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.

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.

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.

Eritema Multiforme, Una Rara Presentación De Exantema En Población Pediátrica. A Propósito De Un Caso. / Erythema Multiforme, A Rare Occurrence Of Exanthema In The Pediatric Population. On The Subject Of A Case.

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

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

Bartonella henselae as a putative trigger for chronic type 2 leprosy reactions

Abstract Leprosy reactions are an acute inflammatory phenomenon that can arise before diagnosis, during treatment, or after cure of leprosy. These reactions are considered one of the main diseases that cause physical disabilities. Immunosuppressive treatment for these immune responses makes these patients susceptible to coinfections, which can trigger new leprosy reactions. The main objective of this study was to evaluate the occurrence of infection by Bartonella sp. in blood samples from 47 patients who had untreatable episodes of type 2 leprosy reactions for more than six months, comparing them with a control group. Cultures and molecular methods (PCR) were used. Amplicons from species-specific reactions and sequencing showed a higher prevalence of Bartonella henselae infection in patients, 19/47 (40.4 %), compared to control, 9/50 (18.0 %), p= 0.0149. Five patients accepted treatment for coinfection, and all showed improvement in leprosy reactions with treatment for B. henselae infection. We conclude that these bacteria can trigger chronic reactions of type 2 leprosy and should be investigated in these patients. Summary line Patients who have chronic type 2 leprosy reactions are more susceptible to Bartonella henselae infection than controls: 19/47 (40.4 %) compared 9/50 (18.0 %), p= 0.0149.

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.

Post nirmatrelvir/ritonavir erythema multiforme in a patient with coronavirus disease infection

ABSTRACT Erythema multiforme (EM), an immune-mediated skin condition, can occur after infection or following the use of medications. In this study, we describe a patient who developed EM after nirmatrelvir/ritonavir administration. An 81-year-old woman presented with fever and dyspnea. Laboratory investigations showed positive coronavirus disease (COVID-19) based on polymerase chain reaction assay, and she received a 5-day regimen of nirmatrelvir/ritonavir. We observed development of EM after this treatment and initiated prednisone (1 mg/kg) therapy, which led to rapid improvement. Our study is the first to report EM in a patient with COVID-19, who received nirmatrelvir/ritonavir and showed a favorable response.

Vasculite por tuberculose ocular associada a eritema nodoso e à doença de Poncet / Ocular tuberculosis vasculitis associated with erythema nodosum and Poncet's disease

RESUMO A tuberculose intraocular deve ser sempre aventada como diagnóstico diferencial devido à sua alta significância nos agravos, além da alta morbidade da infecção sistêmica. Essa condição pode se apresentar associada a manifestações extraoculares pouco prevalentes da tuberculose. O autor relatou um caso de vasculite por tuberculose ocular, associada a eritema nodoso e à doença de Poncet, com resolução dos achados e sintomas após esquema padrão para tuberculose.

ABSTRACT Intraocular tuberculosis should always be deemed as a differential diagnosis due to its high importance, in addition to the high morbidity of systemic infection. This condition may be associated with extraocular manifestations that are not prevalent in tuberculosis. The author reported a case of ocular tuberculosis vasculitis associated with erythema nodosum and Poncet's disease, with resolution of the medical findings and symptoms after the standard treatment for tuberculosis.

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.

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.

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.

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.

Father-Daughter Duo: How Unmasking Leprosy in Father Helped in Leprosy Diagnosis in the Daughter - A Case Report

In highly endemic countries like India, where tuberculosis (TB) and leprosy infection may coexist, screening the other disease before initiating treatment is important to prevent Rifampicin resistance since both diseases are treated with and sensitive to Rifampicin. Here, we report a leprosy case involving the unmasking of leprosy in a treated patient with Pulmonary TB. In this case, a high index of suspicion of Erythema Nodosum Leprosum (ENL) in a patient with no history of leprosy disease or treatment with anti-leprosy drugs was observed. He, however, had a history of taking anti-tuberculous medicine 1.5 years earlier. This case report also acknowledges the physician’s prompt referral of this patient to a dermatologist. Taking a detailed family history and screening helped us diagnose leprosy in the patient’s daughter. It also emphasises the atypical presentation of leprosy, which (although described in textbooks) is being reported here.

The relationship between erythema nodosum and prognosis in systemic sarcoidosis: a retrospective cohort study

Abstract Background: Erythema Nodosum (EN) is the most common skin manifestation in sarcoidosis and has often been associated with a good prognosis. Objectives: To compare the clinical characteristics and treatment-related features in patients with sarcoidosis according to whether or not EN was seen as a presenting symptom at the time of diagnosis. Methods: A 20-year single-center retrospective study was performed. The following two groups were identified: one group with EN as one of the presenting symptoms at the time of diagnosis of sarcoidosis (EN group) and a second group without EN as a presenting symptom at diagnosis (non-EN group). The clinical characteristics and treatment modalities were collected from the medical records. Results: A total of 122 patients (31 in the EN group, 91 in the non-EN group) were included. Radiological stages of pulmonary disease were significantly lower in the EN group. Articular involvement was more common in the EN group (p = 0.001), whereas other systemic organ involvements (p = 0.025), especially neurological involvement (p = 0.036), were significantly more common in the non-EN group. In the EN group, a higher percentage of patients were managed without systemic therapy (71.0% vs. 54.9%) and spontaneous remission was more frequent (25.0% vs. 14.1%), however, this wasn't statistically significant. Study limitations: Retrospective design. Conclusions: The lower radiological stage of pulmonary sarcoidosis and lower frequency of systemic organ involvement in patients with EN augment the prognostic value of EN highlighted in the literature. However, this study couldn't confirm that the patients with EN would need less systemic therapy in the course of their disease.

Exacerbation of Erythema Nodosum Leprosum Possibly Triggered by COVID-19 Vaccine : A Case Repor

Erythema nodosum leprosum (ENL) is an immune complex mediated type III hypersensitivity reaction seen in patients of borderline lepromatous and lepromatous leprosy. It can be caused by a wide array of triggers and can be seen before, during, or after completion of anti-leprosy therapy. There are multiple well-known triggers for type 2 reactions like the initiation of multidrug therapy, Mantoux testing, vaccination, mental and physical stress, and physiological states like pregnancy. Herein, we report a case of exacerbation of ENL in a middle-aged woman, probably due to COVID-19 vaccine while she was well-controlled on immunosuppressive therapy. The episode was treated with non-steroid anti-inflammatory drugs and oral steroids and the symptoms resolved within 2 weeks. Although causality was highly possible between the occurrence of ENL and COVID-19 vaccine, physicians should be aware that it can be easily managed with proper care and medicines and this should not be a basis for deferring the vaccine.