Dermatologic manifestations of multisystem inflammatory syndrome in children during the COVID-19 pandemic

Abstract Objectives: multi-system ınflammatory syndrome in children (MIS-C) is an immune-mediated process that develops after infections like SARS-CoV-2. The authors aimed to reveal the mucocutaneous findings of patients diagnosed with MIS-C at presentation and evaluate the frequency of these mucocutaneous findings and their possible relationship with the severity of the disease. Methods: A prospective study was conducted of 43 children admitted to a tertiary hospitals between January 2021 and January 2022 who met Centers for Disease Control and Prevention criteria for MIS-C. Results: 43 children (25 [58.1%] male); median age, 7.5 years [range 0.5-15 years]) met the criteria for MIS-C. The most common symptom was cutaneous rash 81.4%, followed by gastrointestinal symptoms 67.4%, oral mucosal changes 65.1%, and conjunctival hyperemia 58.1%. The most common mucosal finding was fissured lips at 27.9%, diffuse hyperemia of the oral mucosa at 18.6%, and strawberry tongue at 13.9%. Urticaria (48.8%) was the most common type of cutaneous rash in the present study's patients. The most common rash initiation sites were the trunk (32.6%) and the palmoplantar region (20.9%). The presence or absence of mucocutaneous findings was not significantly associated with disease severity. Study limitations: The number of patients in the this study was small. Conclusions: The present study's prospective analysis detected mucocutaneous symptoms in almost 9 out of 10 patients in children diagnosed with MIS-C. Due to the prospective character of the present research, the authors think that the characteristic features of cutaneous and mucosal lesions the authors obtained will contribute to the literature on the diagnosis and prognosis of MIS-C.

Skin, mucosa and nail findings in hospitalized pediatric patients with Coronavirus disease-2019 (COVID-19)

Abstract Background: Cutaneous manifestations of Coronavirus Disease-2019 (COVID-19) disease have not yet been fully described in hospitalized pediatric patients. Objectives: This prospective study aims to demonstrate the skin, mucosal, and nail findings of hospitalized children with COVID-19. Methods: The authors included hospitalized pediatric patients. Two dermatologists examined skin, hair, nails, and mucosa. Patients with drug eruptions were excluded with an anamnesis, clinical and laboratory test results. Results: Out of 46 enlisted patients, 19 (41,3%) patients displayed skin, mucosal or nail findings. Skin findings were seen on 14 (30.4%) patients. Ten (22%) patients presented skin findings matching described patterns. Half of the patients with patterned rashes had confluent erythematous/maculopapular/morbilliform rashes. Eleven out of 46 (23.9%) patients developed periorbital erythema and edema. Ten (22%) patients had at least one oral mucosal finding. One telogen effluvium, one blue nail, and one flag sign on nails were noticed. Nine (19.5%) patients out of 46 had developed MIS-C. MIS-C patients had mucocutaneous manifestations except one (88.8%). Study limitations: The authors have detected a higher rate of mucocutaneous manifestations compared to out-patients with mild COVID-19 because the study is based on hospitalized patients only. Conclusions: Pediatric COVID-19 patients are more susceptible to developing mucocutaneous manifestations compared to adults. The authors propose COVID-19 should be acknowledged as one of the viral exanthem rashes of childhood. The authors noticed that the most common findings were periorbital erythema and edema. The confluent erythematous/maculopapular/morbilliform rashes appear to be the most common patterns associated with severe COVID-19.

Oral lesions after infection by SARS CoV 2: two case reports / Lesiones bucales posteriores a infección por SARS CoV 2 presentación de dos casos

Introduction: The SARS CoV 2 infection has resulted in several health, economic, and social crises in all areas. The disease shows a substantial biological diversity in humans causing a series of sequels in the trans- or post-infection period in the entire organism. Case Report: The manifestations that occur in the oral cavity and pharynx have not been evaluated. In this study, two clinical cases are reported. The first patient, a 67-year-old male, presents erosive lesions on the dorsal surface of his tongue after SARS CoV 2 infection. Results: Therapy consisting of reinforcing oral cleaning, use of antifungal solutions, mouthwashes containing superoxidation solution and B complex was given to the patient. The reported lesions improved satisfactorily. The second case, a 47-year-old male patient, presented vesiculobullous lesions on the lingual and labial mucosa accompanied by severe painful symptoms after SARS CoV 2 infection. An incisional biopsy was performed. The histopathological result was compatible with pemphigus vulgaris, and the treatment protocol was started with 0.1% topical mometasone and 2g miconazole gel, observing adequate involution of the lesions after 20 days. Conclusions: The aim of this study is to report on the lesions affecting the oral cavity and pharynx in post-COVID patients with the aim of carrying out a thorough intraoral examination, establishing a clinical or histopathological diagnosis to implement a specific treatment plan in each case to improve the health and quality of life of the patients. Keywords: SARS-CoV-2; Oral manifestations; Oral ulcer; Pemphigus; Mouth; Mucous membrane.

Introducción: La infección por virus de SARS CoV 2 ha dejado a su paso una estela de crisis en materia de salud, económica, social y en todos los ámbitos a la fecha seguimos realizando la observación del comportamiento de la enfermedad en los seres humanos con una diversidad biológica importante y que ha traído como consecuencia una serie de secuelas que se presentan en el periodo trans o posterior a la infección en toda la economía corporal. Reporte de Caso: Se ha evaluado poco las manifestaciones que se presentan en la cavidad bucal y faringe; se presentan dos casos clínicos el primero paciente masculino de 67 años de edad posterior a la infección por SARS CoV 2 presenta diluciones de continuidad en bordes laterales de la lengua se indica terapia y refuerza limpieza bucal, antimicótico, colutorios con solución de superoxidación y complejo B, las úlceras involucionan de manera satisfactoria. Resultados: El segundo caso masculino de 47 años posterior a la infección por SARS CoV 2 debuta con lesiones vesículo-ampollosas en mucosa lingual, labial con sintomatología dolorosa severa, se realiza biopsia incisional donde el resultado histopatológico es compatible con pénfigo vulgar, se inicia protocolo de tratamiento con mometasona tópica al 0.1% y miconazol gel 2g observándose una adecuada involución de las lesiones a los 20 días. Conclusiones: El objetivo de este trabajo es poner en contexto de la comunidad médica y científica las lesiones concernientes a la cavidad bucal y faringe que están presentando los pacientes postcovid con el objetivo de realizar una exhaustiva exploración intraoral, establecer un diagnóstico clínico o histopatológico y con base en esto instaurar un plan de tratamiento específico en cada caso en particular con el fin fundamental de mejorar la salud y calidad de vida del paciente.

Clinical efficacy of moxibustion for ulcerative colitis and its influence on vitamin D receptor / 针灸推拿医学（英文版）

Objective: To observe the clinical efficacy of herbal cake-partitioned moxibustion for ulcerative colitis (UC) and elucidate its mechanism by targeting the vitamin D receptor (VDR) signaling pathway. Methods: A total of 63 patients with UC were randomly divided into an observation group (30 cases, treated with herbal cake-partitioned moxibustion) and a control group (33 cases, treated with sham herbal cake-partitioned moxibustion). Moxibustion treatment was performed at Qihai (CV6) and bilateral Tianshu (ST25) and Shangjuxu (ST37), 3 times per week for 12 weeks. The total effective rate, visual analog scale (VAS) score for abdominal bloating and pain, and hospital anxiety and depression scale (HADS) score were compared between the two groups. Enzyme-linked immunosorbent assay was used to detect the concentrations of serum C-reactive protein (CRP), 25-hydroxyvitamin D [25(OH)D], and interleukin-12 (IL-12)/interleukin-23 (IL-23) p40. Immunohistochemistry was used to observe the expression levels of VDR and regenerating gene Ⅳ (Reg Ⅳ) proteins in colonic mucosa. The expression levels of VDR, cytochrome p45027B1 (CYP27B1), and Reg Ⅳ mRNAs were detected by real-time fluorescence quantitive polymerase chain reaction. Results: After treatment, the total effective rate in the observation group was 86.7％, which was significantly higher than 51.5％ in the control group (P<0.05). After treatment, the VAS scores for abdominal bloating and pain in the observation group were significantly decreased (P<0.01), as well as the HADS-depression subscale (HADS-D) and HADS-anxiety subscale (HADS) scores (P<0.05), while only the VAS score for abdominal pain in the control group was reduced (P<0.05), and the improvements of the scores in the observation group were more significant than those in the control group (P<0.05). After treatment, the serum CRP concentrations in both groups and the IL-12/IL-23 p40 concentration in the observation group were significantly decreased (P<0.05), and the concentrations in the observation group were lower than those in the control group (P<0.05). The expression levels of VDR protein and mRNA in the colon in both groups were all increased (P<0.01), and the expression levels of Reg Ⅳ protein and mRNA and CYP27B1 mRNA were all decreased in the two groups (P<0.05 or P<0.01); the improvements in the observation group were more notable than those in the control group (P<0.05 or P<0.01). Conclusion: Herbal cake-partitioned moxibustion can effectively alleviate abdominal pain and diarrhea in patients with UC, improve depression and anxiety disorders, and regulate the expression of related proteins in the VDR signaling pathway. The mechanism may be related to inhibiting intestinal inflammation by reducing the release of the proinflammatory cytokine IL-12/IL-23 p40.

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.

Assessment of dynamic thiol-disulfide homeostasis in patients with lipoid proteinosis (Urbach-Wiethe syndrome)

SUMMARY OBJECTIVE: Lipoid proteinosis is a rare autosomal recessive genetic dermatological disease that occurs due to the accumulation of hyaline material in the skin and mucous membranes. This study aimed to investigate whether dynamic thiol-disulfide homeostasis is a new marker of oxidative stress in patients suffering from lipoid proteinosis. METHODS: The study group involved 17 patients with lipoid proteinosis and 17 healthy controls with same gender and age. Native thiol, total thiol, disulfide levels, and thiol-disulfide indexes were measured with the fully automated spectrophotometric method described by Erel and Neselioglu, and the results of the two groups were statistically analyzed. RESULTS: Serum total thiol and native thiol levels were significantly lower in lipoid proteinosis group compared to the control group (p=0.020 and p=0.014, respectively). The disulfide levels were found to be higher in lipoid proteinosis group, but there was no significant difference between two groups. CONCLUSIONS: Impaired dynamic thiol-disulfide homeostasis was observed in lipoid proteinosis patients, suggesting that thiol-disulfide homeostasis may have a role in the pathogenesis of this disease.

Ocular cicatricial pemphigoid. From a rheumatology and ophthalmology point of view / Penfigoide ocular cicatrizal, enfoque diagnóstico y terapéutico integral entre el oftalmólogo y el reumatólogo

ABSTRACT Ocular cicatricial pemphigoid (OCP) is a chronic, immune-mediated, bullous, cicatricial disease within the spectrum of mucocutaneous membranous pemphigoids (MMP). Although the diagnosis is often ophthalmological, due to the autoimmune nature of the pathology, it requires a joint approach with rheumatologists and immunologists. The objective of this narrative review was to explore the evidence available in the literature from 2000 to 2020 with respect to clinical manifestations, diagnosis, and treatment. The clinical presentation varies widely, from mild cases with slow progression of years of progression, to severe cases with a torpid and rapidly progressive evolution to fibrosis, refractory to multiple treatments. A com plete evaluation of the patient will help guide the diagnosis. The gold standard for diagnosis is conjunctival biopsy with direct immunofluorescence, although on occasions it can be reached if the symptoms are characteristic. Treatment is local and systemic according to its severity and evolution. The evidence on topical and systemic therapeutics is obtained mainly from uncontrolled observational and experimental studies. Immunomodulatory therapy has made it possible to preserve vision and, in many cases, prevent sequelae. The evolu tion is linked to the early diagnosis and immunosuppressive treatment, so it is essential to be aware of this disease, the diagnostic methods, as well as the immunomodulating and immunosuppressive therapies available.

RESUMEN El penfigoide ocular cicatrizal (POC) es una enfermedad crónica, inmunomediada ampollar, mucosinequiante, comprendida dentro del espectro de penfigoides membranosos mucocutáneos (PMM). El diagnóstico es, con frecuencia, oftalmológico, pero debido al carácter autoinmune de la patología, requiere el abordaje en conjunto con reumatólogos e inmunólogos. El objetivo de esta revisión narrativa fue explorar la evidencia disponible en la literatura, desde el año 2000 hasta el 2020, en lo que respecta a sus manifestaciones clínicas, diagnóstico y tratamiento. La presentación clínica varía ampliamente, desde casos leves con progresión lenta de años de evolución hasta casos severos con evolución tórpida y rápidamente progresiva a la fibrosis, refractarios a múltiples tratamientos. Una evaluación completa del paciente ayudará a guiar el diagnóstico. El estándar de oro diagnóstico es la biopsia conjuntival con inmunofluorescencia directa, si bien en ocasiones puede diagnosticarse por la clínica característica. El tratamiento es local y sistêmico de acuerdo con su severidad y evolución. En los últimos 20 anos, la evidencia sobre los tratamientos tópicos y sistêmicos corresponde en su mayoría a estudios observacionales y experimentales no controlados. Los métodos de tratamiento inmunomoduladores han permitido preservar la visión y, en muchos casos, prevenir secuelas. La evolución está ligada al diagnóstico temprano y a los tratamientos disponibles, por lo que es fundamental el conocimiento de esta patología, los métodos diagnósticos y los tratamientos inmunomoduladores e inmunosupresores.

Combined Therapy of Lung and Intestine in Treatment of Lung Diseases： A Review / 中国实验方剂学杂志

The theoretical origin of the combined therapy of lung and intestine can be traced back to the Inner Canon of Huangdi （《黄帝内经》）， which explains the physiological and pathological interaction between the lung and the large intestine. In recent years， researchers have investigated the scientific essence of the "lung-intestine axis" theory from many aspects， which enriches the relevant theoretical basis， and applied it to the treatment of COVID-19， acute lung injury， and other lung diseases. The close relation between lung and intestine in many aspects embodies the holistic conception of traditional Chinese medicine and explains the holistic theory of interrelation between organs， which correlate to each other physiologically and pathologically. Intestinal microecological disorders can affect lung immune function and cause respiratory diseases， and respiratory diseases are usually accompanied by gastrointestinal symptoms. Lung diseases can be prevented and treated by regulating intestinal flora. According to histoembryology， the epithelial tissue of the lung and intestine comes from primitive foregut. In immunology， both lung and intestine contain mucosa-associated lymphoid tissue， and the pathological changes of the respiratory tract are also closely related to intestinal microorganisms. The tissue origin of lung and large intestine， the correlation of mucosal immunity， and the synchronization of ecological changes provide a scientific basis for the combined therapy of lung and intestine. Therefore， this paper summarizes the theoretical origin， modern research mechanism， and clinical application of combined therapy of lung and intestine， in order to provide a new direction for its application in clinical and scientific research.

Alternative Oral Mucous Membrane Pemphigoid Therapy Using Tacrolimus: A Case Report

ABSTRACT: Mucous membrane pemphigoid (MMP) is a blistering disease that predominantly involves the mucous membranes and that can lead to major negative impacts on patient quality of life. The first-line MMP treatment is based on the use of topical and systemic corticosteroids. In this report, we presented a 45-year-old female patient presented blisters in the inferior gingiva for over 8-months. The patient reported being allergic to corticosteroids. Under the clinical hypothesi s of oral lichen planus and MMP, an incisional biopsy was performed, and the histopathological diagnosis of MMP was established. Thus, it was instituted an alternative therapy with tacrolimus 0.03 %. The patient showed an excellent clinical outcome with no recurrence five months after the end of therapy. Tacrolimus 0.03 % may represent an effective therapeutic alternative in MMP treatment and may be used in cases of hypersensitivity to standard therapy.

RESUMEN: El penfigoide de la membrana mucosa (PMM) es una enfermedad ampollosa que afecta predominantemente a las membranas mucosas y que puede provocar importantes impactos negativos en la calidad de vida del paciente. El tratamiento de primera línea de PMM se basa en el uso de corticosteroides tópicos y sistémicos. En este informe, presentamos un caso de una paciente femenina de 45 años que presentó ampollas en la encía inferior durante más de 8 meses. La paciente informó ser alérgica a los corticosteroides. Bajo la hipótesis clínica de liquen plano oral y PMM, se realizó una biopsia incisional y se estableció el diagnóstico histopatológico de PMM. Por lo tanto, se instituyó una terapia alternativa con tacrolimus tópico al 0,03 %. La paciente mostró un excelente resultado clínico sin recurrencia después de 5 meses de la terapia final. Tacrolimus 0,03 % puede representar una alternativa terapéutica efectiva en el tratamiento de PMM y se puede usar en casos de hipersensibilidad a la terapia estándar.

Penfigoide de las mucosas a lo largo de los años en nuestro Servicio / Mucous membrane pemphigoid through the years in our Department

Introducción: el penfigoide de las mucosas (PM), antes llamado mucoso, cicatrizal o mucosinequiante, representa un grupo heterogéneo de enfermedades ampollares autoinmunes inflamatorias crónicas que comprometen las mucosas o la piel, con tendencia a dejar secuelas cicatrizales. Existen autoanticuerpos contra distintos componentes de la zona de la membrana basal (BPAG1, BPAG2, integrina α6ß4, laminina 332, colágeno VII, entre otros), por lo que la inmunofluorescencia directa (IFD) es de suma importancia, así como la clínica, para su diagnóstico.Objetivo: realizar una revisión de los casos de PM diagnosticados durante un período de 24 años (enero de 1997- marzo de 2021) en el Sector de Enfermedades Ampollares del Hospital Ramos Mejía para determinar la epidemiología, la clínica y la terapéutica de esta enfermedad.Diseño: estudio retrospectivo descriptivo y observacional, en el que se analizaron las características clínicas e inmunopatológicas de 34 pacientes con diagnóstico de PM atendidos en el Servicio de Dermatología del Hospital Ramos Mejía desde enero de 1997 hasta marzo de 2021. Materiales y métodos: mediante las historias clínicas y los regis-tros iconográficos, se evaluaron las siguientes variables: prevalencia del diagnóstico de PM en los pacientes atendidos en el Sector, sexo, edad, antecedentes personales, mucosas afectadas, tiempo de evolución hasta el diagnóstico, hallazgos en la IFD, seguimiento clínico y tratamientos instaurados. Resultados: se estudió la evolución clínica de 34 pacientes diagnosticados con PM (5,3% del total de pacientes evaluados en el Sector de Patologías Ampollares). El sexo más afectado fue el femenino y la edad promedio en el momento del diagnóstico fue de 64 años. El 70,6% de los pacientes presentaron comorbilidades asociadas como hipertensión e hipotiroidismo. La mayoría refirió algún evento emocional como factor desencadenante. El sitio más comprometido fue la mucosa ocular y la cavidad oral fue la segunda en frecuencia. El tiempo de evolución promedio hasta el momento del diagnóstico fue de 4 años y 11 meses. El hallazgo más frecuente en la IFD fue la IgG lineal. El 17,6% de los pacientes interrumpieron el seguimiento clínico. El tratamiento más utilizado fue el mofetil micofenolato, con el que se obtuvo buena respuesta terapéutica. Conclusiones: el PM es una enfermedad autoinmune infrecuente que compromete las mucosas y, ocasionalmente, la piel. En este estudio, se observó que la principal mucosa afectada fue la conjuntival, a diferencia de lo referido en la bibliografía internacional dermatológica. El diagnóstico interdisciplinario temprano es fundamental para evitar las secuelas irreversibles.

Introduction: mucous membrane pemphigoid (MMP), also known as benign mucous membrane pemphigoid, cicatricial or mucosynechial pemphigoid, belongs to an heterogeneous group of chronic inflammatory autoimmune blistering diseases, which involves the mucous membranes (oral, ocular, pharyngeal, nasal, esophageal, laryngeal and anogenital) and/ or skin with tendency to scar formation. There are autoantibodies against different components of the basement membrane zone (BPAG 1- BPAG2, Integrin α6ß4, Laminin 332, Col VII, among others). The direct immunofluorescence (DIF) will be of paramount importance, as well as the clinical diagnosis.Objective: review the cases diagnosed with mucous membrane pemphigoid for 24 years (January 1997- March 2021) in the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital to establish the epidemiologic, clinic presentation and available treatments in this pathology.Design: retrospective descriptive and observational study of the clinical and immunopathological characteristics of 34 patients with MMP that were treated at the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital between January 1997 and March 2021.Materials and methods: though the medical histories and the photographic registries, we evaluated the following variables: prevalence of MMP within the patients that came to consult at the Blistering Disease Clinic at the Dermatology Department, sex, age, personal history, the affected mucous, evolution time until the diagnosis, direct immunofluorescence findings, clinical follow-ups and treatments.Results: we studied the clinical evolution of 34 patients diagnosed with MMP at our institution (5.3% from the total of patients at the Blistering Disease Clinic).The most affected gender was female and the average age at diagnosis was 64 years. 70.6% presented comorbidities such as hypertension and hypothyroidism. Most of our patients referred an emotional triggering event. The most affected membrane mucous was the ocular one and the oral was the second one. The delay in diagnosis was 4 years and 11 months. Linear deposits of IgG was the most frequent result in the direct immunofluorescence. 17.6% did not continue clinical follow-up. Mycophenolate mofetil was the most used drug with a good therapeutic response. Conclusions: MMP is a rare autoimmune disease that affects mucous membrane and occasionally the skin. In this study, the ocular involvement was the most frequent one, differing with the international reports. The early interdisciplinary diagnosis is essential to avoid irreversible sequelae.

Penfigoide de membrana mucosa asociado a carcinoma pulmonar ¿penfigoide paraneoplásico? / Mucous membrane penfigoid associated with pulmonary carcinoma ¿paraneoplastic penfigoid?

El penfigoide de la membrana mucosa es una enfermedad autoinmune, que afecta a los diferentes estratos de la mucosa y la piel, y se caracteriza por lesiones vesiculo-ampollosas polimórficas. Presentando combinaciones variables de lesiones orales, oculares, cutáneas, genitales, nasofaríngeas, esofágicas y laríngeas. Su relación con las neoplasias malignas es poco conocida y sigue siendo controvertida. El presente artículo describe el manejo multidisciplinario de una paciente de 46 años de edad que desarrolló un penfigoide de membrana mucosa a nivel de la cavidad oral. Esta patología estuvo asociada a un carcinoma de pulmón, y posteriormente presentó alteraciones multiorgánicas. El tratamiento fue realizado por las especialidades de dermatología, medicina interna, cirugía general, oftalmología, estomatología, cirugía oral y maxilofacial, dicho tratamiento se llevó a cabo por etapas y tuvo una duración de 2 meses aproximadamente, logrando la resolución de las lesiones que presentaba la paciente. Consideramos que se abre una puerta para futuras investigaciones que confirman la relación entre el penfigoide de la membrana mucosa y el carcinoma de pulmón.

Mucous membrane pemphigoid is an autoimmune disease that affects different layers from mucosa and skin, characterized by polymorphic vesicular-bullous lesions, presenting variable combinations of oral, ocular, skin, genital, nasopharyngeal, esophageal, and laryngeal lesions. Its relationship to malignant neoplasms is poorly understood and remains controversial. This article describes the multidisciplinary management of a 46-year-old patient who developed mucous membrane pemphigoid at the oral cavity level. This pathology was associated with lung carcinoma, and later presented multiorgan alterations, for which a treatment was carried out with the specialties of dermatology, internal medicine, general surgery, ophthalmology, stomatology, oral and maxillofacial surgery, said treatment was carried out in stages and lasted for approximately 2 months, achieving the resolution about lesions that patient presented. We believe that a door opens for future research confirming the relationship between mucous membrane pemphigoid and lung carcinoma.

Imatinib-induced cutaneous adverse reactions / 中华皮肤科杂志

Imatinib mesylate is the first-line drug for the treatment of chronic myeloid leukemia and malignant gastrointestinal stromal tumors, and causes mucocutaneous adverse reactions at a high frequency. Based on Chinese and international literature, the authors comprehensively describe various mucocutaneous symptoms induced by imatinib mesylate, and summarize their pathogenesis and management strategies, aiming to help dermatologists improve their understanding of mucocutaneous adverse reactions to this drug, and provide timely diagnosis and treatment.

Consensus of Chinese experts on protection of skin and mucous membrane barrier for health professions fighting against coronavirus disease 2019 / 中华皮肤科杂志

Health professions preventing and controlling coronavirus disease 2019 are prone to skin and mucous membrane injuries, which may cause acute and chronic dermatitis, secondary infections and aggravation of underlying skin diseases. This is a consensus of Chinese experts on measures and advice on hand cleaning- and medical glove- related hand protection, mask- and goggles- related face protection, ultraviolet- related protection, as well as eye, nasal and oral mucosa, outer ear and hair protection. It is necessary to strictly follow standards on wearing protective equipments and specifications on sterilizing and cleaning. Both insufficient and excessive protection will adversely affect the skin and mucous membrane barrier. At the same time, using moisturizing products is highly recommended to achieve better protection.

Síndrome Sjögren y penfigoide de las membranas mucosas en paciente con antecedente de timoma: Reporte de caso y revisión de literatura / Sjögren Syndrome and Mucous Membrane Pemphigoid in a Patient with a History of Thymoma: Case Report and Literature Review

RESUMEN Los timomas son las neoplasias mediastínicas más comunes en adultos. Los pacientes con timoma son frecuentemente diagnosticados con síndromes paraneoplásicos y generalmente están asociados con varias condiciones autoinmunes. El síndrome de Sjögren (SS) es un trastorno inflamatorio autoinmune, que se caracteriza por la destrucción mediada por linfocitos de las glándulas exocrinas, que conduce a la ausencia de secreción glandular, en particular las salivales y las lagrimales; la hiposalivación puede variar desde una ligera reducción en el flujo de saliva con molestias transitorias, a un grave deterioro de la salud oral y malestar psicológico. El penfigoide de membranas mucosas (PMM) es una enfermedad autoinmune crónica, muestra predominante afectación de la mucosa, afectando con mayor frecuencia la cavidad oral, seguida de la conjuntiva, la cavidad nasal y el esófago; intraoralmente incluye gingivitis descamativa, vesículas, erosiones cubiertas por pseudomembranas y úlceras. El objetivo de este trabajo es presentar el primer caso de una paciente con antecedente de timoma, la cual, conjuntamente presentó SS y PMM; además evidenciar la importancia del manejo multidiciplinario para un diagnóstico preciso y temprano, ya que así, aumenta la eficiencia y la eficacia de la estrategia de tratamiento.

ABSTRACT Thymomas are the most common mediastinal neoplasms in adults. Patients with thymoma are frequently diagnosed with paraneoplastic syndromes and are generally associated with several autoimmune conditions. Sjögren's syndrome (SS) is an autoimmune inflammatory disorder, characterized by the lymphocyte-mediated destruction of the exocrine glands, which leads to the absence of glandular secretion, particularly salivary and lacrimal; hyposalivation can vary from a slight reduction in the flow of saliva with transient discomfort, to a serious deterioration of oral health and psychological distress. Mucous membrane pemphigoid (MMP) is a chronic, autoimmune disease that shows predominant mucosal involvement, most commonly affecting the oral cavity, followed by the conjunctiva, the nasal cavity and the esophagus; intraorally includes scaly gingivitis, vesicles, erosions covered by pseudomembranes and ulcers. The objective of this work is to present the first case of a patient with a history of thymoma, which, jointly presented SS and MMP; also highlight the importance of multidisciplinary management for an accurate and early diagnosis, as this increases the efficiency and efficacy of the treatment strategy.

Intraoral acanthosis nigricans and its clinical relevance as a manifestation of a paraneoplastic syndrome: a case report / Acantosis nigricans intraoral y su relevancia clínica como manifestación de un síndrome paraneoplásico: reporte de un caso

Malignant-or-paraneoplastic acanthosis nigricans is a verrucous and hyperpigmented tumor affecting the mucosa and skin. In most cases malignant acanthosis nigricans is a distant manifestation of an intra-abdominal primary cancer. While the diagnosis of malignant acanthosis nigricans is challenging, some specific clinical and histopathological findings could lead to an accurate diagnosis. A rare clinical case of a 59-year-old female, who was referred to the maxillofacial surgery service due to a painful oral lesion in the palatine region, is presented. Upon examination, papillomatous lesions were observed on the hard palate, that were later diagnosed as intraoral malignant acanthosis nigricans secondary to gastric cancer. Both local and systemic evaluations are discussed, highlighting the relevance of a multidisciplinary approach consistent with the fact that these manifestations, although infrequent, should generate suspicion among clinicians and therefore motivation to perform a diligent and complete study since it can reveal the presence of a malignant pathology.

La acantosis nigricans maligna o paraneoplásica es un tumor verrugoso e hiperpigmentado que afecta la mucosa y la piel. En la mayoría de los casos, la acantosis nigricans maligna es una manifestación distante de un cáncer primario intraabdominal. Si bien el diagnóstico de acantosis nigricans maligna es desafiante, algunos hallazgos clínicos e histopatológicos específicos podrían conducir a un diagnóstico preciso. Se presenta un caso clínico raro de una mujer de 59 años, que fue derivada al servicio de cirugía maxilofacial debido a una lesión oral dolorosa en la región palatina. En el examen, se observaron lesiones papilomatosas en el paladar duro, que posteriormente se diagnosticaron como acantosis nigricans maligna intraoral secundaria a cáncer gástrico. Se discuten tanto las evaluaciones locales como las sistémicas, destacando la relevancia de un enfoque multidisciplinario consistente con el hecho de que estas manifestaciones, aunque poco frecuentes, deberían generar sospecha entre los clínicos y, por lo tanto, motivación para un estudio diligente y completo, ya que puede revelar la presencia de una patología maligna.

Diagnosis and treatment of skin diseases manifesting as desquamative gingivitis / 中华皮肤科杂志

Skin diseases manifesting as desquamative gingivitis (DG) can be divided into recurrent DG-and chronic DG-related skin diseases,including oral lichen planus,mucosal pemphigoid,pemphigus vulgaris and so on.A thorough medical history,detailed oral and histopathological examinations and serum immunological tests can be helpful for correct diagnosis of DG-related skin diseases.The treatment of DG-related skin diseases includes topical and systemic therapies.It is necessary to individualize treatment protocols due to treatment response.During the treatment of DG,oral hygiene should be strengthened,secondary fungal and bacterial infections should be avoided,and attention should be paid to the protection of oral cavity and periodontal tissues.

BP180-related autoimmune blistering diseases / 中华皮肤科杂志

BP180-related autoimmune blistering diseases include bullous pemphigoid,lichen planus pemphigoides,linear IgA bullous dermatosis,pemphigoid gestationis and cicatricial pemphigoid.There are multiple autoantibody-reactive sites on the extracellular region of BP180.Current studies show that there is heterogeneity in the autoimmune blistering disease-related target sites on BP 180,and different clinical manifestations of the same disease are related to the heterogeneity of target sites.However,further studies and analysis are still needed for the mechanism of the heterogeneity.

Bronquiolitis obliterante secundaria a sindrome de stevens-johnson. Presentacion de 2 casos y revision de la literatura / Obliterans bronchiolitis secondary to stevens-johnson syndrome: case reports

Stevens-Johnson syndrome corresponds to a hypersensitivity reaction produced by various etiologies, for example exposure to drugs, microbial agents, or by an idiopathic cause. It is marked by an acute vesicular-bullous eruption, which affects the skin and mucous membranes, with systemic manifestations of variable severity, and it may present a fatal evolution. Stevens-Johnson syndrome can occasionally present chronic pulmonary complications, such as bronchiolitis obliterans; however, other etiologies are more frequent in our environment, for example severe pneumonia due to adenovirus. Our objective is to present two cases of bronchiolitis obliterans post Stevens-Johnson syndrome and to make a literature review.

El síndrome de Stevens-Johnson corresponde a una respuesta de hipersensibilidad producida por diversas etiologías, que incluyen exposición a drogas, agentes microbianos o idiopática. Se manifiesta por una erupción vesículo-bulosa aguda, que afecta la piel y las mucosas, con manifestaciones sistémicas de severidad variable, pudiendo presentar una evolución fatal. El síndrome de Stevens-Johnson puede presentar ocasionalmente complicaciones pulmonares crónicas, como bronquiolitis obliterante, siendo en nuestro medio más frecuente otras etiologías, como la observada luego de una neumonía grave por adenovirus. El objetivo es presentar dos casos de bronquiolitis obliterante post síndrome de Stevens-Johnson y hacer una revisión de la literatura.

Desenlaces en pacientes operados con queratoprótesis boston tipo 1 en un centro oftalmológico especializado en la ciudad de Medellín / Outcomes in patients with boston type 1 keratoprosthesis in a specialized center in Medellín

Objetivo: reportar las características y descenlaces de pacientes con queratoprótesis Boston tipo 1. Diseño: estudio observacional descriptivo de corte transversal. Método: se incluyeron los pacientes operados con queratoprótesis Boston tipo 1 residentes en Colombia, mayores de 17 años, sin queratoprótesis previas y que tuviera más de 6 meses de seguimiento, para un total de 39 ojos de 39 pacientes. La cirugía se indicó en los casos con o sin queratoplastia previa que tuvieran agudeza visual (AV) igual o inferior a cuenta dedos y déficit de limbo bilateral o vascularización corneal profunda en más de 3 cuadrantes. Se recolectó y analizó la información de la historia clínica en forma retrospectiva y prospectiva. Las variables principales fueron: agudeza visual, retención del dispositivo y complicaciones. Se realizó un análisis univariado presentando los resultados en frecuencias absolutas y porcentajes para las variables categóricas y para las variables cuantitativas como promedio y desviación estándar (DE) si tienen distribución normal o como mediana y rango intercuartil si no tienen distribución normal. Resultados: La mediana de seguimiento fue de 53.5 meses (rango 6 meses a 10 años). La mayoría de pacientes (66.7%) habían tenido previamente queratoplastia penetrante. El diagnóstico de base fue en 53.8% condiciones no inflamatorias yen 46.2% condiciones inflamatorias. La AV preoperatoria en todos los ojos estaba entre percepción de luz y cuenta dedos; posterior a la cirugía el 97.4% tuvieron mejoría de la AV. Para el fi nal del seguimiento la AV permaneció mejor que la preoperatoria en 53.8%, fue igual a esta en 17.94% y peor que esta en 28.2%. Hubo necrosis periprostética en 28.2%, y 38.5% tuvieron recambio del dispositivo. Al final del seguimiento 89.7% de los pacientes tenían una queratoprótesis in-situ. Conclusión: la queratoprótesis Boston tipo 1 es una opción viable para mejorar la AV en pacientes con trasplantes de córnea fallidos o en quienes se prevé alta tasa de fallo o rechazo de este. En la gran mayoría de los pacientes se logra mejoría inicial significativa de la AV, la cual se mantiene al final del seguimiento en más de la mitad de ellos. Refinamientos progresivos en la técnica quirúrgica y el manejo postoperatorio disminuirán las complicaciones que llevan a la perdida visual progresiva

Purpose: to report characteristics and outcomes in patients with Boston type 1 keratoprosthesis. Design: observational descriptive cross-sectional study. Method: Patients with Boston type 1 queratoprosthesis who were living in Colombia, 17 years or older, without previous keratoprosthesis and with more than 6 months follow up were included, for a fi nal of 39 eyes from 39 patients. Surgery was indicated for those with or without previous keratoplasty who had visual acuity (VA) of counting fi ngers or worse and bilateral limbal stem cell deficiency or deep corneal vascularization in more than 3 quadrants. Information was collected retrospectively and prospectively from medical registries and was analyzed. Main variables were: visual acuity, device retention and complications. An univariate analysis was conducted and results are presented in absolute frequencies and percentages for categoric variables and for quantitative variables in average and standard deviation (SD) if they have normal distribution and in median and interquartile range if they do not have normal distribution. Results: Median follow up was 53.5 months (range 6 months to 10 years). Most patients (66.7%) had previous penetrating keratoplasty. Baseline diagnoses were in 53.8% non-infl ammatory conditions and in 46.2% inflammatory conditions. Preoperative VA ranged between light perception and counting fingers in all eyes; aft er surgery 97.4% had improvement in VA. By the end of follow up VA remained better than preoperative in 53.8%, was the same as preoperative in 17.94% and worse than preoperative in 28.2%. There was periprosthetic necrosis in 28.2%, and 38.5% had keratoprothesis exchange. At the end of follow up 89.7% of patients had a keratoprosthesis in-situ. Conclusion: Boston type 1 keratoprosthesis is a viable option to improve VA in patients with previously failed corneal transplants or in cases that anticipate bad prognosis for keratoplasty. In most patients there was a signifi cant initial improvement in vision, and more than half of them retained better than initial VA on their last follow up. Further refinements in surgical technique and postoperative care would decrease complications that lead to progressive visual loss.

Nasolabial and extended nasolabial flaps for reconstruction in oral submucous fibrosis

OBJECTIVES: The objective of the study was to evaluate the results of nasolabial/extended nasolabial flaps as a modality for treatment of oral submucous fibrosis. MATERIALS AND METHODS: Eleven patients of Stage III or IVa maximum interincisal opening were selected to be operated. Nasolabial/extended nasolabial flaps were done for both the sides. All of the flaps were done in a single stage and were inferiorly based. A similar flap harvest/surgical technique was utilized for all the cases. RESULTS: The preoperative mouth opening ranged from 5 to 16 mm, with a mean of 10.09 mm. At 6 months the mouth opening ranged from 29 to 39 mm. Some of the complications encountered were poor scar, wisdom tooth traumatising the flap, decreased mouth opening due to non compliance and too much bulk. All of theses were managed satisfactorily. CONCLUSION: The nasolabial flap is a very reliable flap to restore the function of oral cavity. Important adjuvant measures are habit cessation, lifestyle changes, and aggressive physiotherapy.