johnson syndrome (SJS) can be defined as a rare, serious disorder of the skin and mucous membrane characterized by widespread vesiculobullous rash with epidermal sloughing and necrosis involving mainly eyes, oral cavity, and skin. SJS can be diagnosed if there is <10% of the skin involvement. SJS occurs as an idiosyncratic reaction to various medications. Among them, the most common are antimicrobial agents (AMAs), antiepileptics, and non-steroidal anti-inflammatory drugs (NSAIDs). SJS is one of the dermatological emergencies for which initial treatment can only be supportive like fluids and nasogastric or parenteral feeding and symptomatic measures like analgesic mouth rinse for mouth ulcer. Beyond this, no treatment for SJS is approved. Cases of drug-induced SJS as diagnosed by Skin and VD department were included in the study. Interpretations were drawn out from that data and causality assessment was done according to the WHO-UMC causality assessment. Total four cases of drug-induced SJS were available. two cases of male patients and two of female patients. Out of them, three cases were by NSAIDs induced and one case was anti-epileptic (phenytoin) induced. In the present study, it was found that three of the cases of drug-induced SJS were caused NSAIDs and one case by anti-epileptic. According to the WHO-UMC Causality assessment, three cases were probable and one was unclassified.

Mycoplasma pneumoniae?induced rash and mucositis: A new entity

Mycoplasma pneumoniae is a well-known cause of community-acquired pneumonia, mostly associated with dermatological manifestations especially with mucosal involvement and targetoid cutaneous lesions. For many years, it was considered among the spectrum of erythema multiforme. Recently, some authors have recommended the creation of a new syndrome called “mycoplasma-induced rash and mucositis.” This new syndrome has distinct epidemiological, clinical and histological features making it different from drug-induced Stevens-Johnson syndrome, toxic epidermal necrosis and erythema multiforme. Herein, we report two patients with acute Mycoplasma pneumoniae respiratory tract infection presenting severe mucocutaneous lesions in accordance with this new syndrome.

Comparison of the performance of SCORTEN and ABCD-10 in evaluating the prognosis of Stevens-Johnson syndrome/toxic epidermal necrolysis / 中华皮肤科杂志

Objective:To compare the performance of the severity-of-illness score for toxic epidermal necrolysis (SCORTEN) and ABCD-10 (age, bicarbonate, cancer, dialysis, 10% body surface area) scoring systems in predicting death in patients with Stevens-Johnson syndrome (SJS) /toxic epidermal necrolysis (TEN) .Methods:Clinical data were collected from 85 patients with SJS/TEN who were hospitalized in Sichuan Provincial People′s Hospital from January 2010 to April 2021, and retrospectively analyzed. The predicted mortality and actual mortality were compared at each score level of SCORTEN and ABCD-10. The receiver operating characteristic (ROC) curve and Hosmer-Lemeshow goodness-of-fit test were used to evaluate the predictive power and calibration of SCORTEN and ABCD-10 on mortality.Results:Among the 85 patients, 37 were males and 48 were females, and their ages were 52.36 ± 19.31 years (range, 14 - 88 years) . There were 61 cases of SJS, 6 of SJS/TEN overlap, and 18 of TEN. Ten patients died in hospital and the fatality rate was 11.76%. Among the SCORTEN and ABCD-10 components, age > 40 years or ≥ 50 years, epidermal exfoliation > 10% body surface area on the 1st day after admission, heart rate > 120 beats per minute, serum urea nitrogen level > 10 mmol/L and serum bicarbonate level < 20 mmol/L were significantly correlated with death ( χ2 = 4.46, 6.18, 25.50, 15.13, 7.59, 8.38, respectively, all P < 0.05) , while malignancies, serum glucose level > 14 mmol/L, and pre-hospital dialysis were not significantly correlated with death ( χ2 = 0.35, 0.10, 1.38, respectively, all P > 0.05) . There were no significant differences between the predicted mortality and actual mortality at every score level of SCORTEN and ABCD-10 (all P > 0.05) . The ROC curve showed that both SCORTEN and ABCD-10 had good predictive power for death (areas under the curve: 0.874 and 0.867, 95% CI: 0.758 - 0.990, 0.773 - 0.962, respectively) , but the model goodness-of-fit of SCORTEN was superior to that of ABCD-10 ( P = 0.944, 0.048, respectively) . Conclusion:Both SCORTEN and ABCD-10 scoring systems could accurately predict mortality of SJS/TEN patients at early stage, but SCORTEN showed more favourable predictive power and calibration.

Clinical features and prognosis of eight cases of Mycoplasma pneumoniae-induced rash and mucositis / 中华皮肤科杂志

Objective:To analyze clinical features and prognosis of Mycoplasma pneumoniae-induced rash and mucositis (MIRM) . Methods:Among patients who were diagnosed with erythema multiforme/severe erythema multiforme or Stevens-Johnson syndrome at discharge from the First Affiliated Hospital, Sun Yat-sen University from November 2004 to May 2021, patients with MIRM were screened out according to diagnostic criteria for MIRM and after exclusion of other causes, and their clinical manifestations, laboratory and auxiliary examinations, treatment and prognosis were analyzed.Results:Eight patients were found to meet the MIRM diagnostic criteria, including 4 males and 4 females, with the age at onset being 15.63 ± 9.16 years (range, 4 - 30 years) . All the 8 patients had fever, and 5 of them had upper respiratory symptoms such as cough and sore throat. Oral mucosal damage occurred in all the patients, 5 of whom presented with blood crusts on the lips; eye damage occurred in 7 patients, which manifested as conjunctiva hyperemia and increased secretions. All the patients presented with skin lesions, including 5 with targetoid lesions and 4 with blisters. All the patients were serologically positive for anti- Mycoplasma pneumoniae IgM. One patient experienced recurrent upper respiratory tract infections such as dry cough, each episode was closely related to Mycoplasma pneumoniae infection, and whole exome sequencing of the peripheral blood showed heterozygous mutations in the NLRC4 and IRGM genes. Histopathological examination of skin lesions was performed in 3 patients, and the results were consistent with the diagnosis of erythema multiforme. Seven patients were treated with systemic glucocorticoids, 6 with intravenous immunoglobulin, 5 with azithromycin, and 5 with acyclovir, valacyclovir or ribavirin. After an average 2.9-year follow-up, 3 patients were cured, 1 was blind, 1 experienced recurrent dry cough, oral ulcers and rashes on the limbs, and the remaining 3 developed eye damage such as meibomian gland dysfunction, punctal stenosis and corneal epithelial damage. Conclusions:MIRM mostly occurred in children and young adults, and was mainly accompanied by prodromal symptoms such as fever, sore throat and cough. MIRM mainly manifested as obvious mucosal damage and some targetoid lesions. Most patients could recover after a single attack, and recurrent episodes may be related to mutations in autoinflammation- and infection-related genes in some patients.

Research progress on ocular lesions of Stevens-Johnson syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

Stevens-Johnson syndrome(SJS)refers to a type of adverse reactions of the skin and mucosa mainly caused by drug allergy, which can be life-threatening in serious cases. For SJS survivors, ocular damage are accepted as the most severe complications. In recent years, because of the extensive research of its genetic susceptibility and immune pathogenesis, varied susceptibility genes which are involved in the ocular complications of SJS have been discovered. However, further efforts are still needed to clarify relevant mechanisms due to the high ethnicity and drug specificity. Ocular lesions of SJS are prone to be neglected in the early stage, and patients often receive ophthalmic treatment after a severe visual loss. Especially in China, the ocular surface of most patients has already been seriously damaged when they went to the ophthalmology department, while existing treatments achieve unsatisfactory results in improving visual acuity. Therefore, recent research progress is reviewed in this article on etiology and treatment of SJS ocular lesions, in order to raise clinicians' awareness of ocular complications, and provide references for the diagnosis and treatment of diseases and research in related fields.

Dental management in the Intensive Care Unit in the treatment of toxic epidermal necrolysis associated with phenytoin: case report / Manejo odontológico na Unidade de Terapia Intensiva no tratamento da necrólise epidérmica tóxica associada a fenitoína: relato de caso

ABSTRACT Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are adverse reactions, usually medicine-related, in which extensive and painful mucocutaneous lesions are formed. Oral lesions are characterized by the presence of erosions and ulcers. The treatment usually requires intensive care in a hospital setting, where the Dentist is essential in the multidisciplinary team, as he will act in the treatment of oral lesions. In this article, a 26-year-old female patient was reported, who presented TEN-compatible mucocutaneous lesions associated with the drug Phenytoin and required admission to a Teaching Hospital. The treatment consisted of the immediate suspension of the Phenytoin use and systemic therapy with corticosteroids and immunosuppressants. Oral lesions were monitored by the hospital dentistry team. The procedures adopted were: oral hygiene with 0.12% non-alcoholic Chlorhexidine Digluconate solution, hydration of the oral tissue with an essential fatty acid, application of frozen chamomile tea, and prescription of topical Triamcinolone Acetonide. Also, low-level laser therapy sessions (red and infrared) were performed. Oral lesions evolved with significant improvement. In this case, the importance of the Dentist in the multi-professional team is demonstrated. Although there is no protocol, the procedures used in the present case can be an alternative to therapies used in SSJ and TEN oral lesions.

RESUMO A Síndrome de Stevens Johnson (SSJ) e a Necrólise Epidérmica Tóxica (NET) são reações adversas, geralmente medicamentosas. Na pele, formam-se máculas que se tornam pápulas, vesículas e bolhas, além de eritema extenso e desprendimento da epiderme. As lesões bucais caracterizam-se pela presença de erosões e úlceras dolorosas. O tratamento geralmente é realizado em âmbito hospitalar, com necessidade de cuidados intensivos, onde o Cirurgião-dentista é fundamental na equipe multiprofissional, pois atuará na terapêutica das lesões bucais. Neste artigo foi relatado o caso de uma paciente do sexo feminino, de 26 anos, a qual apresentou lesões mucocutâneas compatíveis com NET associadas ao medicamento Fenitoína e necessitou de internamento em um Hospital Universitário. O tratamento realizado foi a suspensão imediata do fármaco desencadeador, além de terapia sistêmica com corticóide e imunossupressor. As lesões bucais foram acompanhadas pela equipe de odontologia hospitalar. As condutas adotadas foram: higiene bucal com solução de Digluconato de Clorexidina 0,12% não alcoólica, hidratação da mucosa bucal com Ácido Graxo Essencial, aplicação de raspas de chá de gelo de camomila e prescrição de Triancinolona Acetonida tópica. Além disso, foram realizadas sessões de laserterapia de baixa intensidade (vermelho e infravermelho). As lesões bucais evoluíram com melhora significativa. A paciente permaneceu internada durante 22 dias, sendo 12 em Unidade de Terapia Intensiva. Este caso demonstra a importância do Cirurgião-dentista na equipe multiprofissional. Apesar de não haver um protocolo para as lesões bucais, as condutas utilizadas no presente caso podem ser uma alternativa de terapêuticas utilizadas em lesões de SSJ e NET.

Reacciones cutáneas adversas graves en la infancia / Severe adverse skin reactions in the childhood

RESUMEN Las reacciones cutáneas a drogas son cada vez más frecuentes en edades pediátricas, con un alto impacto en la salud de los niños. Pueden manifestarse en formas muy disímiles, desde un exantema transitorio hasta cuadros graves con afectación multisistémica potencialmente fatales. En la presente revisión se hace énfasis en las farmacodermias graves en la infancia, con el objetivo de promover el conocimiento por parte del personal médico para facilitar su diagnóstico y tratamiento oportuno. Se desarrolló una búsqueda en la Biblioteca Virtual de Salud de Infomed y en Google: se revisaron 28 trabajos científicos sin limitación de año y país, 24 de ellos pertenecen a los últimos cinco años y de estos 17 a los últimos tres. El dominio de los elementos para el diagnóstico precoz y las opciones terapéuticas son indispensables para elegir la conducta adecuada frente a estas reacciones cutáneas graves y disminuir la morbimortalidad por estas afecciones (AU).

ABSTRACT Skin reactions to drugs are increasingly common at pediatric ages, with a high impact on children's health. They can appear in very dissimilar forms, from a transient rash to serious pictures with potentially fatal multisystem involvement. This review focuses on severe pharmacodermies in the childhood, with the aim of promoting medical staff knowledge to facilitate their timely diagnosis and treatment. A search was led in the Infomed Virtual Health Library and in Google: 28 scientific papers were reviewed without limitation of year and country, 24 of them belong to the last five years and from these 17 to the last three. Mastery of the elements for early diagnosis and therapeutic options are indispensable to choose the appropriate behavior against these serious skin reactions and to decrease morbidity and mortality due to these conditions (AU).