Cutaneous findings in neonates and their socio-demographic profile: a prospective observational study

Background: A wide range of disorders can manifest as skin lesions due to the great difficulty the skin encounters while transitioning from an aqueous to a dry environment in a neonate. Among the skin conditions commonly seen in neonates are temporary benign lesions, atopic dermatitis (and associated disorders), bullous dermatoses. Aim was to study the various patterns of skin lesions in neonates and to estimate the prevalence of physiological and pathological skin lesions.Methods: This prospective observational study was conducted in the department of pediatrics at Acharya Shri Chander College of Medical Sciences and Hospital (ASCOMS) a tertiary care hospital in Jammu. A total of 300 neonates (day 1 to day 28 of life) and having any skin lesions or manifestations born at the hospital or visiting out patient抯 department were included in the study. The data collected was compiled on excel sheet and was analyzed statistically by using statistical package for the social sciences (SPSS) software version 21.0.Results: It was found that the most of the skin manifestations were observed in neonates born to multiparous mothers at term gestation and having average weight. The male to female ratio was 1.3:1. The frequency of the cutaneous manifestations were seen more in neonates born via the caesarean section.Conclusions: It is concluded that the most common skin lesion was erythema toxicum followed by icterus neonatorum.

Beyond the Gut: Extraintestinal Manifestations of Inflammatory Bowel Disease in Children and Adolescents / Más allá del intestino: manifestaciones extraintestinales de la enfermedad inflamatoria intestinal en niños y adolescentes

Abstract Background: Extraintestinal manifestations (EIM) are common in children and adults and their presence is associated with a higher severity of inflammatory bowel disease (IBD). In pediatrics, studies are scarce in Latin America and do not exist in Colombia. Objective: To describe the prevalence of EIM in children with IBD and the associated demographic, clinical, and biochemical characteristics. Methodology: Retrospective study of patients diagnosed with pediatric-onset IBD between 2007 and 2022 treated at an institution in Bogotá. A descriptive analysis was performed and in the groups with and without EIM clinical and biochemical variables were compared using Fisher's exact test, Student's T, and Mann-Whitney's U. Results: Of 71 confirmed cases, 45% had typical ulcerative colitis, 27% Crohn's disease, 7% atypical ulcerative colitis, 1.4% colonic Crohn's, and 20% unclassifiable IBD. Thirteen patients (18%) had at least one EIM, and mucocutaneous was the most frequent (58%). The time from symptoms onset to diagnosis was higher in the EIM group (13.2 years vs. 10.2 years; p = 0.02). Nocturnal diarrhea, hyporexia, and weight loss were more frequent in children with EIM. The EIM group showed lower hemoglobin levels, and higher globular sedimentation rate, and received biologics more frequently (38% vs. 23%, p = 0.2). Conclusions: The frequency of MEI in this series is similar to that reported in the literature; its presence is associated with higher disease severity, higher frequency of biological use, and longer time IBD diagnosis.

Resumen Antecedentes: Las manifestaciones extraintestinales (MEI) son comunes en niños y adultos y su presencia se asocia con mayor gravedad en la enfermedad inflamatoria intestinal (EII). En pediatría, los estudios son escasos en Latinoamérica y no existen en Colombia. Objetivo: Describir la prevalencia de las MEI en niños con EII y las características demográficas, clínicas y bioquímicas asociadas. Metodología: Estudio retrospectivo de pacientes diagnosticados con EII de inicio pediátrico entre 2007 y 2022 atendidos en una institución en Bogotá. Se realizó un análisis descriptivo y en los grupos con y sin MEI se compararon variables clínicas y bioquímicas por medio de la prueba exacta de Fisher, T de Student y U de Mann-Whitney. Resultados: De 71 casos confirmados, el 45 % tenía colitis ulcerativa típica, el 27 % enfermedad de Crohn, el 7 % colitis ulcerativa atípica, el 1,4 % Crohn colónico y el 20 % EII no clasificable. 13 pacientes (18 %) presentaron al menos una MEI, y las mucocutáneas fueron las más frecuentes (58 %). El lapso desde el inicio de los síntomas hasta el diagnóstico fue mayor en el grupo MEI (13,2 años frente a 10,2 años; p = 0,02). La diarrea nocturna, la hiporexia y la pérdida de peso fueron más frecuentes en niños con MEI. El grupo MEI mostró niveles más bajos de hemoglobina, más elevados de velocidad de sedimentación globular y recibieron biológicos con mayor frecuencia (38 % frente a 23 %, p = 0,2). Conclusiones: La frecuencia de MEI en esta serie es similar a lo reportado en la literatura; su presencia se asocia con una mayor gravedad de la enfermedad, mayor frecuencia de uso de biológicos y mayor tiempo de diagnóstico de la EII.

Acute sarcoidosis Löfgren Syndrome treated by Chinese medicinal formula based on Syndrome Differentiation: a case - based review / Tratamiento de síndrome de Löfgren de sarcoidosis con fórmula medicinal china basada en diferenciación de síndrome: una revisión basada en casos

Löfgren syndrome (LS) is a unique acute manifestation of sarcoidosis and characterized by erythema nodosum, bilateral hilar lymphadenectasis, and/or bilateral ankle arthritis or periarthritis. A 37 - year - old female patient with LS presented with fever accompanied by multiple joint swelling and pain, nodular skin erythema, and bilateral hilar lymphadenectasis. The patient had received treatment involving non - steroidal anti - inflammatory drugs and glucocorticoids in other hospitals, but the effects were poor, and the conditions reemerged. The LS duration has lasted for more than 3 months. Following traditional Chinese medicine (TCM) treatment, syndrome differentiation as well as giving patients oral Chinese medicine decoction, the symptoms of the patient were rapidly relieved within one week and did not recur during a six - month follow - up period. This case is the first clinical report of acute sarcoidosis LS treated using T CM and reflects the significant advantages of this form of therapy in emergency treatment

El síndrome de Löfgren (LS) es una manifest ación única y aguda de sarcoidosis, caracterizada por eritrema nodoso, linfadenectasis hilar bilateral, y/o a r tritis de tobillo bilateral o periartritis. Una paciente de 37 años de sexo femenino con LS se presentó con fiebre, acompañada de inflamación y do lor múltiple de articulaciones, eritrema nodular cutáneo, y linfadenectasis hilar bilateral. La paciente recibió un tratamiento que consistió en antiinflamatorios no esteroidales y glucocorticoides en otros hospitales, pero los efectos fueron leves y las c ondiciones reemergieron. El LS ha durado más de tres meses. Siguiendo el tratamiento de medicina tradicional china (MTC), la diferenciación de síndrome, así como darles a los pacientes una decocción de medicina china por vía oral, los síntomas de la pacien te rápidamente fueron aliviados en el curso de una semana y no recidivaron durante los seis meses de un seguimiento. El caso es el primer reporte clínico de tratamiento de sarcoidosis aguda asociada a LS usando TCM y refleja las significativas ventajas de esta forma de terapia en el tratamiento de emergencia.

Oral mucosal manifestations of Sweet’s syndrome: a case report and literature review / 口腔疾病防治

Objective@#To explore the oral mucosal manifestations of Sweet’s syndrome and provide a reference for its early detection and correct diagnosis.@*Methods@#The oral mucosal manifestations of a 60-year-old female patient with Sweet’s syndrome are described in detail, followed by a discussion of the related literature.@*Results@#The patient had skin erythema of both lower extremities, which was accompanied by oral mucosal ulceration and pain for 3 days. The patient presented with mild cutaneous lesions and diffuse large-scale erosion in the oral mucosa with obvious pain. During the onset of the disease, the patient was accompanied by fever with a temperature of 38.5°C. After visiting the Department of Stomatology, laboratory tests showed an increase in C-reactive protein (35.2 mg/L) and an accelerated erythrocyte sedimentation rate (77.00 mm/h). Scattered red plaques and mild tenderness were observed in the knees and lower limbs. Histopathological examination of the skin lesions revealed scattered infiltration of immature neutrophils across the entire dermis. The patient responded well to glucocorticoid therapy. According to the clinical signs and laboratory examination, combined with the lesion histopathological results, a diagnosis of Sweet’s syndrome was given. The patient was administered 1 mL compound Betamethasone injection only once intramuscularly. In the meantime, the patient was asked to gargle with compound chlorhexidine solution and topically apply recombinant bovine basic fibroblast growth factor solution to the damaged mucosa three times a day for 1 week. After 4 days of medication, the patient’s body temperature had returned to normal and the oral lesions were significantly reduced. After 2 weeks, the erythema in the leg and knee had almost all subsided, and the oral mucosal lesions had disappeared. The patient was followed up 6 months after treatment, with no recurrence of skin lesions. After 2 years of follow-up, the disease was stable with no recurrence. A review of the relevant literature shows that Sweet’s syndrome is a rare inflammatory reactive dermatosis with unknown etiology, which can be divided into three clinical types: specific, tumor-related, and drug-induced. The male/female prevalence ratio is 1:4. The salient clinical manifestations are abrupt onset of painful erythematous plaques or nodules most commonly involving the extremities, often accompanied by pyrexia, elevated neutrophil count, elevation of the erythrocyte sedimentation rate, and positive C-reactive protein. The use of glucocorticoids is the most common treatment for this disease, and most patients see a rapid improvement in skin lesions; however, some may experience infection or recurrence after withdrawal. Some patients with Sweet’s syndrome are accompanied by oral lesions, but cases of oral mucosal damage have been rarely reported, and this condition is easily misdiagnosed.@*Conclusion@#Oral mucosal lesions may be extraterritorial manifestations of Sweet’s syndrome, and the patient’s systemic condition should be comprehensively considered. Skin biopsy should be completed as soon as possible to make a clear diagnosis, so as not to delay the disease.

Estado reaccional de enfermedad de Hansen como causa de fiebre de origen desconocido

Los fenómenos reaccionales de la lepra son complicaciones inflamatorias que a menudo se presentan como verdaderas emergencias médicas durante el curso del tratamiento o incluso después de culminarlo. A menudo, los síntomas sistémicos como la fiebre pueden aparecer antes que las manifestaciones cutáneas y desconcertar al médico. Se realiza la presentación del caso de un paciente masculino, 39 años, sano, que acude con cuadro de 2 meses de evolución de fiebre, pérdida de peso, con posterior aparición de manchas rojas en todo el cuerpo. Se realizaron biopsia de piel y baciloscopia, con los cuales se pudieron llegar al diagnóstico de lepra.

The reactional phenomena of leprosy are inflammatory complications that often present as true medical emergencies during treatment or even after completion. Often, systemic symptoms such as fever can appear before skin manifestations and baffle the doctor. The case presented is a 39-year male patient, healthy, who consult with a 2-month history of fever, and weight loss, with subsequent appearance of red spots all over the body. A skin biopsy and smear microscopy were performed, with which leprosy was diagnosed.

Respuesta inmune en pacientes con diagnóstico de lepra

Introducción: La lepra es una entidad de expresión florida con afectación frecuente en el tegumento cutáneo y los nervios periféricos, por la predisposición que presenta el Mycobacterium leprae a estas estructuras. Las reacciones leprosas pueden aparecer en el curso de la enfermedad. Estas interrumpen la evolución crónica usual y la estabilidad clínica de los pacientes que la padecen. Objetivo: Caracterizar los estados reaccionales de la lepra. Materiales y métodos: Se realizó un estudio descriptivo en el período de enero de 2019 a septiembre de 2022, en pacientes que acudieron a la Consulta Provincial de Lepra en el Hospital Universitario Clínico Quirúrgico Comandante Faustino Pérez Hernández, de Matanzas. El universo estuvo constituido por 8 pacientes que presentaron estados reaccionales en la etapa mencionada. Se recogieron de las historias clínicas variables como: edad, sexo, clasificación de la lepra según Ridley-Jopling, tipo de estado reaccional, forma clínica y momento de aparición. Resultados: La mayor frecuencia estuvo entre el rango de 50 a 64 años, con un 50 %. El sexo masculino representa el 62,5 %. Se mostró prevalencia de la lepra lepromatosa en el 62,5 %. La reacción tipo II y las formas graves fueron las más frecuentes, con un 62,5 % y 75 % respectivamente. Existió predominio de las reacciones leprosas durante y después del tratamiento, sin diferencias entre estas, con un 37,5 %. Conclusiones: La reacción tipo II y las formas graves de presentación fueron las predominantes en pacientes masculinos, representados en el grupo etario de 50 a 64 años. La forma clínica preponderante en estos eventos fue la lepromatosa.

Introduction: Leprosy is a floridly expressed entity with frequent involvement of the cutaneous integument and peripheral nerves due to the predisposition of Mycobacterium leprae to these structures. Leprosy reactions may appear during the course of the disease. These interrupt the usual chronic course and the clinical stability of patients suffering from the disease. Objective: To characterize the reactional states of leprosy. Materials and methods: A descriptive study was carried out from January 2019 to September 2022 in patients who attended the Provincial Leprosy Clinic at the Clinical Surgical University Hospital Comandante Faustino Pérez Hernández, in Matanzas. The universe consisted of 8 patients who presented reactional states in the aforementioned stage. The variables, collected from the clinical records, were: age, sex; classification of leprosy according to Ridley-Jopling, type of reactional state, clinical form and time of onset. Results: The highest frequency was between 50 and 64 years, with 50%. The male sex represents 62.5%. Lepromatous leprosy prevalence was shown in 62.5%. The type II reaction and severe forms were the most frequent with 62.5% and 75% respectively. There was predominance of leprosy reactions during and after treatment without differences between them, with 37.5%. Conclusions: The type II reaction with severe forms of presentation was predominant in male patients represented in the age group of 50 to 64 years. The predominant clinical form in these events was the lepromatous one.

Endocarditis infecciosa y eritema multiforme minor

El eritema multiforme es una reacción inmunomediada que envuelve la piel y algunas veces a la mucosa. Las infecciones y medicamentos constituyen las causas más comunes. Típicamente se presenta como lesiones "en diana" sobreelevadas con centro pálido y borde eritematoso o lesiones atípicas como vesículas o ampollas. Se presenta el caso de una mujer de 68 años que acude por tumoración a nivel del antebrazo derecho con signos inflamatorios más fiebre, se constata por ecografía aneurisma micótico en dicho miembro y se realiza ecocardiografía transtorácica donde se observan vegetaciones en válvulas aortica y mitral. Durante la internación presenta aparición de lesiones ampollosas con bordes eritematosos distribuidas en tronco y brazos con biopsia que informa erupción liquenoide ampollosa compatible con eritema multiforme minor.

Erythema multiforme is an immune-mediated reaction that involves the skin and sometimes the mucosa. Infections and medications are the most common causes. Typically presents as raised "target" lesions with a pale center and erythematous border or atypical lesions such as vesicles or bullae. We present the case of a 68-year-old woman who presented with a tumor on the right forearm with inflammatory signs plus fever, a mycotic aneurysm was confirmed by ultrasound in the limb and a transthoracic echocardiography was performed where vegetations were observed on the aortic and mitral valves. During hospitalization, she presented bullous lesions with erythematous borders distributed on the trunk and arms with a biopsy that reported bullous lichenoid eruption compatible with erythema multiforme minor.

Erythema annulare centrifugum: a case report

Erythema annulare centrifugum (EAC) is a rare cutaneous disease characterized by polycyclic lesion simulating urticarial papules that enlarge centrifugally with central clearing and trailing scales. . It is classified as a reactive erythema and has been associated with various underlying conditions, including malignancies. Diagnosis is established on history, clinical features, and supporting examination. No treatment seems to be effective for this disease and there is still a lack of consensus regarding the best approach. Current choices are focused on treating the underlying subjective complaints. We reported a case of erythema annulare centrifugum in a 49 year old female patient.

Erythema annulare centrifugum: a case report

Erythema annulare centrifugum (EAC) is a rare cutaneous disease characterized by polycyclic lesion simulating urticarial papules that enlarge centrifugally with central clearing and trailing scales. . It is classified as a reactive erythema and has been associated with various underlying conditions, including malignancies. Diagnosis is established on history, clinical features, and supporting examination. No treatment seems to be effective for this disease and there is still a lack of consensus regarding the best approach. Current choices are focused on treating the underlying subjective complaints. We reported a case of erythema annulare centrifugum in a 49 year old female patient.

Dermatological manifestations of hematologic neoplasms. Part II: nonspecific skin lesions/paraneoplastic diseases

Abstract Cutaneous manifestations occur in the course of hematologic malignancies and precede, accompany or occur late in relation to the diagnosis. They result from paraneoplastic phenomena, tumor infiltrations, immunosuppression resulting from the hematologic disease itself or its treatment. The dermatologist must be aware of these conditions that may be helpful both in the diagnosis of the underlying disease and in reducing patient morbidity. This review (part II) addresses the paraneoplastic dermatological changes associated with systemic hematologic malignancies.

Risk Factors for Erythema Nodosum Leprosum: A Case Control Study in a Tertiary Hospital of Western Odisha, India

Erythema nodosum leprosum (ENL) is a serious, often recurring and disabling, immunologically mediated reaction occurring in leprosy which often requires hospitalization. There are published several studies of ENL, but systematic studies regarding the risk factors associated with ENL in the post elimination era are few. The aim of the study was to determine the risk factors associated with ENL in a tertiary care centre in Western Odisha. This is a case control study involving 292 patients of leprosy who attended the Dermatology OPD of this tertiary care centre. These constituted 97 patients with ENL and 195 patients without ENL who attended the OPD during this period. Detailed history, clinical examination, slit skin smears were done. These included gender details, age, area of residence (rural/urban), education and socioeconomic status. The most common subtype of leprosy observed in ENL was lepromatous leprosy followed by borderline lepromatous type. Patients diagnosed with initial high BI and lepromatous leprosy were found to be significant risk factors for development of ENL. Skin diseases, Anaemia and Diabetes Mellitus were found to be more prevalent in ENL patients

A Delphi consensus on the nomenclature and diagnosis of lichen planus pigmentosus and related entities

Background: Although well known in clinical practice, research in lichen planus pigmentosus and related dermal pigmentary diseases is restricted due to lack of consensus on nomenclature and disease definition. Aims and Objectives: Delphi exercise to define and categorise acquired dermal pigmentary diseases. Methods: Core areas were identified including disease definition, etiopathogenesis, risk factors, clinical features, diagnostic methods, treatment modalities and outcome measures. The Delphi exercise was conducted in three rounds. Results: Sixteen researchers representing 12 different universities across India and Australia agreed to be part of this Delphi exercise. At the end of three rounds, a consensus of >80% was reached on usage of the umbrella term ‘acquired dermal macular hyperpigmentation’. It was agreed that there were minimal differences, if any, among the disorders previously defined as ashy dermatosis, erythema dyschromicum perstans, Riehl’s melanosis and pigmented contact dermatitis. It was also agreed that lichen planus pigmentosus, erythema dyschromicum perstans and ashy dermatosis did not differ significantly apart from the sites of involvement, as historically described in the literature. Exposure to hair colours, sunlight and cosmetics was associated with these disorders in a significant proportion of patients. Participants agreed that both histopathology and dermatoscopy could diagnose dermal pigmentation characteristic of acquired dermal macular hyperpigmentation but could not differentiate the individual entities of ashy dermatosis, erythema dyschromicum perstans, Riehl’s melanosis, lichen planus pigmentosus and pigmented contact dermatitis. Limitations: A wider consensus involving representatives from East Asian, European and Latin American countries is required. Conclusion: Acquired dermal macular hyperpigmentation could be an appropriate conglomerate terminology for acquired dermatoses characterised by idiopathic or multifactorial non-inflammatory macular dermal hyperpigmentation

Parvovirus B19 infection in a patient of juvenile arthritis: A case report

Parvovirus B19 infection, or fifth disease or erythema infectiosum, is a self-limiting viral exanthem and can occur along with other connective tissue disorders. Occasionally, children may have preceding chronic joint complaints suggestive of juvenile idiopathic arthritis. A 7-year-old female patient presented to the outpatient department with a rash over her face, upper and lower limbs, and trunk, which was preceded by fever. The patient is a known case of juvenile arthritis. On examination, there were multiple erythematous and skin-colored plaques over cheeks; few pustules over lips; multiple erythematous papules over upper limbs, lower limbs, and trunk; multiple erythematous macules over palms; and swelling over interphalangeal joints of bilateral hands, elbow and knee joints with genu valgum deformity of limbs. The patient was given topical antibiotic and steroid combination cream and oral antihistaminic for her cutaneous complaints.

Necrolytic Migratory Erythema—A Case Report of Pancreatic Occupying Lesion / 罕见病研究

Glucagonoma is a rare neuroendocrine tumor of α cells of the pancreas. The tumor excessively secretes glucagon and causes glucagonoma syndrome.70%-90% of patients with glucagonoma will develop necrolytic migratory erythema (NME). We reported a patient of glucagonoma syndrome who was presented to the dermatology outpatient clinic with a 2-year-history of recurrent erythema and scaling on the skin migrating throughout the body. A skin biopsy was performed and resulting features matched with NME, whilst imaging examinations suggested a soft tissue density tumor present in the tail of the pancreas with somatostatin receptor expression and laboratory tests found an elevated levels of serum glucagon. After the diagnosis was confirmed, the patient was treated with surgical resection of the glucagonoma and the skin eruptions resolved rapidly in 4 days. Meanwhile, we reviewed relevant literature published in recent years and summarized its clinical characteristics in order to improve its understanding by clinicians, including clinical manifestations, laboratory and imaging examinations, diagnosis and treatments.

Skin manifestations of 61 children with coronavirus disease 19 / 中华皮肤科杂志

Objective:To analyze skin manifestations associated with coronavirus disease 19 (COVID-19) in children.Methods:Children diagnosed with COVID-19 accompanied by skin manifestations were retrospectively collected from outpatient clinics or teleclinics at the Department of Dermatology, Children′s Hospital, Capital Institute of Pediatrics from November 1st, 2022 to December 10th, 2022, and their clinical characteristics were analyzed. Analysis of variance was used for comparing measurement data, and Fisher′s exact test for comparing enumeration data.Results:A total of 61 children with COVID-19 accompanied by skin lesions were included, they were aged from 22 days to 17 years (2.83 ± 2.47 years, and their course of disease ranged from 2 to 14 days. Skin lesions manifested as acute urticaria in 25 cases (41.0%), eruptive/maculopapular lesions in 10 cases (16.4%), facial vascular edema in 6 cases (9.8%), urticarial vasculitis in 5 cases (8.2%), pityriasis rosea and erythema multiforme each in 4 cases (6.6%), purpura in 2 cases (3.3%), mixed skin lesions in 2 cases (3.3%), and folliculitis, erythema nodosum, as well as angioedema of the limbs each in 1 case (1.6%). The age of children with different skin manifestations significantly differed ( F = 4.67, P < 0.001). Forty-eight patients (78.69%) presented with generalized skin lesions, while 13 (21.31%) with localized skin lesions; 10 (16.4%) had itching, 3 (4.9%) had a burning sensation, while 48 (78.7%) showed no symptoms. Skin lesions persisted for ≤ 3 days in 36 cases (59.0%) and for > 3 days in 25 cases (41.0%), and all lesions persisted for less than 2 weeks. All 61 patients had fever up to 38.5 ℃; 1 (1.6%) developed skin lesions before the fever, 41 (67.2%) developed lesions during the fever, and 19 (31.2%) developed lesions after the fever. The skin manifestations significantly differed among various groups divided by patients with different lesion distribution, self-reported symptoms, duration of lesions, and sequence between fever and lesion onset (all P < 0.05). No recurrence was observed after recovery, and skin lesions subsided without pigment changes or scaring. Conclusion:COVID-19 was often accompanied by various skin lesions in children, which mainly manifested as urticaria and eruptive/maculopapular lesions.

Pancreatic glucagonoma with metastasis followed up for 15 years: A case report / 中华内分泌代谢杂志

To explore the clinical characteristics, diagnosis, and management of pancreatic glucagonoma, a retrospective analysis of the clinical data and diagnostic algorithm of a patient with pancreatic glucagonoma was conducted, along with literature review. Pancreatic glucagonoma is a rare neuroendocrine tumor that originates from the pancreatic alpha cells. The main manifestations of glucagonoma syndrome(GS) include necrolytic migratory erythema, diabetes, anemia, and other systemic involvement. Early diagnosis of GS is challenging and crucial. Early identification and recognition of skin lesions contribute to timely diagnosis and treatment of the disease. Surgical resection is an effective treatment modality for glucagonoma.

Detection of ATP2C1 gene mutations in a family with generalized familial benign chronic pemphigus / 中华皮肤科杂志

A 60-year-old female proband presented with recurrent erythema, blisters and erosions all over the body for 30 years, which had been aggravated 10 days prior to the presentation. Skin examination showed erythematous swelling of the bilateral eyelids with scattered dark red crusts, scattered erythema and erosions on the nasolabial folds and chin, large areas of erythema and erosions on the neck, bilateral axillae, left cubital fossa, perineum and perianal area, accompanied by bright red granulation tissues and positive Nikolsky′s sign. The proband had two sons, both of whom occasionally presented with erythema and erosions on the axillae and groin, and had not been diagnosed or treated. Blood samples were collected from the proband and her two sons, and genomic DNA was extracted and subjected to whole-exome sequencing. A heterozygous deletion mutation c.955_957del (p.A319del) was identified in the ATP2C1 gene in the proband and her two sons, which had not been previously reported. The patient was finally diagnosed with generalized familial benign chronic pemphigus.

Analysis of clinical and pathological features of 54 adult inpatients with erythema nodosum / 中华皮肤科杂志

Objective:To investigate clinical and histopathological features of adult erythema nodosum (EN) .Methods:Clinical data were collected from 54 adult inpatients with histopathologically confirmed EN in Department of Dermatology and Venereology, the First Affiliated Hospital of Wannan Medical College from November 2019 to July 2022, and analyzed retrospectively.Results:Among the 54 EN patients, there were 6 males and 48 females, their ages were 42.50 ± 11.68 years (range, 18 - 73 years), and their disease course ranged from 1 day to 10 years; 30 patients (55.56%) were diagnosed with idiopathic EN, and 24 (44.44%) with secondary EN. The most common etiological factor in secondary EN was infection (17 cases), including respiratory tract infection (9 cases), tuberculosis infection (6 cases), upper respiratory tract infection comorbid with active hepatitis B virus infection (2 cases) ; the following common etiological factor was connective tissue disease (7 cases), including Behcet′s syndrome (4 cases), Sj?gren′s syndrome (1 case), and undifferentiated connective tissue diseases (2 cases). The patients′ ages were significantly younger in the secondary EN group (38.33 ± 12.15 years) than in the idiopathic EN group (46.17 ± 10.20 years, t = 2.58, P = 0.013). All patients had skin lesions on their lower limbs, lesions were limited to both lower limbs in 24 patients with idiopathic EN and 12 with secondary EN, and the proportion of patients with lesions limited to both lower limbs was significantly lower in the secondary EN group than in the idiopathic EN group ( χ2 = 5.44, P = 0.020). Compared with the idiopathic EN group, the secondary EN group showed significantly increased white blood cell counts ([7.56 ± 2.46] × 10 9/L vs. [6.04 ± 1.60] × 10 9/L, t = 2.62, P < 0.05) and C-reaction protein levels (34.34 ± 46.48 mg/L vs. 11.45 ± 18.13 mg/L, t = 2.28, P < 0.05). In the idiopathic EN group, 23 patients mainly showed histopathological features of septal panniculitis, while 17 patients in the secondary EN group mainly showed histopathological features of mixed panniculitis or lobular panniculitis, and the proportion of patients with histopathological features of mixed panniculitis or lobular panniculitis was significantly higher in the secondary EN group than in the idiopathic EN group ( χ2 = 12.18, P < 0.001) . Conclusion:EN was more common in female adults; idiopathic EN was the most common type, and secondary EN may be a cutaneous sign of systemic diseases; for EN patients at a relatively young age, with lesions involving both lower limbs or more sites, higher white blood cell counts and C-reaction protein levels, and histopathological manifestations of lobular panniculitis, systemic examinations were required to rule out underlying causes.

Bilateral uveitis in silent ulcerative colitis: case report

Silent or subclinical inflammatory bowel diseases (IBD) is a relatively new term that has been used to describe individuals with asymptomatic active mucosal bowel inflammation, often unaware of their disease due to either the lack of or mild inflammatory symptoms. These patients are at risk for gastrointestinal and extra-gastrointestinal manifestations, with more advanced complications. In this article we intend to describe a case report of a patient with chronic history of many organ involvements including ocular, skin, and musculoskeletal, which was later placed under the umbrella of silent ulcerative colitis. (AU)

Láser de baja potencia como alternativa terapéutica al eritema multiforme idiopático. Informe de caso

El eritema multiforme es una enfermedad aguda de la piel y/o de las mucosas de naturaleza inmunológica, siendo está de etiología desconocida. Solo el 20% de los casos se dan en niños. El diagnóstico se basa en la historia clínica y el examen físico y el tratamiento no está sistematizado. La terapia con láser de baja potencia está siendo cada vez más utilizada en el campo estomatológico. El objetivo fue evidenciar los beneficios del láser de baja potencia como alternativa terapéutica. Este caso corresponde a un paciente de 10 años de edad, que acudió a la cátedra de Patología Bucal de la Facultad de Odontología de la Universidad Nacional de Asunción (UNA), derivado del Hospital General de Barrio Obrero con antecedentes de internación por presentar lesiones erosivas en labios, boca y máculas en toráx, abdomen, orejas y miembros. En el examen intraoral costras serohemáticas negruzcas en labios, lesiones ulceradas en lengua, piso de boca, paladar duro y mucosa yugal. Se procedió a hacer una lavado de la zona con agua oxigenada, en las áreas afectadas se colocó azul de metileno al 0,01% por 5 minutos, y para la aplicación de laserterapia se realizó una única sesión. El paciente mostró gran evolución a las 24 horas de la aplicación del tratamiento. A los 8 días estaba sin lesiones y asintomático. La fotobioestimulación a nivel celular que ofrece el láser de baja potencia es una herramienta verosímil que se suma a nuevas opciones terapéuticas.

Erythema multiforme is an acute disease of the skin and/or mucous membranes of an immunologic nature, the etiology of which is unknown. Only 20% of cases occur in children. Diagnosis is based on clinical history and physical examination and treatment is not systematized. Low power laser therapy is being increasingly used in the stomatological field. The objective was to demonstrate the benefits of low power laser as a therapeutic alternative. This case corresponds to a 10-year-old patient, who came to the Department of Oral Pathology of the Faculty of Dentistry of the National University of Asuncion (UNA), referred from the General Hospital of Barrio Obrero with a history of hospitalization for presenting erosive lesions on the lips, mouth and macules on the thorax, abdomen, ears and limbs. In the intraoral examination, blackish serohematic crusts on the lips, ulcerated lesions on the tongue, floor of the mouth, hard palate and jugal mucosa. The area was washed with hydrogen peroxide, methylene blue 0.01% was applied to the affected areas for 5 minutes, and only a single session was performed for the application of the laser therapy. The patient showed great evolution 24 hours after the application of the treatment. After 8 days he was completely free of lesions and asymptomatic. The photo-biostimulation at the cellular level offered by the low power laser is a credible tool that adds to new therapeutic options.

O eritema multiforme é uma doença aguda da pele e/ou mucosas de natureza imunológica, cuja etiologia é desconhecida. Apenas 20% dos casos ocorrem em crianças. O diagnóstico é baseado na história clínica e no exame físico. O tratamento não é sistematizado. A terapia laser de baixa potência é cada vez mais utilizada no campo estomatológico. O objectivo foi demonstrar os benefícios do laser de baixa potência como uma alternativa terapêutica. Este caso corresponde a um paciente de 10 anos de idade que veio ao Departamento de Patologia Oral da Faculdade de Odontologia da Universidade Nacional de Asunción (UNA) e foi encaminhado do Hospital Geral do Bairro Obrero com um historial de hospitalização por apresentar lesões erosivas nos lábios, boca e máculas no tórax, abdómen, orelhas e membros. No exame intraoral, crostas serohaemáticas negras nos lábios, lesões ulceradas na língua, chão da boca, palato duro e mucosa jugal. A área foi lavada com peróxido de hidrogénio, azul de metileno 0,01% foi aplicado nas áreas afectadas durante 5 minutos, e foi realizada uma única sessão de laserterapia. O paciente mostrou uma grande evolução 24 horas após a aplicação do tratamento. Após 8 dias, estava completamente livre de lesões e assintomático. A foto-biostimulação a nível celular oferecida pelo laser de baixa potência é uma ferramenta credível que se soma a novas opções terapêuticas.