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Objective:To compare the clinical efficacy and safety of water jet-assisted dermabrasion versus electric dermabrasion in combination with suction blister epidermal grafting in the treatment of vitiligo.Methods:A total of 60 vitiligo patients were enrolled from the Department of Dermatology, Xijing Hospital from March 2020 to March 2022. Thirty patients firstly received water jet-assisted dermabrasion, 30 firstly received electric dermabrasion, and then all were treated with suction blister epidermal grafting. Follow-up visits were conducted once a month, and the repigmentation of skin lesions and efficacy were evaluated and compared between the two groups 6 months after surgery.Results:There were 30 patients with 312 skin lesions in the water jet-assisted dermabrasion group, including 13 males and 17 females, with the ages and disease duration being 24.41 ± 3.12 years and 5.13 ± 2.34 years respectively; there were 30 patients with 301 skin lesions in the electric dermabrasion group, including 11 males and 19 females, with the ages and disease duration being 22.73 ± 5.11 years and 4.88 ± 2.21 years respectively. No significant differences were observed in the age, gender, disease duration, and dermabrasion sites between the two groups (all P > 0.05). Six months after the operation, 187 (59.94%) skin lesions were healed, 103 (33.01%) were markedly improved, and 22 (7.05%) were improved in the water jet-assisted dermabrasion group; in the electric dermabrasion group, 166 (55.15%) lesions were healed, 108 (35.88%) were markedly improved, and 27 (8.97%) were improved; there was no significant difference in the total response rate between the water jet-assisted dermabrasion group (92.95%) and the electric dermabrasion group (91.03%; χ2 = 0.27, P = 0.602). The water jet-assisted dermabrasion group showed significantly higher degree of repigmentation (90.47% ± 2.53%), matching degree of skin color (3.53 ± 0.21 points), and patient satisfaction scores (3.32 ± 0.27 points) compared with the electric dermabrasion group (82.40% ± 5.33%, 2.71 ± 0.32 points, 2.68 ± 0.41 points, t = 5.30, 8.28, 5.09, respectively, all P < 0.05). No adverse reactions/events were seen in either group. Conclusions:The water jet-assisted dermabrasion combined with suction blister epidermal grafting and electric dermabrasion combined with suction blister epidermal grafting showed similar efficacy in the treatment of vitiligo, with good safety profiles. However, the degree of repigmentation, matching degree of skin color, and patient satisfaction rates were all higher in the patients receiving water jet-assisted dermabrasion than those receiving electric dermabrasion.
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La dermatitis herpetiforme es una enfermedad autoinmune que se caracteriza por la presencia de lesiones vesiculares y prurito en la superficie extensora de las extremidades, nalgas y parte baja de la espalda. Se presenta el caso clínico de una mujer de 21 años, natural y procedente de Iquitos, que presentó múltiples vesículas dolorosas, pruriginosas, de base eritematosa y purulentas en ambos codos durante un mes. Posteriormente, estas lesiones se diseminaron a ambas rodillas y se agregó prurito intenso. La paciente experimentó períodos alternantes de remisión y exacerbación. La correlación del cuadro clínico, resultados serológicos y de biopsia, junto con respuesta terapéutica a la dapsona confirmaron el diagnóstico de dermatitis herpetiforme, con una evolución favorable y remisión de la enfermedad.
Dermatitis herpetiforme is an autoimmune disease characterized by the presence of vesicular lesions and itching on the extensor surface of the limbs, buttocks, and lower back. The clinical case of a 21-year-old woman, a native of and from Iquitos, is presented. She presented multiple painful, itchy vesicles with an erythematous and purulent base on both elbows for a month. Subsequently, these lesions spread to both knees, and intense itching was added. The patient experienced alternating periods of remission and exacerbation. The correlation of the clinical picture, serological and biopsy results, along with the therapeutic response to dapsona, confirmed the diagnosis of dermatitis herpetiforme, with a favorable evolution and remission of the disease.
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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.
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ABSTRACT The Brazilian fauna of Meloidae is poorly studied, even though it includes more than 160 species. In this paper, we aimed at widening the knowledge on four species of blister beetles from this country. Specifically, we defined the uncertain range of Tetraonyx angulicollis, as extended in south-eastern Brazil rather than in Mexico, and implemented the description of the species with figures. We studied the taxonomy and distribution of three almost unknown species of Nemognatha from Brazil, São Paulo State, providing descriptions and figures of sexual characters and colour variability of N. beauregardi, to which is probably referable as a junior synonym of N. plaumanni, of N. rufoscutellaris and of N. cfr. gounellei. Moreover, we assigned these three species to the subgenus Pauronemognatha, recently recorded from South America.
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Desde hace varios años es conocida la infección transmitida por el virus de la viruela símica, caracterizada por afecciones que transitan desde leves lesiones cutáneas hasta afecciones más complejas; sin embargo, desde el mes de mayo, ha habido un rebrote de casos con notificación de alerta epidemiológica a nivel mundial, declarándose luego una emergencia de salud pública por la OMS en el mes de julio. La mayoría de los casos fueron observados en hombres que tienen sexo con hombres lo que sugiere la mayor exposición las relaciones sexuales y eventualmente ha propuesto la interrogante de nuevas mutaciones genéticas. Se presenta el primer caso de Viruela del Mono en Paraguay, en paciente adulto joven, masculino, de compromiso leve, sin repercusión sistémica, y con evolución favorable. Las medidas sanitarias son nuevamente el pilar fundamental para la toma de directrices y manejos protocolizados a fin de alcanzar una correcta vigilancia en salud pública.
The infection transmitted by the monkeypox virus has been known for a few years, characterized by conditions from mild skin lesions to another more complex; however, since the month of May, there has been a resurgence of cases with notification of epidemiological alert worldwide, later declaring a public health emergency by the WHO in July. Most of the cases were observed in men who have sex with men, suggesting the greatest exposure to sexual intercourse and eventually raising the question of new genetic mutations. The first case of Monkeypox in Paraguay is presented, in a young adult male patient, with mild involvement, without systemic repercussions, and with a favorable evolution. Sanitary measures are once again the fundamental pillar for the adoption of protocolized guidelines and procedures in order to achieve proper public health surveillance.
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RESUMEN El lupus eritematoso sistémico ampolloso (LESA) es una enfermedad vesículo-ampollosa mediada por autoanticuerpos en pacientes con lupus eritematoso sistémico (LES). Se observan vesículas y ampollas tensas sobre una piel edematosa, eritematosa y, en ocasiones, normal en cualquier región del cuerpo, incluyendo áreas mucosas y que no han sido fotoexpuestas. Se presenta el caso de un paciente varón de 16 años de edad con nefritis lúpica, que al séptimo día de hospitalización presenta múltiples ampollas serosas y hemorrágicas sobre el rostro, el tronco, el abdomen y las extremidades superiores. El estudio histológico mostró una dermatosis ampollar subepidérmica con numerosos neutrófilos.
ABSTRACT Bullous systemic lupus erythematosus (BSLE) is a vesiculobullous disease mediated by autoantibodies in patients with systemic lupus erythematosus (SLE). Tense vesicles and bullae are seen on an edematous, erythematous and sometimes normal skin in any body region, including mucous membranes and non-photoexposed areas. This is the case of a 16-year-old male patient with lupus nephritis who, on the seventh day of hospitalization, presented multiple serous and hemorrhagic blisters on the face, trunk, abdomen and upper extremities. The histological study showed a subepidermal bullous dermatosis with numerous neutrophils.
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Introdução: a Epidermólise Bolhosa hereditária (EB) é uma doença rara, caracterizada por formações de bolhas na pele e nas mucosas que sofrem mínimos traumas ou aparecem espontaneamente. As principais divisões de EB são Epidermólise Bolhosa Simples, Epidermólise Bolhosa Juncional, Epidermólise Bolhosa Distrófica e a Síndrome de Kindler. As manifestações bucais geralmente encontradas nos pacientes com EB são as bolhas no epitélio oral, microstomia, anquiloglossia, doença periondontal, hipoplasia do esmalte, cárie dentária, atrofia da maxila e prognatismo mandibular, variando para cada tipo da doença. Objetivo: relatar as manifestações bucais dos portadores de Epidermólise Bolhosa residentes no município de Barra da Estiva. Metodologia: trata-se de um estudo transversal, quantitativo e descritivo que foi desenvolvido no município de Barra da Estiva BA com a população de portadores de Epidermólise Bolhosa, através da realização de anamnese e exame clínico. Estatística descritiva foi utilizada para tabular os dados coletados. Resultados: foram analisados 5 portadores de Epidermólise Bolhosa, a maioria do sexo masculino (60%), com média de idade de 5,6 anos. As manifestações bucais encontradas foram bolhas orais (100%), anquiloglossia (100%), língua despapilada (100%), hipoplasia de esmalte (80%), cárie (40%) e abertura de boca limitada em média de 28,6 mm. Conclusão: os portadores de Epidermólise Bolhosa apresentaram manifestações orais características da doença, tendo como consequências maiores dificuldades para realizar higienização bucal e tratamento odontológico, sendo necessário conhecimento prévio dos cirurgiões-dentistas para o atendimento desses pacientes.
Introduction: inherited Epidermolysis Bullosa (EB) is a rare disease, characterized by blistering of the skin and mucous membranes that suffer minimal trauma or appear spontaneously. The main divisions of EB are Simple Epidermolysis Bullosa, Junctional Epidermolysis Bullosa, Dystrophic Epidermolysis Bullosa and Kindler Syndrome. The oral manifestations usually found in patients with EB are blisters in the oral epithelium, microstomy, ankyloglossia, periodontal disease, enamel hypoplasia, dental caries, atrophy of the jaw and mandibular prognathism, varying for each type of disease. Objective: to report the oral manifestations of Epidermolysis Bullosa patients living in the municipality of Barra da Estiva. Methods: cross-sectional study, quantitative and descriptive that was developed in the municipality of Barra da Estiva-BA with the population of people with Epidermolysis Bullosa, through anamnesis and clinical examination. Descriptive statistics was used to tabulate the data collected. Results: 5 children with Epidermolysis Bullosa were analyzed, most male (60%), with an average age of 5,6 years. Oral manifestations found were oral blisters (100%), anquiloglossia (100%), loss of papillae of the tongue (100%), enamel hypoplasia (80%), tooth decay (40%) and mouth opening limited to the average of 28.6 mm. Conclusion: epidermolysis Bullosa children presented oral manifestations common to the disease, leading to difficulties in performing oral hygiene and dental treatment, requiring prior knowledge of dental surgeons for the care of these patients.
الموضوعات
Humans , Male , Female , Child, Preschool , Child , Oral Manifestations , Blister , Epidermolysis Bullosa , Dental Enamel Hypoplasia , Microstomia , Demography , Epidemiology, Descriptive , Evaluation Studies as Topicالملخص
Objective:To explore the etiology and treatment of craniopharyngioma with aneurysm.Methods:Seven cases of craniopharyngioma with aneurysm from March 2014 to October 2019 treated in Sanbo Brain Hospital, Capital Medical University were retrospectively analyzed. Among the 7 patients, there were 5 males and 2 females. There were 4 cases of recurrent craniopharyngiomas, 1 case of primary tumor and 2 cases of non-recurrence tumor. Three patients with blood blister-like aneurysms were treated with microsurgical suture after craniopharyngioma resection. Among the three cases with internal carotid artery fusiform aneurysm, 1 case underwent craniopharyngioma resection after internal maxillary artery-radial artery-middle cerebral artery bypass and isolation of the aneurysm; 1 case only underwent internal maxillary artery-radial artery-middle cerebral artery bypass and isolation of the aneurysm for non-recurrence tumor; 1 case underwent craniopharyngioma resection and dynamic observation of aneurysm. One case with a cystic aneurysm of the middle cerebral artery was clipped and the craniopharyngioma did not relapse.Results:All patients had no serious postoperative complications. During the follow-up period, there was no recurrence of craniopharyngioma, no recurrence of treated aneurysms, and the stability of aneurysms was observed.Conclusions:Inflammatory stimulation of craniopharyngioma cystic fluid and operation itself are the important reasons for the occurrence of aneurysms after craniopharyngioma surgery. Choosing appropriate surgical methods can complete the removal of craniopharyngioma and the treatment of aneurysms at one time.
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@#Full thickness skin graft is a simple and reliable method for closure of small facial wound defect. A thorough understanding of how a skin graft heals and how to perform the procedure is essential for successful outcome. We report the use of full thickness skin graft in a wound closure of a facial skin defect caused by Paederus fuscipes, locally known as charlie. An 8-year old boy developed blister and painful swelling over his right cheek following skin contact with charlie. This lesion gradually became extensive, eventually leading to tissue loss and facial wound defect. A full thickness skin grafting was performed with satisfactory functional and excellent aesthetic result.
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Resumo A bullosis diabeticorum (BD) é uma manifestação cutânea infrequente do diabetes e pode afetar os membros superiores. Caracteriza-se pelo aparecimento espontâneo e indolor de flictenas não inflamatórias de aspecto sanguinolento, que podem evoluir para necrose, requerendo que se faça diagnóstico diferencial com doenças dermatológicas, como porfiria cutânea tarda, pseudoporfiria, epidermólise bolhosa adquirida e penfigoide bolhoso, e doenças vasculares, como vasculites, doença arterial periférica, doença de Buerger, entre outras. Neste relato, descreve-se o caso de um paciente masculino de 77 anos, hipertenso e diabético mal controlado, apresentando aparecimento espontâneo de lesões bolhosas de membros superiores, que evoluíram, após ruptura espontânea, para ulcerosas necróticas. A biópsia da lesão diagnosticou a presença de espessamento hialino de vasos dérmicos e de bolhas subcórneas, compatíveis com o diagnóstico de BD. Após a interrupção do tabagismo e a otimização do controle glicêmico associado a corticoterapia tópica, houve melhora, com cicatrização das lesões. A apresentação da BD em membros superiores é mais rara, sendo necessário o diagnóstico diferencial com outras lesões cutâneas e vasculares.
Abstract Bullosis diabeticorum (BD) is an uncommon cutaneous manifestation of diabetes that can affect the upper limbs. It is characterized by spontaneous and painless non-inflammatory bloody blisters, which can progress to necrosis, requiring differential diagnosis to rule out other dermatological diseases, such as porphyria cutanea tarda, pseudoporphyria, epidermolysis bullosa acquisita, and pemphigoid, and vascular diseases, such as vasculitis, peripheral arterial disease, and Buerger's disease, among others. In this report, we describe a 77-year-old male patient with poorly controlled diabetes and hypertension who presented with spontaneous onset of lesions on the upper limbs, initially with bullous characteristics, progressing to necrotic ulcers after spontaneous rupture. A biopsy revealed hyaline thickening of the dermal vessels and subcorneal bullae, consistent with a diagnosis of BD. After smoking cessation and optimization of glycemia control combined with topical corticosteroid therapy, the condition improved and lesions began to heal. This presentation of BD involving the upper limbs is rare, requiring differential diagnosis to rule out other cutaneous and vascular lesions.
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The presence of tension blister often predicts severe soft tissue damage,which not only increases the risk of wound complications but also prolongs the surgical treatment time. However,the developed tension blister has been proposed as a potential decompressive approach for it may relieve the pressure of osteofascial compartment and improve the likelihood of relieving clinical symptoms,as well as avoid unnecessary surgery in cases of suspected osteofascial compartment syndrome. Recently,the osteofascial system has been increasingly recognized that associations were found between the tension blister and osteofascial self-release processing. Thus,the timing of blister occurrence and regression substantially influences physicians′ clinical decisions,making blister management as part of the treatment of fractures. In this review,the authors give an overview of the characteristics,mechanism,stress reduction effect,prevention,current treatment status and complications of the fracture-related tension blister,hoping to help orthopedic physicians understand and treat the tensile blister.
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In this paper,we reviewed the various advanced technologies and methods that could help patients for measuring adherence of patients.There exist intelligent technologies that are available for measuring medication adherence,including medication event monitoring system (MEMS(R)),smart blister packs,radio frequency identification(RFID) embedded smart drawers,and wisely aware RFID dosage (WARD) system.Utilization of these advanced technologies and systems have aided in enhancing the adherence to a greater extent.For example,MEMS(R) refers to the electronic cap that counts the number of bottles opened,but it can be employed only with bottles.Smart blisters are pharmaceutical packagings that possess the capability of monitoring when a pill or tablet is taken out of its packing.All those intelligent technologies can help in active monitoring of patients regarding adherence and capable of eradicating various medication errors due to which adherence is affected.
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Objective:To investigate the clinical significance of suction blister transplantation in improving the efficacy of ReCell technique in the treatment of vitiligo.Methods:Patients were divided into three groups, namely, vitiligo patients without history of suction blister therapy, patients with ineffective suction blister therapy and patients with effective suction blister therapy. There were 30 patients in each group. All patients were treated with standard procedure of ReCell technique. The color recovery effect of leukoplakia was observed 3 and 6 months after operation, and the incidence of complications was also observed.Results:The effective rate of color recovery 3 and 6 months after operation were as follow: in patients without history of suction blister group, the effective rate of three months was 53.3%, and that of six months was 63.3%; in patients with ineffective suction blister group, the effective rate was 43.3% in three months and 50.0% in six months, and in patients with effective suction blister group, the effective rate was 76.7% in three months and 90.0% in six months. No obvious complications were observed in the three groups.Conclusions:For the treatment of stable vitiligo with ReCell technique, suction blister method is a simple and effective method for screening patients.
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Abstract Introduction: Henoch Schönlein purpura (HSP) is the most common type of vasculitis in childhood. HSP affects small blood vessels, and it rarely leads to serious complications such as bullous small vessel vasculitis, as it occurred in the case presented here. Case presentation: 5-year-old male who was brought to a primary healthcare center due to having arthralgia and purple skin lesions on his lower limbs. After the patient was diagnosed with HSP, he developed bullous lesions, so he was hospitalized and analgesic and topical management was started. During his hospital stay, the patient's renal function was monitored, and since he did not experience other complications, he was discharged. Conclusion: The available literature on HSP suggests that its cutaneous bullous manifestation rarely occurs in pediatric population and that, unlike normal HSP cases, it is not always associated with renal and/or gastrointestinal involvement. However, regardless of the dermatological severity of this type of vasculitis, the function of the gastrointestinal and renal systems must be always monitored in these patients.
Resumen Introducción. La púrpura Henoch-Schönlein (PHS) es la forma más común de vasculitis en la infancia; esta se da en pequeños vasos sanguíneos y no es frecuente que genere complicaciones graves como la vasculitis bullosa, tal como sucedió en el caso que se presenta a continuación. Presentación del caso. Paciente masculino de cinco años que fue traído a un centro de atención primaria con un cuadro clínico consistente en artralgias y aparición de lesiones purpúricas en miembros inferiores. Luego de ser diagnosticado con PHS, presentó lesiones bullosas, por lo que fue hospitalizado y se inició manejo analgésico y tópico; durante su estadía en el hospital se vigiló su función renal y, ya que no presentó otras complicaciones, se dio de alta. Conclusión. Las publicaciones disponibles sobre PHS sugieren que su presentación cutánea bullosa en pediatría no es frecuente y que no siempre se relaciona con un compromiso renal y/o gastrointestinal como la variante clásica; sin embargo, siempre debe vigilarse la función de estos sistemas sin importar la gravedad dérmica de esta vasculitis.
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La mastocitosis comprende un conjunto de desórdenes con expansión anormal y acumulación de mastocitos en diversos órganos incluida la piel, en la cual se describen distintas formas clínicas de presentación. El mastocitoma cutáneo solitario constituye el 10-15% de todas las mastocitosis. Presentamos el caso de un niño con mastocitoma cutáneo solitario ampollar, un cuadro infrecuente cuyo diagnóstico oportuno constituye un reto para pediatras y dermatólogos (AU)
Mastocytosis comprises a set of disorders with abnormal expansion and accumulation of mast cells in various organs including the skin, wich describes different form of clinical presentation. The solitary cutaneous mastocytoma constitutes 10-15 % of all mastocytosis. We present the case of a child with solitary blistering mastocytoma, an infrequent condition whose, timely diagnosis constitutes a challenge for pediatricians and dermatologists (AU)
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Humans , Male , Infant , Mastocytosis , Mastocytoma, Skinالملخص
IINTRODUCCIÓN: el bullosis diabeticorum forma parte del espectro de manifestaciones cutáneas de la diabetes mellitus, descrito por Kramer en 1930 y nombrado como bullosis diabeticorum por Cantwell y Martz. Es una enfermedad conocida, pero bastante rara (0.5 a 2% de la población diabética) siendo dos veces más común en hombres. Es una lesión espontánea y no está relacionada con trauma o causa fisiológica evidente, como infección, 3 que no causa dolor o se asocia a signos flogísticos. Se asocia principalmente a las extremidades, pudiendo ser una lesión única o múltiples lesiones. Puede ocurrir en la diabetes mellitus tipo 1 (DM1) y en el tipo 2. Las lesiones típicamente se curan espontáneamente de 2 a 6 semanas, pero pueden ocurrir en el mismo lugar nuevamente. El pronóstico es bueno, sin tratamiento específico necesario o seguimiento diagnóstico con biopsia. Se relata un caso de esta rara patología con documentación por resonancia magnética.
Bullosis diabeticorum is part of the spectrum of cutaneous manifestations of diabetes mellitus, described by Kramer in 1930 and named bullosis diabeticorum by Cantwell and Martz. It is a known disease, but quite rare (0.5 to 2% of the diabetic population) and is two times more common in men. Bullosis diabeticorum is a spontaneous lesion and not related to trauma or obvious physiological cause, such as infection that does not cause pain or is associated with inflammatory signs. Occurs at the limbs and may be single or multiple. It can occur in diabetes mellitus type 1 (DM1) and type 2, usually in the advanced stages. The lesions heal spontaneously typically in 2 to 6 weeks, but they may happen again on the same site. The prognosis is good with no need of special treatment or further diagnosis with biopsy. We report a case of this rare complication with magnetic resonance imaging.
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Humans , Male , Aged , Blister/diagnostic imaging , Diabetic Foot/diagnostic imaging , Diabetes Mellitus, Type 2/complications , Magnetic Resonance Imaging , Blister/etiology , Blister/pathologyالملخص
El penfigoide gestacional es una dermatosis rara, que se presenta durante el embarazo. Se caracteriza por una respuesta autoinmune contra las proteínas de los hemidesmosomas, que genera un clivaje entre la epidermis y la dermis tanto de la piel como de las mucosas. Clínicamente, presenta prurito intenso, placas y pápulas eritematosas, que evolucionan a apollas con distribución en el abdomen y los miembros. Como complicaciones, en el feto puede generar parto prematuro y bajo peso para la edad gestacional, con alto riesgo de mortalidad. (AU)
Gestational pemphygoid is a rare, autoimmune dermatosis that occurs during pregnancy. It is characterized by an autoimmune response against hemidesmosome proteins, generating a cleavage between the epidermis and the dermis in the skin and mucous membranes. Clinically it presents with intense pruritus, plaques and erythematous papules that evolve to blisters that are distributed mainly in the abdomen and limbs. The complications are preterm birth and low weight for gestational age, with high risk of mortality. (AU)
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Humans , Male , Female , Pregnancy , Infant, Newborn , Adult , Pemphigoid Gestationis/diagnosis , Pemphigoid, Bullous/diagnosis , Prednisone/analogs & derivatives , Pemphigoid Gestationis/drug therapy , Pemphigoid, Bullous/etiology , Pemphigoid, Bullous/drug therapy , Diabetes, Gestational/diagnosis , Glucocorticoids/therapeutic useالملخص
Resumen: Este es un caso de dos pacientes femeninas fruto de un embarazo gemelar bicorial y biamniótico, con diagnóstico de epidermólisis bullosa congénita tipo distrófica, que se confirmó genéticamente con la mutación patogénica en el gen COL7A, una variante previamente no reportada y también llamada la variante Hallopeau-Siemens. Las pacientes fueron manejadas por un grupo médico interdisciplinario, enfocando el manejo en prevenir la aparición de nuevas lesiones y complicaciones, y en el manejo del dolor. Debido a la gravedad de las lesiones y las complicaciones asociadas, una de los pacientes murió. La otra está viva a los 8 meses de edad, sin complicaciones graves y con buen estado nutricional. En estos pacientes el tratamiento de las lesiones cutáneas es el pilar del manejo para reducir la morbimortalidad. El objetivo principal de este texto es proporcionar información para que los profesionales de la salud conozcan la enfermedad, pueda ser diagnosticada oportunamente y así brindar un tratamiento de apoyo a los pacientes y sus familias; y sensibilizar sobre una de las enfermedades clasificadas como huérfanas o raras en nuestro país.
Abstract: We present the case of two female patients from a dichorionic diamniotic twin pregnancy with diagnosis of congenital dystrophic epidermolysis bullosa at Clinica Universitaria Bolivariana, Medellín, Colombia. Molecular genetic testing confirmed a pathogenic mutation in the gene COL7A, a variant previously not reported and also called the Hallopeau-Siemens variant. Patients were followed by an interdisciplinary medical team focusing on prevention of new lesions, complications, and pain management. Because of the severity of the lesions and associated complications one of the patients died, the other one is 8-months-old without severe complications and well nutritional status. In these patients, treatment of skin lesions is the mainstay to reduce morbidity and mortality. The main purpose of this report is to provide information so that health professionals know the disease and it can be diagnosed opportunely and thus provide supportive treatment to patients and their families; and also sensitize health providers about one of the diseases listed as orphan or rare in our country.
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Skin involvement in systemic lupus erythematosus (SLE) occurs in more than 75% of patients with this condition. Vesicles and blisters in lupus erythematosus (LE) may be present in SLE secondary to interface vacuolar changes in the epidermis, in discoid LE also secondary to vacuolar epidermal changes, and in bullous LE secondary to antibodies anti-collagen VII deposits with neutrophilic aggregates. In addition, blisters can occur due to the association of SLE with other autoimmune blistering diseases (e.g. bullous pemphigoid). BSLE is a rare blistering disease that mainly occurs in females (3040 years old), and less frequently in children and adolescents. The most common presentation is rapid and widespread development of tense vesicles and bullae over erythematous macules or plaques. Preferential sites are: superior trunk, proximal superior limbs, and face (lips) with symmetrical distribution. Mucosal involvement is common on perioral, pharyngeal, laryngeal, and genital areas. The involvement of sun-exposed areas is not mandatory. The lesions usually progress with no scarring, but hypo or hyperchromia may be present. We report an 18-year-old female patient with blistering lesions at admission, who was diagnosed with BSLE. She was initially treated with systemic prednisone and hydroxychloroquine. Her condition evolved with relapsing lesions, which required the introduction of Dapsone. The authors emphasize the relevance of recognizing BSLEa rare presentation of SLEwhich may evolve with marked clinical presentation
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Humans , Female , Adolescent , Skin Diseases, Vesiculobullous , Lupus Erythematosus, Systemic/diagnosis , Blister , Rare Diseasesالملخص
The Aeromonas species, belonging to the family Aeromonadaceae, are opportunistic pathogens found in humans with an incidence rate of 76 cases per million inhabitants in Southern Taiwan. The incidence of Aeromonas septicemia is relatively low, accounting for less than 15% of cases. Patients diagnosed with Aeromonas hydrophila bacteremia who were presented with skin blisters and septic shock have been reported to have a mortality rate of 100%. Aeromonas infection must be considered in the differential diagnosis of gangrene-like tissue damage or skin lesions in patients with end-stage renal disease, due to the potential sources of infections. A 49-year-old Taiwanese diabetic woman with end-stage renal disease had underwent regular hemodialysis. She was referred to our hospital due to a one-day course of fever, dyspnea, hypotension, and fulminant hemorrhagic blisters covering her whole body. A physical examination uncovered multiple hemorrhagic blisters, along with a ruptured blister over the lower left leg. Laboratory tests revealed an elevation of liver enzymes, impaired renal function, lactatemia, and high anion-gap metabolic acidosis. Cultures of both blood and hemorrhagic blister fluid grew Aeromonas hydrophila. However, she experienced persistent shock despite aggressive intravenous fluid, empiric antibiotics, and inotropic agents with norepinephrine and dopamine. Early diagnosis and prompt management using intravenous fluids, antibiotics and surgical debridement is recommended in order to improve a patient's survival rate.