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.

Clinical features and prognostic factors of severe cutaneous adverse drug reactions: A single-center retrospective study of 209 cases in China.

BACKGROUND: Severe cutaneous adverse drug reactions (SCAR) are life-threatening and contain drug reactions with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis (AGEP). METHODS: We aimed to evaluate clinical features and prognostic factors for SCAR patients. From January 2010 to April 2022, 209 patients with SCAR (DRESS, n = 46, SJS/TEN, n = 128, AGEP, n = 35) were included in this study. Clinical symptoms, laboratory tests, causative drugs, disease courses, treatments, and outcomes were investigated. RESULTS: Antibiotics ranked first (35.9 %) followed by traditional Chinese medicine (15.8 %) and antiepileptic drugs (14.8 %) among causative drugs of SCAR. One patient (2.2 %) with DRESS and seven patients (5.5 %) with SJS/TEN died in the hospital, while there was no AGEP-related mortality. The multivariate logistic regression analysis showed that high Registry of Severe Cutaneous Adverse Reactions score (OR = 2.340, 95 % CI = 1.192-4.591) and hemoglobin < 100 g/L (OR = 0.126, 95 % CI = 0.016-0.983) were independent risk factors of DRESS. Anemia (OR = 0.191, 95 % CI = 0.037-0.984) and body surface area detached involved at day 1 (OR = 2.749, 95 % CI = 1.115-6.778) were independent risk factors of SJS/TEN for severe acute complications and hospital death (P < 0.05). Lymphocytopenia (OR = 0.004, 95 % CI = 0.000-0.553) was a risk factor of AGEP for acute complications (P = 0.028). CONCLUSION: This study reveals the clinical features and independent prognostic factors for SCAR, which may be helpful in the clinical management for SCAR patients.

Fotobiomodulação na Síndrome de Stevens-Johnson no Câncer de Mama Metastático: Relato de Caso / Photobiomodulation in Stevens-Johnson Syndrome in a Metastatic Breast Cancer: Case Report / La Fotobiomodulación en el Síndrome de Stevens-Johnson en el Cáncer de Mama Metastásico: Informe de Caso

Introdução: A síndrome de Stevens-Johnson é uma condição rara e potencialmente fatal que requer diagnóstico precoce e tratamento adequado para garantir bom prognóstico. Em virtude da complexidade da síndrome e da falta de tratamento padrão para as feridas, o uso da fotobiomodulação tem sido discutido. Relato do caso: Mulher, 30 anos, com câncer de mama metastático, portadora das síndromes de Stevens-Johnson e de Li-Fraumeni Like, em uso da fotobiomodulação como estratégia adjuvante no tratamento da dor e das feridas na pele. As feridas cutâneas eritematosas envolveram quase toda a superfície corpórea, com lesões escamosas, crostosas e bolhosas, dolorosas, com pontos hemorrágicos em região posterior de ambos os lóbulos das orelhas e na região occipital, dificultando a acomodação da paciente em decúbito lateral e em posição semirreclinada. Foram realizadas duas aplicações da fotobiomodulação (vermelho, com comprimento de onda de 660 nm) nas regiões occipital e de orelhas, com dose de 2 Joules por ponto; e 4 Joules em região escapular para analgesia (infravermelho, com comprimento de onda de 808 nm). Ambas as aplicações foram seguidas de mobilização e liberação miofascial na região escapular. Em 48 horas, houve regressão das lesões cutâneas e melhora da dor, facilitando posicionamento adequado e indolor no leito. Após 15 sessões de fisioterapia, a paciente recebeu alta hospitalar. Conclusão: O uso da fotobiomodulação se mostrou eficaz para o tratamento complementar da fase aguda da síndrome de Stevens-Johnson no que diz respeito à regeneração tecidual e analgesia.

Introduction: Stevens-Johnson syndrome is a rare but potentially fatal condition, which requires early diagnosis and treatment to ensure good prognosis. Due to the complexity of the syndrome and the lack of a standard wound care treatment, the use of photobiomodulation has been discussed. Case report: A 30-year-old woman with metastatic breast cancer and Stevens-Johnson and Li-Fraumeni Like syndromes using photobiomodulation as an adjuvant strategy in the treatment of pain and skin wounds. The erythematous cutaneous lesions involved almost the entire body surface with painful, scaly, crusted and bullous lesions with bleeding spots in the posterior region of both ears lobes and in the occipital region which made it difficult to accommodate the patient in lateral decubitus and in semi-recumbent position. Two photobiomodulation applications (red, with a wavelength of 660 nm) were performed, where lesions in the occipital region and ears were treated with a dose of 2 Joules per point and 4 Joules in the scapular region for pain relief (infrared, with a wavelength of 808 nm), followed by mobilization and myofascial release in the scapular region. In 48 hours, the cutaneous lesions reduced, and the pain improved, which facilitated the adequate and painless positioning in bed. After 15 physiotherapy sessions, the patient was discharged. Conclusion: Photobiomodulation has been shown to be effective in the complementary treatment of the acute phase of Stevens-Johnson Syndrome regarding tissue regeneration and analgesia.

Introducción: El síndrome de Stevens-Johnson es una condición rara y potencialmente fatal que requiere diagnóstico temprano y tratamiento adecuado para asegurar un buen pronóstico. Debido a la complejidad del síndrome y la falta de un tratamiento estándar de las heridas, se ha discutido el uso de fotobiomodulación. Informe del caso: Mujer, 30 años, con cáncer de mama metastásico, portadora de los síndromes de Stevens-Johnson y Li-Fraumeni Like en uso de la fotobiomodulación como estrategia adyuvante para el tratamiento del dolor y las heridas cutáneas. Las heridas cutáneas eritematosas comprometían casi toda la superficie corporal, con lesiones dolorosas, descamativas, costrosas y ampollosas, con puntos hemorrágicos en la región posterior de ambos lóbulos de las orejas y en la región occipital, que dificultaban la acomodación del paciente en decúbito lateral y en posición cómoda semirrecostada. Se realizaron dos aplicaciones de fotobiomodulación (rojo, con longitud de onda de 660 nm) en región occipital y auricular con dosis de 2 Joules por punto; y 4 Joules en la región escapular para analgesia (infrarrojo, con longitud de onda de 808 nm). Ambas aplicaciones fueron seguidas de movilización y liberación miofascial en la región escapular. En 48 horas hubo una regresión de las lesiones cutáneas y mejoría del dolor, lo que facilitó una adecuada e indolora posición en la cama. Después de 15 sesiones de fisioterapia, la paciente fue dada de alta del hospital. Conclusión: El uso de la fotobiomodulación demostró ser efectivo para el tratamiento complementario de la fase aguda del síndrome de Stevens-Johnson en lo que respecta a la regeneración tisular y la analgesia.

[Metastatic Renal Cancer with Stevens-Johnson Syndrome after Nivolumab Treatment : A Case Report].

A 65-year-old male was admitted to our hospital with an abscess on his buttocks. Computed tomography (CT) on admission incidentally revealed left kidney cancer, and retroperitoneal nephrectomy was performed. Pathological examination confirmed a diagnosis of renal cell carcinoma. One month after surgery, CT showed findings suggesting primary liver cancer or liver metastasis along with retroperitoneal metastasis. Although metastatic kidney cancer was suspected, the possibility of primary liver cancer could not be ruled out. Therefore, we initiated treatment using sorafenib, which is indicated for both types of cancer, as first-line treatment for intermediate-risk based on the International Metastatic Renal Cell Carcinoma Database Consortium (IMDC) risk classification. After three months of sorafenib treatment, the patient showed hyponatremia, anemia, and hand-foot syndrome and was admitted to the hospital. CT showed an enlarged area that appeared to be a metastatic site, after which we suspended sorafenib. Four months after sorafenib treatment, nivolumab was initiated as a second-line treatment. However, on day 28 after the administration of nivolumab eruptions appeared all over the patient's body. The patient was diagnosed with Stevens-Johnson syndrome due to nivolumab. We initiated corticosteroid therapy, and the eruptions gradually improved. Prednisolone was gradually reduced to 5mg/day, after which the patient was discharged. Six months after discharge, the eruptions had generally become epithelialized and no metastatic lesions had grown. The patient remained under observation without proceeding to third-line treatment. It is crucial to carefully monitor the patient's condition, especially in cases involving serious immune-related adverse events.

Stevens-Johnson syndrome/toxic epidermal necrolysis successfully treated with Chinese herbal medicine Pi-Yan-Ning: A case report.

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare adverse cutaneous reaction with a low incidence and high mortality. Despite posing a serious threat to patients' health and lives, there is no high-quality evidence for a standard treatment regimen. Here we report the case of a 62-year-old man with stage IV pancreatic cancer who experienced immunotherapy-induced SJS/TEN. After consensus-based regular treatments at a local hospital, his symptoms became worse. Thus, he consented to receive Chinese herbal medicine (CHM) therapy. The affected parts of the patient were treated with the CHM Pi-Yan-Ning which was applied externally for 20 min twice a day. After 7 days of treatment, the dead skin began peeling away from the former lesions that had covered his hands, feet, and lips, indicating that skin had regenerated. After 12 days of treatment, the patient's skin was completely recovered. In this case, SJS/TEN was successfully treated with Pi-Yan-Ning, suggesting that there might be tremendous potential for the use of Pi-Yan-Ning in the treatment of severe skin reactions to drug treatments. Further basic investigations and clinical trials to explore the mechanism and efficacy are needed.

Hyperbaric oxygen treatment for toxic epidermal necrolysis: A case report.

Toxic epidermal necrolysis (TEN) is a potentially life-threatening muco-cutaneous disease, largely caused by an idiosyncratic reaction to medication or infectious disease, and is characterised by acute necrosis of the epidermis. No definitive consensus regarding the treatment of TEN has been agreed. A 60-year-old woman, diagnosed with multiple myeloma three months prior, was admitted with signs of TEN to the intensive care burns unit. She had been given ciprofloxacin to treat a urinary tract infection. She complained of malaise and pain, with maculopapular and bullous eruptions over her whole body on the third day of ciprofloxacin administration. Her supportive cares included intravenous immunoglobulins, pain control with analgesics, wound care, nutrition, and fluid support. Hyperbaric oxygen treatment (HBOT) was added on the second day of admission. The patient underwent 5 sessions of HBOT at 243.1 kPa (2.4 atmospheres absolute). Desquamation was noted to stop after the first session of HBOT and re-epithelisation commenced rapidly. The patient was discharged from the burn unit after 14 days of hospital admission. Improvement in this case was temporally related to the initiation of HBOT.

Stevens-Johnson syndrome/toxic epidermal necrolysis successfully treated with Chinese herbal medicine Pi-Yan-Ning: A case report / 中西医结合学报

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare adverse cutaneous reaction with a low incidence and high mortality. Despite posing a serious threat to patients' health and lives, there is no high-quality evidence for a standard treatment regimen. Here we report the case of a 62-year-old man with stage IV pancreatic cancer who experienced immunotherapy-induced SJS/TEN. After consensus-based regular treatments at a local hospital, his symptoms became worse. Thus, he consented to receive Chinese herbal medicine (CHM) therapy. The affected parts of the patient were treated with the CHM Pi-Yan-Ning which was applied externally for 20 min twice a day. After 7 days of treatment, the dead skin began peeling away from the former lesions that had covered his hands, feet, and lips, indicating that skin had regenerated. After 12 days of treatment, the patient's skin was completely recovered. In this case, SJS/TEN was successfully treated with Pi-Yan-Ning, suggesting that there might be tremendous potential for the use of Pi-Yan-Ning in the treatment of severe skin reactions to drug treatments. Further basic investigations and clinical trials to explore the mechanism and efficacy are needed.

Fotobiomodulação na síndrome de stevens-johnson: relato de caso / Photobiomodulation for stevens-johnson syndrome: case report

Introdução: A Síndrome Stevens-Johnson (SSJ) é uma doença causada por hipersensibilidade a imunocomplexos e pode ser desencadeada por distintos fármacos, dentre eles a fenitoína. Devido sua complexidade e raridade, ainda nãohá consenso de tratamento padrão ouro, porém sabese da necessidade da atuação multidisciplinar. Para os cuidados com as feridas, pode-se citar os curativo se a fotobiomodulação (FBM). Objetivo: Relatar o uso da FBM como terapia complementar em um caso de SSJ no Hospital Universitário Regional dos Campos Gerais (HU-UEPG). Métodos: Paciente sexo feminino, 26 anos, deu entrada na unidade de terapia intensiva (UTI) com diagnóstico de SSJ secundária ao uso de fenitonína, escore de SCORTEN 1, com área sem epitélio íntegro 10- 30% e área acometida por lesões de 94,5%, poupando apenas o couro cabeludo. Foi abordada e tratada por uma equipe multidisciplinar e solicitado vaga em centro de especializado em queimados. No sétimo dia de UTI foi iniciado tratamento com FBM, 2 J por ponto, distância entre pontos de 2cm, comprimento onda vermelho (660nm), nas feridas que não apresentavam secreção, foram cinco sessões com intervalo de três dias entre a terceira e a quarta. Resultados: A paciente apresentou melhora visível das lesões cutâneas e recebeu alta hospitalar 5 dias após cessação da FBM. Conclusão: O uso da FBM pode ser efetiva no tratamento complementar da fase aguda SSJ desencadeada por fenitoína.

Introduction: Stevens-Johnson Syndrome (SJS) is a disease caused by hypersensitivity to immune complexes and can be triggered by different drugs, including phenytoin. Due to its complexity and rarity, there is still no consensus on gold standard treatment, but the need for multidisciplinary action is known. For wound care, dressings and photobiomodulation (PBM) can be mentioned. Objective: This study is to report the use of PBM as complementary therapy in a case of SJS at Hospital Universitário Regional dos Campos Gerais (HU-UEPG). Methods: A 26-year-old female patient was admitted to the intensive care unit (ICU) diagnosed with SJS secondary to the use of phenytoin, SCORTEN score 1, with an area without intact epithelium 10-30% and an area affected by injuries of 94.5 %, saving only the scalp. She was approached and treated by a multidisciplinary team which requested a place in a specialized burn center. On the seventh day of ICU, treatment with PBM, 2J per point was started, distance between points of 2cm, red wave length (660nm), in wounds that did not present secretion, with a total of five sessions with an interval of three days between the third and fourth. Results: The patient showed a visible improvement of skin lesions and was discharged from hospital 5 days after cessation of PBM. Conclusion: Use of PBM can be effective in complementary treatment of acute SJS phase triggered by phenytoin.

Vajra Bhasma, Ayurvedic medicine: a rare and unusual cause of Lyell's syndrome and its successful management.

Toxic epidermolysis necrosis (TEN) or Lyell syndrome is a potentially life-threatening immunological adverse skin disease, which mostly occurs secondary to the intake of an offending drug. It commonly manifests as a widespread exfoliating bullous lesion in skin and mucous membrane mimicking superficial burns and may result in hypovolemic and/or septic shock. Authors report an unusual case of Lyell's syndrome in a 42-year-old woman, secondary to the intake of Ayurveda medicine 'Vajra Bhasma' (Diamond Ash) prescribed by an Ayurveda physician for treatment of her trigeminal neuralgia. After 8th day of continuous medication intake, she had prodromal illness and rapidly developing exfoliative skin lesion extended over 80% of total body surface area, breathing difficulty, dizziness and anuria. The case was successfully managed by timely diagnosis, adequate hydration and administration of immunoglobulins. After 17 days, the skin epithelium regenerated, and she improved clinically with some depigmented lesions at discharge, which were normalised without any sequel during her further follow-up visits in hospital. Identification and withdrawal of the suspected drug, adequate resuscitation and early immunoglobulin administration are critical in management of TEN.

General characteristics, economic burden, causative drugs and medical errors associated with medical damage litigation involving severe cutaneous adverse drug reactions in China.

WHAT IS KNOWN AND OBJECTIVE: To investigate the general characteristics, economic burden, causative drugs and medical errors associated with litigation involving severe cutaneous adverse drug reactions (SCADRs) in China, with the aims of improving rational medication use and reducing the extent of damage from SCADRs. METHODS: This study analysed 150 lawsuit judgements involving SCADRs from 2005 to 2019, collected from China Judgments Online. RESULTS AND DISCUSSION: In total, 50% of lawsuits stemmed from SCADRs occurring in general hospitals. The average time elapsed from the date of occurrence of the SCADRs to the end of litigation procedures was 1055 days. Of the patients involved, 51% were female and more than two thirds (69%) were under 60 years old. The most common outcome of SCADRs was death (39%), followed by disabilities (30%). The average responsibility of the medical provider was 48 ± 29%. The average amount of compensation was $43 424. Of the cases studied, 51% of SCADRs were Stevens-Johnson syndrome or toxic epidermal necrolysis, which together accounted for 75% of cases with known clinical subtype. The overall average economic burden of SCADRs was $99 178, of which indirect costs made up the largest proportion (more than 60%). The most common causative drug groups were antimicrobial drugs (49%), Chinese patent medicine and Chinese herbal medicine (17%), and antipyretic analgesics (16%). Finally, 61% of medical errors were found to stem from violation of duty of care, 20% from violation of informed consent and 18% from violations related to the medical record writing and management system. WHAT IS NEW AND CONCLUSION: Severe cutaneous adverse drug reactions not only severely affect patient survival and quality of life, but also impose a heavy economic burden in terms of health care and societal costs. Medical providers should be better educated on strategies to reduce risk to patients and establish mechanisms of risk sharing and management.

Toxic Epidermal Necrolysis and Steven-Johnson Syndrome: A Comprehensive Review.

Significance: Toxic epidermal necrolysis (TEN) and Steven-Johnson syndrome (SJS) are potentially fatal acute mucocutaneous vesiculobullous disorders. Evidence to date suggests that outcomes for patients with both TEN and SJS are largely dependent on stopping the causative agent, followed by supportive care and appropriate wound management in a specialized burns unit. These are life-threatening conditions characterized by widespread full-thickness cutaneous and mucosal necrosis. This article outlines the approach to holistic management of such patients, in a specialized unit, highlighting various practical aspects of wound care to prevent complications such as infection, mucosal and adhesions, and ocular scaring. Recent Advances: There is improved understanding of pain and morbidity with regard to the type and frequency of dressing changes. More modern dressings, such as nanocrystalline, are currently favored as they may be kept in situ for longer periods. The most recent evidence on systemic agents, such as corticosteroids and cyclosporine, and novel treatments, are also discussed. Critical Issues: Following cessation of the culprit trigger, management in a specialized burns unit is the most important management step. It is now understood that a multidisciplinary team is essential in the care of these patients. Following admission of such patients, dermatology, ear, nose, and throat surgery, ophthalmology, urology, colorectal surgery, and gynecology should all be consulted to prevent disease sequelae. Future Directions: Looking forward, research is aimed at achieving prospective data on the efficacy of systemic immunomodulating agents and dressing types. Tertiary centers with burns units should develop policies for such patients to ensure that the relevant teams are consulted promptly to avoid mucocutaneous complications.

Retrospective study of 213 cases of Stevens-Johnson syndrome and toxic epidermal necrolysis from China.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe adverse drug reactions with high mortality. The use of corticosteroids and the management of complications (e.g. infection) in SJS/TEN remains controversial. METHODS: A retrospective study was performed among 213 patients with SJS/TEN who were hospitalized in our department between 2008 and 2018, to investigate the causative agents, clinical characteristics, complications, and prognoses of SJS/TEN mainly treated by systemic corticosteroids combined with intravenous immunoglobulin (IVIG). RESULTS: The causative drugs of SJS/TEN in these patients mainly consisted of antibiotics (61/213, 28.6%), anticonvulsants (52/213, 24.4%), and nonsteroidal anti-inflammation drugs (24/213, 11.3%), among which carbamazepine was the most frequently administered drug (39/213, 18.3%). There were significant differences in the maximum dosage, time to corticosteroid tapering, and the total dosage of corticosteroid between the SJS group and the TEN group, as well as among the three groups (P = 0.000), whereas in the initial dose of corticosteroid was not statistically significant among the three groups (P = 0.277). In a series of 213 cases, 18.4 cases (8.6%) were expected to die based on the score for the toxic epidermal necrolysis (SCORTEN) system, whereas eight deaths (3.8%) were observed; the difference was not statistically significant (P = 0.067; SMR = 0.43, 95% CI: 0.06, 0.48). The most common complications were electrolyte disturbance (174/213, 81.7%), drug-induced liver injury (64/213, 30.0%), infection (53/213, 24.9%), and fasting blood sugar above 10 mmol/L (33/213, 15.5%). Respiratory system (22/213, 10.3%) and wound (11/213, 5.2%) were the most common sites of infection. Multivariate logistic regression analysis indicated that the maximum blood sugar (≥10 mmol/L), the time to corticosteroid tapering (≥12 d), the maximum dosage of corticosteroid (≥1.5 mg/kg/d), and the total body surface area (TBSA) (≥10%) were defined as the most relevant factors of the infection. CONCLUSION: The mortality of patients in this study was lower than that predicted by SCORTEN, although there was no significant difference between them. Hyperglycemia, high-dose corticosteroid, and the TBSA were closely related to the infections of patients with SJS/TEN.

Lamotrigine and its relationship with Stevens-Johnson syndrome and toxic epidermal necrolysis / Lamotrigina y su relación con el síndrome de Stevens-Johnson y la necrólisis epidérmica tóxica

Lamotrigine is an antiepileptic drug that has been widely used for epilepsy, as a mood stabilizer (for type 1 bipolar disorder) and in the management of neuropathic pain, it is used both in monotherapy and in complementary therapy. Considered relatively new, approved by the Food and Drug Administration in 1994, its benefits include a greater margin of safety compared to other anticonvulsants. However, although in a lower percentage, it causes severe adverse skin reactions such as Stevens-Johnson syndrome and toxic epidermal necrolysis. A review is made about the probable pathways that trigger this delayed hypersensitivity immune response.

La lamotrigina es un fármaco anticonvulsivo que ha sido utilizado ampliamente para tratar la epilepsia, como estabilizador del ánimo (en casos de trastorno bipolar tipo 1) y en el manejo del dolor neuropático; se usa tanto en monoterapia como en terapia complementaria. Considerado como un medicamento relativamente nuevo, aprobado por la Food and Drug Administration en 1994, dentro de sus beneficios se encuentra un mayor margen de seguridad en comparación con otros anticonvulsivos; sin embargo, aunque en menor porcentaje, es causa de reacciones cutáneas adversas graves, como el síndrome de Stevens-Johnson y la necrólisis epidérmica tóxica. En el presente estudio se realiza una revisión de las probables vías que desencadenan esta respuesta inmunitaria de hipersensibilidad tardía.

[Recalcitrant cicatrizing conjunctivitis. Treatment of nine patients with rituximab]. / Conjuntivitis cicatrizal recalcitrante. Tratamiento de nueve pacientes con rituximab.

Cicatrizing conjunctivitis is the final consequence of several diseases. The most severe among them are cicatricial pemphigoid and chronic Stevens-Johnson syndrome. Systemic immunosuppressive drugs and steroids are usually an effective approach to these diseases. However, a few patients follow a recalcitrant course unremitting to usual therapy. We describe the treatment with rituximab of seven patients with cicatricial pemphigoid and two with chronic Stevens-Johnson syndrome. Eight of them also received gammaglobulin and all achieved clinical remission. Three relapsed and required two or three new courses of rituximab with good control of disease activity. Rituximab proved to be an efficacious drug for chronic recalcitrant cicatrizing conjunctivitis.

La conjuntivitis cicatrizal es la consecuencia de distintas enfermedades oculares. Entre ellas, las más graves son el penfigoide cicatrizal y el síndrome de Stevens-Johnson crónico. El tratamiento de estas enfermedades con corticoides e inmunosupresores es habitualmente exitoso, pero unos pocos pacientes siguen un curso recalcitrante. En los últimos años se introdujo el uso de rituximab, asociado o no a gammaglobulina endovenosa, en forma abierta, para el control de la inflamación conjuntival. Describimos aquí el tratamiento de siete pacientes con penfigoide y dos con Stevens-Johnson recalcitrante, con rituximab. Ocho recibieron también gammaglobulina y todos alcanzaron la remisión de la actividad. Tres recayeron y recibieron dos o tres nuevos cursos de la medicación con mejoría sintomática. El rituximab probó ser una droga efectiva para el tratamiento de la conjuntivitis cicatrizal crónica recalcitrante.

Conjuntivitis cicatrizal recalcitrante: Tratamiento de nueve pacientes con rituximab / Recalcitrant cicatrizing conjunctivitis. Treatment of nine patients with rituximab

La conjuntivitis cicatrizal es la consecuencia de distintas enfermedades oculares. Entre ellas, las más graves son el penfigoide cicatrizal y el síndrome de Stevens-Johnson crónico. El tratamiento de estas enfermedades con corticoides e inmunosupresores es habitualmente exitoso, pero unos pocos pacientes siguen un curso recalcitrante. En los últimos años se introdujo el uso de rituximab, asociado o no a gammaglobulina endovenosa, en forma abierta, para el control de la inflamación conjuntival. Describimos aquí el tratamiento de siete pacientes con penfigoide y dos con Stevens-Johnson recalcitrante, con rituximab. Ocho recibieron también gammaglobulina y todos alcanzaron la remisión de la actividad. Tres recayeron y recibieron dos o tres nuevos cursos de la medicación con mejoría sintomática. El rituximab probó ser una droga efectiva para el tratamiento de la conjuntivitis cicatrizal crónica recalcitrante.

Cicatrizing conjunctivitis is the final consequence of several diseases. The most severe among them are cicatricial pemphigoid and chronic Stevens-Johnson syndrome. Systemic immunosuppressive drugs and steroids are usually an effective approach to these diseases. However, a few patients follow a recalcitrant course unremitting to usual therapy. We describe the treatment with rituximab of seven patients with cicatricial pemphigoid and two with chronic Stevens-Johnson syndrome. Eight of them also received gammaglobulin and all achieved clinical remission. Three relapsed and required two or three new courses of rituximab with good control of disease activity. Rituximab proved to be an efficacious drug for chronic recalcitrant cicatrizing conjunctivitis.

Miliaria crystallina secondary to herbal remedies-induced toxic epidermal necrolysis: A case report.

Miliaria crystallina is a skin disorder that often erupts in the process of febrile diseases or under hot and humid climatic conditions. Toxic epidermal necrolysis (TEN) is a rare, acute, and life-threatening mucocutaneous disease with a mortality rate of 25-35%. There has been no inevitable connection between the two diseases among previously reported cases, but we observed a case of secondary miliaria crystallina a woman with herbal remedies-induced TEN during the therapeutic process.

Treatment of oral manifestations of toxic epidermal necrolysis with low-level laser therapy in a pediatric patient.

Drug-induced reactions are complications associated with high mortality and significant morbidity. Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are examples of these conditions, which are characterized by skin and mucous lesions. Here, we report a case of a 9-year-old girl who presented with blisters associated with an extensive vesicular rash and multiple ulcerations on the lips and oral cavity. A drug-induced hypersensitivity reaction to antibiotics was suspected, and a diagnosis of TEN was made. The patient was managed with withdrawal of the suspected causative agent, and the oral lesions were treated with low-level laser therapy (LLLT) and oral hygiene. This case highlights that TEN requires interdisciplinary intervention with dental assistance and follow-up to improve symptoms, nutrition, systemic condition, and quality of life.

Terapia de fotobiomodulação no tratamento das lesões orais da síndrome de Stevens-Johnson: relato de caso / Photobiomodulation therapy in the treatment of Stevens-Johnson syndro-me oral lesions: case report

Introdução: A Síndrome de Stevens-Johnson é uma reação rara que se manifesta por exantema que evolui para um extenso deslocamento epidérmico. Na maioria dos casos, há o surgimento de lesões dolorosas em mucosa oral, que podem reduzir a ingestão de nutrientes e dificultar a higiene bucal. A terapia de fotobiomodulação pode ter um importante papel antálgico, na modulação da inflamação e na cicatrização dessas lesões. Objetivo: Relatar um caso clínico no qual as lesões orais da Síndrome de Stevens-Johnson foram tratadas com o auxílio da terapia de fotobiomodulação. Relato de caso: Paciente de 17 anos, do sexo feminino, com diagnóstico de síndrome de Stevens-Johnson, apresentava-se com edema labial, úlceras orais, febre e odinofagia intensa, com tolerância apenas para dieta líquida. Encontrava-se com redução da ingestão alimentar, sem comunicação oral eficiente e com interrupção da higiene bucal devido a dor intensa. A terapia de fotobiomodulação foi utilizada no tratamento das lesões orais. Conclusão: A terapia de fotobiomodulação auxiliou na redução da dor, favoreceu a ingestão de alimentos por via oral e reduziu o tempo de cicatrização das lesões.

Introduction: Stevens-Johnson Syndrome is a rare reaction manifested by a rash that evolves into extensive epidermal dislocation. In most cases there are painful oral mucosal lesions that can reduce nutrient intake and make oral hygiene difficult. Photobiomodulation therapy can play an important antalgic role in modulating inflammation and healing these lesions. Objective: To report a clinical case in which Stevens-Johnson Syndrome oral lesions were treated with the aid of photobiomodulation therapy. Case Report: A 17-year-old female patient diagnosed with Stevens-Johnson syndrome presented with lip edema, oral ulcers, fever, and severe odynophagia, tolerating only a liquid diet. She experienced reduced food intake, no efficient oral communication, and an interruption of oral hygiene due to intense pain. Photobiomodulation therapy was requested to treat the oral lesions. Conclusion: Photobiomodulation therapy helped reduce pain, favored oral food intake, and reduced lesion healing time.