ABSTRACT
Introducción: el penfigoide de las mucosas (PM), antes llamado mucoso, cicatrizal o mucosinequiante, representa un grupo heterogéneo de enfermedades ampollares autoinmunes inflamatorias crónicas que comprometen las mucosas o la piel, con tendencia a dejar secuelas cicatrizales. Existen autoanticuerpos contra distintos componentes de la zona de la membrana basal (BPAG1, BPAG2, integrina α6ß4, laminina 332, colágeno VII, entre otros), por lo que la inmunofluorescencia directa (IFD) es de suma importancia, así como la clínica, para su diagnóstico.Objetivo: realizar una revisión de los casos de PM diagnosticados durante un período de 24 años (enero de 1997- marzo de 2021) en el Sector de Enfermedades Ampollares del Hospital Ramos Mejía para determinar la epidemiología, la clínica y la terapéutica de esta enfermedad.Diseño: estudio retrospectivo descriptivo y observacional, en el que se analizaron las características clínicas e inmunopatológicas de 34 pacientes con diagnóstico de PM atendidos en el Servicio de Dermatología del Hospital Ramos Mejía desde enero de 1997 hasta marzo de 2021. Materiales y métodos: mediante las historias clínicas y los regis-tros iconográficos, se evaluaron las siguientes variables: prevalencia del diagnóstico de PM en los pacientes atendidos en el Sector, sexo, edad, antecedentes personales, mucosas afectadas, tiempo de evolución hasta el diagnóstico, hallazgos en la IFD, seguimiento clínico y tratamientos instaurados. Resultados: se estudió la evolución clínica de 34 pacientes diagnosticados con PM (5,3% del total de pacientes evaluados en el Sector de Patologías Ampollares). El sexo más afectado fue el femenino y la edad promedio en el momento del diagnóstico fue de 64 años. El 70,6% de los pacientes presentaron comorbilidades asociadas como hipertensión e hipotiroidismo. La mayoría refirió algún evento emocional como factor desencadenante. El sitio más comprometido fue la mucosa ocular y la cavidad oral fue la segunda en frecuencia. El tiempo de evolución promedio hasta el momento del diagnóstico fue de 4 años y 11 meses. El hallazgo más frecuente en la IFD fue la IgG lineal. El 17,6% de los pacientes interrumpieron el seguimiento clínico. El tratamiento más utilizado fue el mofetil micofenolato, con el que se obtuvo buena respuesta terapéutica. Conclusiones: el PM es una enfermedad autoinmune infrecuente que compromete las mucosas y, ocasionalmente, la piel. En este estudio, se observó que la principal mucosa afectada fue la conjuntival, a diferencia de lo referido en la bibliografía internacional dermatológica. El diagnóstico interdisciplinario temprano es fundamental para evitar las secuelas irreversibles.
Introduction: mucous membrane pemphigoid (MMP), also known as benign mucous membrane pemphigoid, cicatricial or mucosynechial pemphigoid, belongs to an heterogeneous group of chronic inflammatory autoimmune blistering diseases, which involves the mucous membranes (oral, ocular, pharyngeal, nasal, esophageal, laryngeal and anogenital) and/ or skin with tendency to scar formation. There are autoantibodies against different components of the basement membrane zone (BPAG 1- BPAG2, Integrin α6ß4, Laminin 332, Col VII, among others). The direct immunofluorescence (DIF) will be of paramount importance, as well as the clinical diagnosis.Objective: review the cases diagnosed with mucous membrane pemphigoid for 24 years (January 1997- March 2021) in the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital to establish the epidemiologic, clinic presentation and available treatments in this pathology.Design: retrospective descriptive and observational study of the clinical and immunopathological characteristics of 34 patients with MMP that were treated at the Blistering Disease Clinic at the Dermatology Department at the Ramos Mejia Hospital between January 1997 and March 2021.Materials and methods: though the medical histories and the photographic registries, we evaluated the following variables: prevalence of MMP within the patients that came to consult at the Blistering Disease Clinic at the Dermatology Department, sex, age, personal history, the affected mucous, evolution time until the diagnosis, direct immunofluorescence findings, clinical follow-ups and treatments.Results: we studied the clinical evolution of 34 patients diagnosed with MMP at our institution (5.3% from the total of patients at the Blistering Disease Clinic).The most affected gender was female and the average age at diagnosis was 64 years. 70.6% presented comorbidities such as hypertension and hypothyroidism. Most of our patients referred an emotional triggering event. The most affected membrane mucous was the ocular one and the oral was the second one. The delay in diagnosis was 4 years and 11 months. Linear deposits of IgG was the most frequent result in the direct immunofluorescence. 17.6% did not continue clinical follow-up. Mycophenolate mofetil was the most used drug with a good therapeutic response. Conclusions: MMP is a rare autoimmune disease that affects mucous membrane and occasionally the skin. In this study, the ocular involvement was the most frequent one, differing with the international reports. The early interdisciplinary diagnosis is essential to avoid irreversible sequelae.
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Aged , Aged, 80 and over , Pemphigoid, Benign Mucous Membrane , Pemphigoid, Bullous/diagnosis , Esophageal Mucosa , Mouth MucosaABSTRACT
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)
Subject(s)
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 useABSTRACT
Abstract Psoriasis has been associated with various autoimmune diseases, however, its relation to bullous diseases is infrequent. Of these, bullous pemphigoid appears as the main associated entity, even though both conditions differ considerably in demographic and clinical aspects. We report the case of a 42-year-old female patient, with long-standing psoriasis who consulted due to the exacerbation of psoriatic plaques associated with generalized bullous lesions on the skin and oral mucosa, with one-week duration. With clinical signs and histopathological findings compatible with bullous pemphigoid associated with psoriasis, we decided to treat her with methotrexate 10mg a week. The patient had an excellent response after two months of treatment.
Subject(s)
Humans , Adult , Psoriasis/drug therapy , Methotrexate/therapeutic use , Pemphigoid, Bullous/drug therapy , Dermatologic Agents/therapeutic use , Psoriasis/complications , Psoriasis/diagnosis , Pemphigoid, Bullous/complications , Pemphigoid, Bullous/diagnosis , Treatment Outcome , Fluorescent Antibody Technique, DirectABSTRACT
Abstract: Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals. Increase in incidence rates in the past decades has been attributed to population aging, drug-induced cases and improvement in the diagnosis of the nonbullous presentations of the disease. A dysregulated T cell immune response and synthesis of IgG and IgE autoantibodies against hemidesmosomal proteins (BP180 and BP230) lead to neutrophil chemotaxis and degradation of the basement membrane zone. Bullous pemphigoid classically manifests with tense blisters over urticarial plaques on the trunk and extremities accompanied by intense pruritus. Mucosal involvement is rarely reported. Diagnosis relies on (1) the histopathological evaluation demonstrating eosinophilic spongiosis or a subepidermal detachment with eosinophils; (2) the detection of IgG and/or C3 deposition at the basement membrane zone using direct or indirect immunofluorescence assays; and (3) quantification of circulating autoantibodies against BP180 and/or BP230 using ELISA. Bullous pemphigoid is often associated with multiple comorbidities in elderly individuals, especially neurological disorders and increased thrombotic risk, reaching a 1-year mortality rate of 23%. Treatment has to be tailored according to the patient's clinical conditions and disease severity. High potency topical steroids and systemic steroids are the current mainstay of therapy. Recent randomized controlled studies have demonstrated the benefit and safety of adjuvant treatment with doxycycline, dapsone and immunosuppressants aiming a reduction in the cumulative steroid dose and mortality.
Subject(s)
Humans , Aged , Pemphigoid, Bullous/diagnosis , Steroids/therapeutic use , Autoimmunity/physiology , Fluorescent Antibody Technique/methods , Pemphigoid, Bullous/classification , Pemphigoid, Bullous/etiology , Pemphigoid, Bullous/drug therapy , Diagnosis, DifferentialABSTRACT
Abstract: Bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita are subepidermal autoimmune blistering diseases whose antigenic target is located at the basement membrane zone. Mucous membrane pemphigoid and epidermolysis bullosa acquisita can evolve with cicatricial mucosal involvement, leading to respiratory, ocular and/or digestive sequelae with important morbidity. For each of these dermatoses, a literature review covering all therapeutic options was performed. A flowchart, based on the experience and joint discussion among the authors of this consensus, was constructed to provide treatment orientation for these diseases in Brazil. In summary, in the localized, low-risk or non-severe forms, drugs that have immunomodulatory action such as dapsone, doxycycline among others may be a therapeutic option. Topical treatment with corticosteroids or immunomodulators may also be used. Systemic corticosteroid therapy continues to be the treatment of choice for severe forms, especially those involving ocular, laryngeal-pharyngeal and/or esophageal mucosal involvement, as may occur in mucous membrane pemphigoid and epidermolysis bullosa acquisita. Several immunosuppressants are used as adjuvant alternatives. In severe and recalcitrant cases, intravenous immunoglobulin is an alternative that, while expensive, may be used. Immunobiological drugs such as rituximab are promising drugs in this area. Omalizumab has been used in bullous pemphigoid.
Subject(s)
Humans , Epidermolysis Bullosa Acquisita/drug therapy , Pemphigoid, Benign Mucous Membrane/drug therapy , Pemphigoid, Bullous/drug therapy , Consensus , Prognosis , Societies, Medical , Brazil , Epidermolysis Bullosa Acquisita/diagnosis , Pemphigoid, Benign Mucous Membrane/diagnosis , Pemphigoid, Bullous/diagnosis , Adrenal Cortex Hormones/therapeutic use , Dermatology , Immunosuppressive Agents/therapeutic use , Anti-Inflammatory Agents/therapeutic useABSTRACT
El penfigoide ampollar en niños es extremadamen-te infrecuente. La mayoría de los casos ocurre en adultos mayores, resultando fundamental conside-rar que en medicina todo es posible y esta enferme-dad igualmente podría presentarse en niños, por lo que se debe prestar especial atención a sus manifes-taciones clínicas, realizar los exámenes apropiados para descartar diagnósticos diferenciales y de esta forma, iniciar un tratamiento eficaz en el momen-to preciso. A continuación, presentamos un caso clínico de penfigoide ampollar que ocurrió en un paciente pediátrico atendido en nuestro hospital universitario.
Bullous pemphigoid in children is extremely in-frequent. Most of them predominate in elderly persons, resulting fundamental to consider that in medicine everything could be possible, and this disease could occur in children, so we must pay special attention to its clinical manifestations, to take the appropriate exams to rule out differen-tial diagnoses and in this way perform an effective treatment at the precise moment. Following, we present a clinical case of bullous pemphigoid that occurred in a pediatric patient attended at our university hospital.
Subject(s)
Humans , Female , Child , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/drug therapy , Prednisone/therapeutic use , Diagnosis, DifferentialABSTRACT
Pemphigus foliaceus (PF) is the most common autoimmune skin disease in dogs. It is characterized by pustules, erosions, and crusts which occur due to the presence of autoantibodies that target intercellular adhesion. Histopathological examination is considered the gold standard pattern in the diagnosis, but may sometimes be inconclusive, especially when the characteristic findings are not identified. New diagnostic tests are continuously being developed and immunofluorescence assays, could be a valuable alternative diagnostic tool. This study aimed to evaluate the applicability of direct and indirect immunofluorescence (DIF and IIF) tests for the diagnosis of canine PF. Twenty eight dogs were divided into two groups: Group I with 14 dogs with PF and Group II (control) with 14 dogs with Superficial pyoderma (differential diagnoses of PF). All animals were submitted to skin biopsy to histopathological and DIF. Blood samples were collected to assess IIF. Comparing the DIF results against the histopathology test, there was an agreement of 75% (9/12) with a Kappa index of 0.77 (P<0.001). Considering IIF, the agreement was 100% (14/14), with a Kappa index of 1.0 (P<0.001). We conclude that DIF and IIF are highly effective and were useful and effective complementary examination tests for an improvement in the diagnosis of canine PF.(AU)
O pênfigo foliáceo (PF) é considerado uma das doenças tegumentares autoimunes mais frequentes em cães. Clinicamente, caracteriza-se pela presença de pústulas, erosões e crostas. O exame histopatológico é considerado o teste diagnóstico de eleição, porém pode se mostrar inconclusivo, sobretudo quando os achados característicos da doença não são observados. Novas ferramentas diagnósticas têm sido desenvolvidas e os testes de imunofluorecência são uma valiosa alternativa. Este estudo teve como objetivo avaliar a aplicabilidade das reações de imunofluorescência direta (IFD) e indireta (IFI) para o diagnóstico do PF canino. Vinte e oito cães foram divididos em dois grupos: grupo I com 14 cães com PF e grupo II (controle) com 14 cães com piodermite superficial (um dos principais diagnósticos diferenciais do PF). Todos os animais foram submetidos à biópsia cutânea, seguida de exame histopatológico e IFD. Amostras de sangue foram coletadas para realização da IFI. Comparando-se os valores de IFD com o histopatológico, obtiveram-se valores de concordância de 75% (9/12), com índice Kappa de 0,77 (P<0,001). Já na IFI, a concordância foi de 100% (14/14), com índice Kappa de 1,0 (P<0,001). Concluiu-se, então, que a IFD e a IFI apresentaram excelentes resultados e podem ser consideradas novas alternativas diagnósticas do PF canino.(AU)
Subject(s)
Animals , Dogs , Dogs/abnormalities , Fluorescent Antibody Technique/statistics & numerical data , Fluorescent Antibody Technique/veterinary , Pemphigoid, Bullous/diagnosisABSTRACT
Abstract: Dyshidrosiform pemphigoid is an acquired autoimmune variant of bullous pemphigoid with persistent vesicobullous eruptions localized on the palms or soles, or both. It generally occurs in the elderly and is rarely reported in childhood. Hereby, we describe the first case of dyshidrosiform pemphigoid in a 12-year-old child, which was limited to the dorsal hands and treated successfully with dapsone (diaminodiphenyl sulfone). Along with this report, we also review the clinical features of various types of dyshidrosiform pemphigoid.
Subject(s)
Humans , Male , Child , Pemphigoid, Bullous/diagnosis , Hand , Hand Dermatoses/diagnosis , Pemphigoid, Bullous/pathology , Pemphigoid, Bullous/drug therapy , Dapsone/therapeutic use , Hand Dermatoses/pathology , Hand Dermatoses/drug therapy , Anti-Infective Agents/therapeutic useABSTRACT
Fundamento: La eritrodermia ictiosiforme congénita no ampollosa es una genodermatosis que está presente desde el nacimiento y el pénfigo vegetante es una enfermedad ampollar que aparece en edades tempranas de la vida, las manifestaciones clínicas de estas entidades son diferentes, ambas son infrecuentes cuando se presentan aisladamente, mucho más cuando aparecen en un mismo paciente. Objetivo: Describir el caso de un paciente con eritrodermia ictiosiforme congénita no ampollosa y pénfigo vegetante por ser una situación clínica rara por su incidencia. Presentación de caso: Paciente de 28 años, con antecedentes de padecer de eritrodermia ictiosiforme congénita no ampollosa, que comenzó a desarrollar ampollas y al romperse dejaban erosiones que más tarde dieron lugar a vegetaciones hipertróficas y papilomatosas. Para arribar al diagnóstico se tuvo en cuenta el criterio clínico y la histopatología. Conclusiones: La eritrodermia ictiosiforme congénita no ampollosa y el pénfigo vegetante son entidades infrecuentes en la práctica médica, mucho más cuando se presenten ambas en un mismo paciente.
Background: The non-bullous congenital ichthyosiform erythroderma is a genodermatosis that is present from the birth and the vegetating pemphigus is a bullous disease that occurs at early ages of life, the clinical manifestations of these entities are different, both are uncommon when they are presented in isolation, much more when they appear in a patient Objective: To describe the case of a patient with a non-bullous congenital ichthyosiform erythroderma and vegetating pemphigus a strange clinical situation for their incidence. Case presentation: Patient of 28 years, with antecedents of suffering of non-bullous congenital ichthyosiform erythroderma that began to develop blisters and when breaking they left erosions that later gave place to hypertrophic and papillomatous vegetation. To arrive to the diagnosis was kept in mind the clinical approach and the histopathology. Conclusions: The non-bullous congenital ichthyosiform erythroderma and the vegetating pemphigus are uncommon entities in the medical practice, much more when both are presented in a patient.
Subject(s)
Ichthyosis, Lamellar/diagnosis , Pemphigoid, Bullous/diagnosisABSTRACT
El penfigoide ampolloso (PA) es una enfermedad cutánea ampollar subepidérmica autoinmunitaria que habitualmente se presenta en ancianos. Clásicamente el PA se caracteriza por presentar ampollas tensas, grandes en piel normal o eritematosa, con o sin prurito. Es difícil identificar al penfigoide ampollar prodrómico (PAP) o en estadio temprano. El PAP no se presenta con lesiones vesiculares o ampollar es como el penfigoide clásico. Se han publicado reportes en los que la enfermedad se manifiesta como prurito generalizado sin lesiones visibles, placas urticarianas, placas eritematosas, parches de eccema y dishidrosis, lesiones tipo prúrigo nodular, eritrodermia y lesiones vegetantes. Se reporta el caso de un paciente en la octava década de su vida que acude a nuestro servicio por presentar cuadro clínico e histopatológico de PA; mediante la anamnesis encontramos que debutó con la forma no ampollar del penfigoide, siendo tratado por diferentes diagnósticos sin mejoría hasta que presentó la fase ampollar clásica siendo el diagnóstico evidente. Presentamos este caso para tener en cuenta dentro del diagnóstico diferencialde aquellas lesiones inespecífica de curso tórpido, el estadio temprano del PA, pues un reconocimiento y tratamiento y temprano puede prevenir la progresión a PA clásico cuyo curso es crónico, con fases de exacerbaciones y remisiones espontáneas, que puede resultar en morbilidad significativa.
Bullous pemphigoid (BP) is an autoimmune subepidermal blistering skin disease that typically occurs in the elderly. Classically (BP) is characterized by tense blisters, large normal or erythematous skin, with or without pruritus. Itis difficult to identify the prodromal bullous pemphigoid(PBP) or early stage. The PBP is not presented with vesicular lesions or bullous pemphigoid such as classic. Reports have been published in which the disease manifests as generalized pruritus without visible lesions, urticarial plaques, erythematous plaques, patches of eczema and dyshidrosis, nodular prurigo-like lesions, erythroderma and vegetative lesions. We report the case of a patient inthe eighth decade of his life referred to our department to present clinical and histopathological picture bullouspemphigoid; through history we find that debuted with the bullous pemphigoid is not being treated for different diagnoses did not improve until presented by the classic blister stage diagnosis being evident. We present this case to account for the differential diagnosis of those injuries nonspecific chronic course, the early stage of the BP, asan early recognition and treatment can prevent progression to classical PA whose course is chronic, with periods of exacerbations and spontaneous remissions, which can result in significant morbidity.
Subject(s)
Humans , Female , Aged, 80 and over , Medical Illustration , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/therapy , Blister/therapy , Case ReportsABSTRACT
Introducción. El penfigoide ampollar (PA) es una enfermedad autoinmune que afecta con mayor frecuencia a personas de edad avanzada.Objetivos. Identificar la edad de presentación, sexo y variantes clínicas del PA. Establecer la asociación con otras enfermedades, la respuesta terapéutica y comparar los resultados con las estadísticas publicadas.Materiales y métodos. Se realizó un análisis retrospectivo y descriptivo de los pacientes que consultaron en el Servicio de Dermatología del Policlínico Bancario en el período comprendido entre agosto de 1995 y el mismo mes de 2010. Se incluyeron aquellos pacientes con diagnóstico de PA. La recolección de datos se obtuvo de los registros histológicos y de las correspondientes historias clínicas.Resultados. Se registraron 45 pacientes; 25 (55,5%) femeninos y 20 (44,5%) masculinos. La edad media fue de 75,9 años. Variantes clínicas: 35 pacientes (77,7%) clásica; 5 (11,1%) localizada; 1(2,2%) vesicular; 2 (4,4%) nodular. Asociación con DBT: 15 pacientes (33,3%); HTA: 29 (64,4%); otras enfermedades sistémicas: 19 (42,2%); enfermedades dermatológicas: 7 (15,5%). La respuesta terapéutica fue buena.Conclusiones. La edad promedio al momento del diagnóstico fue avanzada. No hallamos marcada diferencia en cuanto a la distribución por sexo. La forma clásica predominó sobre las otras variantes clínicas. La asociación con enfermedades sistémicas podría deberse a que estas patologías se dan con mayor frecuencia en el mismo grupo etario. La asociación con enfermedades dermatológicas fue conpsoriasis (la de mayor representación). La respuesta terapéutica fue altamente satisfactoria. En general, nuestros datos son similares a las estadísticas publicadas.
Subject(s)
Humans , Male , Female , Aged , Middle Aged , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/epidemiology , Pemphigoid, Bullous/drug therapy , Skin/pathology , Autoimmune Diseases , Comorbidity , Diabetes Mellitus , Psoriasis , Retrospective StudiesABSTRACT
El penfigoide ampollar es una enfermedad ampollar autoinmune adquirida, que afecta predominantemente a los ancianos y es extremadamente rara en niños. Presentamos el caso de un lactante de 5 meses que ingresa a urgencias por una dermatosis generalizada, caracterizada por ampollas de contenido claro en piel y sin involucrar mucosas. Se corroboró el diagnóstico de penfigoide ampollar por histopatología e inmunofluorescencia directa. El tratamiento consistió en esteroides orales y cuidados de la piel. La paciente ha tenido seguimiento por casi un año y no ha habido recidiva de la enfermedad. El penfigoide ampollar es una enfermedad rara en la infancia, cuya incidencia exacta se desconoce, pero generalmente es de buen pronóstico y su resolución espontánea.
Bullous pemphigoid is an acquired autoimmune disease that mostly affects the elderly and is extremely rare in children. We present the case of a 5 months old infant who was admitted in the emergency department with a generalized dermatosis characterized by skin tense blisters with clear content that spared the mucous membranes. The histopathological and direct immunofluorescence studies confirmed the diagnosis of bullous pemphigoid. The treatment consisted in oral steroids and skin care. The patient has been followed up for almost a year without recurrences. Bullous pemphigoid is a rare disease in childhood with an unknown exact incidence, but with good prognosis and usually self-limited.
Subject(s)
Humans , Female , Infant , Pemphigoid, Bullous , Pemphigoid, Bullous/diagnosis , Skin Diseases, VesiculobullousABSTRACT
Presentamos un niño de 4 meses de edad con un cuadro clínico típico de PA, con las ampollas características y en localizaciones habituales en menores de un año, (predominio en palmas y plantas y respeto de la zona del pañal), con leucocitosis y eosinofilia en los exámenes de laboratorio y con histopatología correspondiente a las lesiones urticarianas del PA e IFD que confirmó el diagnóstico presuntivo clínico. El mismo tuvo muy buena respuesta al tratamiento con corticoides sistémicos, con remisión completa rápida y sin secuelas cicatrizales ni recaídas hasta el momento
We present a 4-month-old with a typical clinical picture of PA, with the characteristic blisters and usually located in less than one year (predominantly on palms and soles and respect for the diaper area) with leukocytosis and eosinophilia laboratory tests and histopathology for the PA urticarial lesions and IFD confirmed clinical presumptive diagnosis. He himself had very good response to treatment with systemic corticosteroids, with complete remission quickly and without sequelae or relapse scarring so far