RESUMO

BACKGROUND: Pemphigus vulgaris (PV) is a potentially life-threatening mucocutaneous autoimmune disease that affects desmoglein-1 and desmoglein-3, leading to intraepithelial vesiculobullous lesions. In the oral mucosa, PV lesions can mimic other diseases such as mucous membrane pemphigoid, other forms of pemphigus, recurrent aphthous stomatitis, erythema multiforme, Stevens-Johnson syndrome, and virus-induced ulcers like herpes simplex virus (HSV), making diagnosis challenging. The co-occurrence of PV with Crohn's disease is rare and predominantly seen in younger patients. The therapeutic mainstay for both PV and Crohn's disease usually involves systemic corticosteroids combined with immunosuppressants and immunobiological drugs. Literature indicates that the use of these drugs, particularly TNF-alpha inhibitors, for managing autoimmune diseases like Crohn's can potentially induce other autoimmune diseases known as autoimmune-like syndromes, which include episodes of lupus-like syndrome and inflammatory neuropathies. There are few cases in the literature reporting the development of PV in individuals with CD undergoing infliximab therapy. CASE REPORT: A young female with severe Crohn's disease, treated with the TNF-alpha inhibitor infliximab, developed friable pseudomembranous oral ulcerations. Histopathological and immunofluorescence analyses confirmed these as PV. The treatment included clobetasol propionate and low-level photobiomodulation, which resulted in partial improvement. The patient later experienced severe intestinal bleeding, requiring intravenous hydrocortisone therapy, which improved both her systemic condition and oral lesions. Weeks later, new ulcerations caused by herpes virus and candidiasis were identified, leading to treatment with oral acyclovir, a 21-day regimen of oral nystatin rinse, and photodynamic therapy, ultimately healing the oral infections. To manage her condition, the gastroenterologists included methotrexate (25 mg) in her regimen to reduce the immunogenicity of infliximab and minimize corticosteroid use, as the patient was in remission for Crohn's disease, and the oral PV lesions were under control. CONCLUSION: Young patients with Crohn's disease should be referred to an oral medicine specialist for comorbidity investigation, as oral PV and opportunistic infections can arise during immunosuppressive therapy. The use of TNF-alpha inhibitors in patients treated for inflammatory bowel disease, such as Crohn's, should be carefully evaluated for potential side effects, including oral PV.

Assuntos

RESUMO

Pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP) are bullous autoimmune diseases that reach the oral mucosa and have common clinical features. The objective of the study was to present and compare the clinical manifestations of PV and MMP and the results of applied treatments. A case series of a stomatology service from 1985 to 2018. Data collection included epidemiological data, comorbidities, medications in use, duration of symptoms before the first visit, previous treatment, symptomatology, clinical description of lesions, presumptive diagnosis, histopathological description, extraoral manifestations, final diagnosis, treatment and follow-up. The medical records of 25 patients were analysed, 19 of whom were diagnosed with MMP and 6 with PV. The female gender was prevalent in MMP (84 %) and the male gender in PV (67 %). More than 60 % of patients complained of pain at their first visit. Patients with MMP took on average 6 months to seek professional help and patients with PV, about 2 months. Desquamative gingivitis was the most common lesion (63 %) in MMP and non-gingival ulcers (67 %) in PV. Minimal therapy was effective in all cases of MMP, and in PV one individual required minimal adjuvant therapy due to worsening of the case. Patients with PV have more intense signs and oral symptoms and may need more intensive treatment than patients with MMP. The use of topical and/or systemic corticosteroids was sufficient for most cases in both diseases.

Pénfigo vulgar (PV) y Penfigoide de la Membrana Mucosa (PMM) son enfermadades autoinmunes ampollosas que llegan a la mucosa oral y tienen características clínicas comunes. El objetivo de este estudio fue presentar y comparar las manifestaciones clínicas de PV y PMM y los resultados de los tratamientos aplicados. En el análisis se incluyó una serie de casos de un servicio de estomatología de 1985 a 2018. La recolección de información incluyó datos epidemiológicos, comorbilidades, medicamentos en uso, duración de los síntomas antes de la primera visita, tratamientos previos, sintomatología, descripción clínica de las lesiones, diagnóstico presuntivo, descripción histopatológica, manifestaciones extraorales, diagnóstico final, tratamiento y seguimiento. Se analizaron las historias clínicas de 25 pacientes, 19 de los cuales fueron diagnosticados de PMM y 6 de PV. El sexo feminino fue prevalente en PMM (84 %) y el sexo masculino en PV (67 %). Más del 60 % de los pacientes se quejaron de dolor durante la primera consulta. Los pacientes con PMM tardaron en promedio 6 meses en buscar ayuda profesional y los pacientes con PV, alrededor de 2 meses. La gingivitis descamativa fue la lesion más común (63 %) en PMM y las úlceras non gengivales (67 %) en PV. La terapia mínima fue efectiva en todos los casos de PMM, y en PV un individuo requirió terapia adyuvante mínima debido al empeoramiento del caso. Los pacientes con PV tienen signos y síntomas orales más intensos y pueden necesitar un tratamiento más intensivo que los pacientes con PMM. El uso de corticosteroides tópicos y/o sistémicos fue suficiente para la mayoría de los casos en ambas enfermedades.

Assuntos

RESUMO

Pemphigus, an autoimmune intraepidermal bullous disease group with roughly eight distinct forms, includes pemphigus vulgaris (PV) and pemphigus foliaceus (PF) as its predominant global forms. Despite the increased utilization of global health records and reporting systems, epidemiological data remain limited and poorly categorized. Therefore, this study aimed to conduct a review to track, identify, and characterize cases of PV and PF published and categorized worldwide. A research question was formulated; studies were selected based on the inclusion criteria; and data from these publications were systematically collected, summarized, and presented using narrative descriptions. The search strategy yielded 3,212 articles, of which 95 underwent critical analysis and data extraction. Studies from 52 countries contributed to the dataset, covering various pemphigus variants. Notably, only two countries, Iran (18.87%) and South Korea (11.43%), accounted for approximately a third of the reported PV cases, while Brazil contributed 40.25% of the foliaceus variants cases documented in the literature. These findings offer valuable insights into the global distribution of pemphigus and inform future research and healthcare efforts.

Assuntos

RESUMO

BACKGROUND: Infections are a major cause of morbidity and mortality in patients with pemphigus vulgaris (PV). One of the most common infections in these patients is candidiasis. This is probably due to the use of systemic immunosuppressants, including oral and intravenous corticosteroids, mainly in megadoses (pulse therapy), although it is unknown if there are other associated factors, in addition to immunosuppressive treatment. We determine the factors associated with candidiasis in PV patients in two second-care level hospitals in Mexico. METHODS: We reviewed 100 cases with PV. Cases were randomly selected from the databases of two second-care level hospitals between January 2010 and December 2019 (10 years). The primary endpoint was the incidence of candidiasis in patients with PV. RESULTS: One hundred patients with PV were enrolled in this retrospective study. Candidiasis was observed in 79 patients (79%). A maximum corticosteroid dose of 55 mg/day during the last year (p = 0.001) and a higher neutrophil/lymphocyte ratio were associated with candidiasis in patients with PV (p = 0.001). CONCLUSION: Risk factors favoring candidiasis in patients with PV are not only related to the use of corticosteroids, but also to demographic factors, the activity of the disease, and the systemic inflammation associated with autoimmunity.

RESUMO

O pênfigo vulgar (PV) é uma doença autoimune que acomete o tecido epitelial dos indivíduos afetados. As manifestações orais são frequentemente os primeiros sinais observados da doença. Objetivou-se apresentar um caso clínico de pênfigo vulgar em uma paciente de 39 anos de idade, atendida em um serviço hospitalar de referência do estado de Pernambuco, Brasil. A paciente queixava-se de lesões dolorosas na cavidade bucal há aproximadamente 1 mês, de progressão rápida. Ao exame clínico intrabucal, realizou-se o teste de Nikolsky, com resultado positivo. Em seguida, realizou-se biópsia incisional, confirmando a hipótese diagnóstica clínica de PV. A paciente recebeu o tratamento multiprofissional e foi medicada para o controle da doença, com melhora significativa do quadro clínico. O diagnóstico precoce da doença pelo profissional é importante, seguida de uma abordagem multiprofissional para um correto diagnóstico da patologia, possibilitando remissão da sintomatologia e melhora da qualidade de vida dos pacientes... (AU)

Pemphigus vulgaris (PV) is an autoimmune disease that affects the epithelial tissue of affected individuals. Oral manifestations are often the first signs observed of the disease. We aimed to present a clinical case of pemphigus vulgaris in a 39-year-old patient seen at a reference hospital service in the state of Pernambuco, Brazil. The patient complained of painful lesions in the oral cavity for about 1 month, with rapid progression. Upon intraoral clinical examination, the Nikolsky test was performed, with a positive result. An incisional biopsy was then performed, confirming the clinical diagnosis of PV. The patient received multiprofessional treatment and was medicated to control the disease, with significant improvement in the clinical picture. The early diagnosis of the disease by the professional is important, followed by a multiprofessional approach for a correct diagnosis of the pathology, enabling remission of the symptoms and improving the quality of life of patients... (AU)

El pénfigo vulgar (PV) es una enfermedad autoinmune que afecta al tejido epitelial de los individuos afectados. Las manifestaciones orales suelen ser los primeros síntomas observados de la enfermedad. El objetivo es presentar un caso clínico de pénfigo vulgar en una paciente de 39 años de edad, atendida en un servicio hospitalario de referencia del estado de Pernambuco, Brasil. El paciente se quejaba de lesiones dolorosas en la cavidad oral desde hacía aproximadamente un mes, con una rápida progresión. Tras el examen clínico intraoral, se realizó la prueba de Nikolsky, con resultados positivos. Posteriormente, se realizó una biopsia incisional que confirmó el diagnóstico clínico de PV. El paciente recibió tratamiento multiprofesional y fue medicado para controlar la enfermedad, con una mejora significativa del cuadro clínico. Es importante el diagnóstico precoz de la enfermedad por parte del profesional, seguido de un abordaje multiprofesional para un correcto diagnóstico de la patología, que permita la remisión de los síntomas y la mejora de la calidad de vida de los pacientes... (AU)

Assuntos

RESUMO

Autoimmune blistering diseases such as bullous pemphigoid (BP) and pemphigus vulgaris (PV) are complex, multifactorial, and polygenic diseases, whose exact pathogenesis is difficult to pinpoint. Research aimed at elucidating the associated epidemiologic risk factors of these two diseases has been hampered by their rare disease status. Further, a lack of centralization and standardization of available data makes the practical application of this information challenging. In order to collate and clarify the available literature we comprehensively reviewed 61 PV articles from 37 different countries and 35 BP articles from 16 different countries addressing a range of disease relevant clinical parameters including age of onset, sex, incidence, prevalence, and HLA allele association. The reported incidence of PV ranged from 0.098 to 5 patients per 100,000 people, while BP ranged from 0.21 to 7.63 patients per 100,000. Prevalence of PV ranged from 0.38 to 30 per 100,000 people and BP ranged from 1.46 to 47.99 per 100,000. The mean age of onset in patients ranged from 36.5 to 71 years for PV and 64 to 82.6 years for BP. Female-to-male ratios ranged from 0.46 to 4.4 in PV and 1.01 to 5.1 in BP. Our analysis provides support for the reported linkage disequilibrium of HLA DRB1*0402 (an allele previously shown to be associated with PV) and DQB1*0302 alleles in Europe, North America, and South America. Our data also highlight that HLA DQB1*0503 (also known to be associated with PV) appears in linkage disequilibrium with DRB1*1404 and DRB1*1401, mainly in Europe, the Middle East, and Asian countries. The HLA DRB1*0804 allele was only associated with PV in patients of Brazilian and Egyptian descent. Only two HLA alleles were reported as associated with BP more than twice in our review, DQB1*0301 and DQA1*0505. Collectively, our findings provide detailed insights into the variation of disease parameters relevant to PV and BP that can be expected to inform future work aimed at unraveling the complex pathogenesis of these conditions across the globe.

Assuntos

RESUMO

OBJECTIVES: To evaluate the effects of photobiomodulation (PBM) in gingival lesions resulting from autoimmune diseases; to compare PBM and topical corticosteroid (CS) treatment; and to assess PBM outcome over time of follow-up. MATERIALS AND METHODS: A comprehensive electronic search was performed in four electronic databases. Treatment effects were measured through visual analog scale of pain (VAS) and clinical evolution of lesion (Thongprasom scale for oral lichen planus (OLP)). Meta-analysis was performed to compare PBM with topical corticosteroid treatment and to evaluate PBM effect over time of follow-up. RESULTS: Seventeen studies were included in this review, of which six were used for the meta-analysis. Meta-analysis results showed no significant differences between PBM and topical CS in pain reduction at baseline (MD = 0.20, 95% CI = - 0.92, 1.32, p = 0.72) and 60-day follow-up (MD = 0.63, 95% CI = - 3.93, 5.19, p = 0.79); however, VAS showed significant pain reduction when compared before and after PBM at 30-day (MD = - 3.52, 95% CI = - 5.40, - 1.64, p = 0.0002) and 60-day (MD = - 5.04, 95% CI = - 5.86, - 4.22, p < 0.00001) follow-up. Thongprasom clinical scale for OLP also showed significant improvement at 30-day follow-up (MD = - 2.50, 95% CI = - 2.92, - 2.08, p < 0.00001) after PBM. CONCLUSION: PBM led to significant reduction of pain and clinical scores of the lesions, not having shown significant differences when compared to topical CS. CLINICAL RELEVANCE: PBM has been used in the treatment of autoimmune gingival lesions, but so far there is little strong evidence to support its use.

Assuntos

RESUMO

Fundamentos: o pênfigo é um grupo de doenças bolhosas autoimunes, sendo as mais comuns o pênfigo vulgar e o pênfigo foliáceo, esse último predominando no Brasil na forma endêmica e conhecido como fogo selvagem. São caracterizados pela produção de anticorpos IgG contra desmogleínas. É descrita redução na incidência do pênfigo foliáceo endêmico e aumento do pênfigo vulgar no país, mas não há estudos em Minas Gerais que abordem este último. Objetivo: descrever o perfil epidemiológico e clínico dos pacientes com pênfigo foliáceo e vulgar atendidos no serviço de dermatologia de um hospital universitário público de Minas Gerais, Brasil. Métodos: realizou-se estudo observacional, descritivo e transversal dos casos com diagnóstico de pênfigo foliáceo endêmico ou vulgar, pelo período de seis meses. Foi preenchido um questionário com dados epidemiológicos e clínicos da doença. Resultados: foram incluídos 122 pacientes no estudo, 64 com pênfigo foliáceo e 58 com pênfigo vulgar. Ao se comparar os pacientes com pênfigo foliáceo endêmico e vulgar, a mediana da idade de manifestação inicial da doença foi menor no foliáceo (p=0,001); as profissões dos pacientes com pênfigo foliáceo predominam no setor primário e, com pênfigo vulgar, no setor terciário (p=0,010); a maioria do grupo com pênfigo foliáceo residia na zona rural e periurbana, enquanto com pênfigo vulgar, na zona urbana (p=0,000); vegetação (p=0,000) e rios/córregos próximos à moradia (p=0,001) são relatados com maior frequência pelos pacientes com pênfigo foliáceo; e a mediana da quantidade de medicações sistêmicas necessárias para controle da doença é maior no pênfigo vulgar (p=0,002). Ao se comparar os pacientes com pênfigo foliáceo endêmico àqueles avaliados em estudo realizado no serviço em 2008, no estudo atual a maioria é de zona periurbana e rural, enquanto no anterior, de área urbana (p=0,030). Limitações do estudo: a população avaliada advém de um serviço de atenção terciária que não é referência para todo estado. Conclusões: os pacientes com pênfigo foliáceo endêmico e vulgar mantêm diferenças estatisticamente significativas nas principais variáveis já descritas na literatura, como idade e zona de moradia. Historicamente, é percebida uma redução de casos de pênfigo foliáceo endêmico e aumento de casos de pênfigo vulgar nos pacientes dessa instituição.

Background: pemphigus is a group of bullous autoimmune diseases. The most common of which are pemphigus vulgaris and pemphigus foliaceus, the latter predominating in Brazil in the endemic form, also known as "fogo selvagem". They are characterized by the production of IgG antibodies against desmogleins. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris have been described in the country, but there are no studies in Minas Gerais that address the latter. Objective: to describe the epidemiological and clinical profile of patients with pemphigus foliaceus and vulgaris treated at the dermatology service of a public university hospital in Minas Gerais, Brazil. Methods: an observational, descriptive and cross-sectional study of cases diagnosed with vulgar or endemic pemphigus foliaceus was carried out for a period of six months. A questionnaire with epidemiological and clinical data on the disease was filled out. Results: a total of 122 patients were included in this study, 64 with pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and pemphigus vulgaris, the median age at onset of the disease was lower in the foliaceus (p=0.001); the professions of patients with pemphigus foliaceus predominate in the primary sector and, with pemphigus vulgaris, in the tertiary sector (p=0.010); most of the pemphigus foliaceus group lived in rural and periurban areas, while those with pemphigus vulgaris lived in urban areas (p=0.000); vegetation (p=0.000) and rivers/streams close to the home (p=0.001) are more frequently reported by patients with pemphigus foliaceus; and the median amount of systemic medications needed to control the disease is higher in pemphigus vulgaris (p=0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the service in 2008, in the current study most are from periurban and rural areas, while in the previous one, from urban areas (p=0.030). Study limitations: the evaluated population comes from a tertiary care service that is not a reference for the entire state. Conclusions: patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences in the main variables described in the literature, such as age and housing area. Historically, a reduction in endemic pemphigus foliaceus cases and an increase in cases of pemphigus vulgaris among the patients of this institution have been observed.

Assuntos

RESUMO

ABSTRACT Visceral leishmaniasis (VL) is an opportunistic disease in immunosuppressed individuals, who may present severe clinical conditions, such as the ones described in this patient. She lived in an endemic region for VL, and was possibly infected with L. (L.) infantum chagasi through the bite of a contaminated sand fly. This initial infection has triggered a pemphigus vulgaris condition by immunogenic proteins present in the mosquito's saliva. The immunosuppression caused by the use of high doses of corticosteroids to control the disease promoted a severe VL condition, with hepatosplenomegaly, thrombocytopenia and hemorrhages, requiring hospitalization and the onset of a subsequent SARS-CoV-2 infection. Due to the intensity of clinical manifestations related to VL, aggravated by COVID-19, she died two days after admission to the Clinical Hospital of Marilia Medical School (HC-Famema).

RESUMO

OBJECTIVE: To assess the prevalence of oral mucosal lesions in patients with Pemphigus Vulgaris. METHODS: Observational studies reporting the prevalence of oral lesions in pemphigus vulgaris patients, without restriction to language and year of publication, were selected in a two-phase process. Search strategies were applied to PubMed, Scopus, Livivo, Web of Science, LILACS, Google Scholar, and OpenGrey databases. Articles assessing the prevalence of oral lesions in patients with conditions other than pemphigus vulgaris were excluded. Risk of bias analysis was performed using the Joanna Briggs Institute's Critical Appraisal Checklist for Studies Reporting Prevalence Data. Synthesis of results was calculated by the software R Statistics version 4.0.2 (The R Foundation). Confidence in cumulative evidence was assessed with the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) criteria. RESULTS: From 1957 studies identified, 40 were included in qualitative synthesis and 38 in meta-analyses. The pooled prevalence of patients with oral lesions solely or concurrent with other mucocutaneous lesions was 90.3%. The prevalence of patients with exclusive oral mucosal lesions was 50.8%. Risk of bias was considered low, and the certainty of evidence was very low. CONCLUSION: Oral lesions were present in approximately nine out of 10 patients with pemphigus vulgaris. The oral mucosa was the most common site of disease onset. Further longitudinal studies are urged to assess the prevalence of oral lesions at different disease stages.

Assuntos

RESUMO

BACKGROUND: We aimed to present a minimally invasive clinical approach to collect a suitable sample for the laboratory diagnosis of desquamative gingivitis. MATERIALS AND METHODS: The proposed technique involves Nikolsky's test for the collection of tissue samples. It consists of the histopathological analysis of the bullous membrane formed in cases with positive Nikolsky's sign (NS). Three patients without a previous diagnosis of the disease agreed to undergo this protocol before a biopsy. RESULTS: The diagnoses of the three cases reported here were mucous pemphigoid (MP), pemphigus vulgaris (PV), and lichen planus (LP). The tissue samples collected using this technique were sufficient to microscopically observe partial-thickness epithelium (diagnosing PV) or full-thickness epithelium (diagnosing MP). The diagnosis of LP was only possible by conventional biopsy. CONCLUSION: We observed, for different cases, some advantages of the proposed technique: minimally invasive, potentially superior to exfoliative cytology, easily performed, and with low costs.

RESUMO

Pemphigus vulgaris (PV) is a rare and potentially lethal autoimmune disease that affects the skin and mucous membranes. Injuries caused by the disease cause pain, risk of infection, and other complications that result in a high mortality rate. Frequently, management of the PV requires intensive care and a multidisciplinary approach. Oral lesions of PV are usually the first clinical signs of the disease and the last lesions to heal, requiring treatment by a specialized dental team. The aim of this study was to report two clinical cases of PV with involvement of the oral mucosa. The patients were admitted to an intensive care unit, and underwent multidisciplinary management of their condition along with low-level laser therapy. Both cases demonstrated the importance of specialized dental care in improving the quality of life of patients with PV.

Assuntos

RESUMO

Resumen El pénfigo vulgar es una enfermedad ampollar crónica de etiología autoinmune, en la cual el diagnóstico y tratamiento precoz disminuye la morbimortalidad de los pacientes. Actualmente se utilizan corticosteroides de primera línea asociados a inmunosupresores. Sin embargo,se ha visto que el uso de Rituximab como terapéutica de primera elección en los países desarrollados ha disminuido las reacciones adversas, con lo que mejora la calidad de vida de los pacientes. Se presentan cinco pacientes con diagnóstico de pénfigo vulgar refractario a tratamientos convencionalesque requirieron tratamiento con Rituximab.

Abstract Pemphigus vulgaris is an autoimmune chronic blistering disease. Its early diagnosis and treatment help reduce patient mortality and morbidity. Corticosteroids associated with immunosuppressants remain the standard treatment for pemphigus vulgaris. However, the use of Rituximab as first-line therapy in developed countries has been effective in reducing adverse effects improving the quality of life of patients. We report five patients with a diagnosis of pemphigus vulgaris refractory to conventional treatments requiring treatment with Rituximab.

RESUMO

Resumen: El pénfigo es una enfermedad autoinmunitaria y crónica de incidencia y prevalencia bajas; sin embargo, puede alcanzar mortalidad de incluso 75% sin tratamiento. Existen dos variedades principales: el pénfigo vulgar y el foliáceo, que en términos clínicos se distinguen por la aparición de ampollas en la piel, con afección de las mucosas en los pacientes con pénfigo vulgar. En esta revisión se detalla la epidemiología, causas, fisiopatología y tratamiento de esta enfermedad, con insistencia en la importancia de los esteroides como piedra angular del tratamiento de estos pacientes.

Abstract: Pemphigus is an autoimmune and chronic disease with low incidence and preva- lence. Nevertheless it could reach a 75% mortality rate without treatment. There are two principal types: vulgar pemphigus and foliaceus pemphigus, which are clinically characterized by skin blister appearance, with mucosal affection in patients with pemphigus vulgaris. This review details the epidemiology, etiology, physiopathology and treatment of this disease, remarking the steroid importance as the cornerstone in the management of these patients.

RESUMO

Se describe el caso clínico de un paciente de 72 años de edad, con diagnóstico de pénfigo vulgar, quien fue asistido en la consulta de Oftalmología del Hospital General Docente Dr. Juan Bruno Zayas Alfonso de Santiago de Cuba porque padecía enrojecimiento ocular, unido a disminución de la visión y secreciones abundantes de aproximadamente 5 días de evolución. En el examen oftalmológico se confirmó la presencia de úlcera corneal bilateral por perforación infecciosa. Se indicó tratamiento con colirios de antibióticos (ceftazidima y amikacina), antiinflamatorio no esteroideo, agentes antihipertensivos y lente de contacto, con lo cual mejoraron las lesiones de ambos ojos. El proceso infeccioso pudo deberse a cierta susceptibilidad a los esteroides e inmunosupresores sistémicos, como terapia asociada al pénfigo vulgar, o al mecanismo autoinmune característico de esta dermatopatía.

The case report of a 72 years patient is described, with diagnosis of pemphigus vulgaris who went to the Ophthalmology Service of Dr. Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba because he suffered from ocular redness, together with a decrease vision and abundant secretions of approximately 5 days of history. In the ophthalmological examination the presence of bilateral corneal ulcer was confirmed due to infectious perforation. Treatment was indicated with antibiotic eyewashes (ceftazidime and amikacine), non esteroidal anti-inflammatory drugs, antihypertensive agents and contact lens, with which the injuries of both eyes improved. The infectious process could be due to certain susceptibility to the steroids and systemic inmunosupressors, as therapy associated with pemphigus vulgaris, or to the autoinmune mechanism characterizing this dermatopathy.

Assuntos

RESUMO

Pemphigus is a bullous autoimmune disease that affects the skin and mucous membranes. It is very difficult to establish the etiology and the triggering factors that influence reactivations in pemphigus vulgaris (PV). The case of a 33-year-old male with chronic history of intranasal cocaine consumption is presented in this report. We present the clinical case of the patient, followed for a total of 86 weeks, with ten relapses secondary to probable cocaine use. The patient was admitted to the emergency department after presenting polymorphic dermatosis characterized by blisters, vesicles, and excoriations extending from the oral cavity to the thorax, and to the inguinal and genital regions, affecting approximately 35 % of the body surface area with a score of 56 on the Pemphigus Skin Disorder Index. Skin biopsies were compatible with PV diagnosis. The patient had clinical improvement with a combination of methylprednisolone 500 mg intravenously (IV) and cyclophosphamide 500 mg IV every 15 days, along with prednisone 50 mg orally (PO) q24 h and mycophenolic acid 500 mg PO q6 h. Persistent cocaine use is highly likely to be the factor triggering lesion reactivation and responsible for the torpid evolution. We cannot definitively conclude whether the change from azathioprine to mycophenolic acid after the tenth relapse was the adjuvant medication responsible for the end of the consolidation phase and complete remission on therapy. This case study could potentially serve as a guide for management of patients who continuously persist with cocaine use, leading to a clinical picture refractory to multiple therapeutic schemes.

RESUMO

Resumen: Introducción: El pénfigo vulgar es una enfermedad grave y poco frecuente en niños. Su diagnóstico y tratamiento oportuno permite modificar el pronóstico. El objetivo es describir las características clínicas y el abordaje diagnóstico y terapéutico de esta poco frecuente enfermedad vesículo-ampollar autoinmune en niños. Caso clínico: Niño de 2 años, previamente sano. Comenzó con dermato sis generalizada con máximo lesional en región umbilical, miembros y genitales, caracterizada por ampollas, algunas denudadas con sangrado fácil, sin compromiso mucoso ni fiebre. Se diagnosticó impétigo bulloso y se indicó antibioticoterapia tópica y sistémica sin mejoría clínica. Evolucionó con extensión lesional, con compromiso de mucosas oral y anal. El estudio histológico e inmunohistoquí- mico de las lesiones y la piel perilesional confirmó pénfigo vulgar. Se inició tratamiento corticoideo e inmunomodulador con buena respuesta. Conclusiones: Dada la similitud de las manifestaciones del pénfigo vulgar con otras enfermedades infecciosas e inflamatorias de mayor prevalencia, se requiere un alto índice de sospecha para evitar demoras en el diagnóstico y el comienzo del tratamiento. En pacientes con manifestaciones vesículo ampollares de evolución no esperada, es necesaria la intercon sulta por dermatólogo y evaluar la oportunidad de biopsia de la lesión y piel perilesional, para estudio histológico e inmunofluorescencia directa, lo que permitirá la confirmación diagnóstica.

Abstract: Introduction: pemphigus vulgaris is a serious and infrequent disease in children. Its timely diagnosis and treatment allows modifying its prognosis. The objective is to describe its clinical characteristics, and the diagnostic and therapeutic approach of this uncommon autoimmune blistering disease in children. Clinical case: 2-year-old male patient, previously healthy. He initially presented generalized dermatosis with maximum lesion areas at the umbilical region, limbs and genitals; characterized by blisters, some denuded, and of easy bleeding, without mucosal involvement nor fever. Bullous im petigo was diagnosed and topical and systemic antibiotic treatment was started, showing no clinical improvement. He developed extension of the lesions with oral and anal mucosal involvement. The histologic and direct immunofluorescent study of lesions and perilesional skin confirm the diagnosis of pemphigus vulgaris. The patient started treatment with corticosteroids and immunomodulatory agents with good clinical response. Conclusions: Due to the similarity with other more prevalent infectious and inflammatory diseases, a high index of suspicion is required in order to avoid delays in the diagnosis and the start of treatment. In patients with blisters with an unexpected clinical evolu tion, it is necessary to conduct a joint evaluation with a dermatologist and to assess the opportunity of performing a biopsy of the lesion and perilesional skin for histological study and direct immunofluo rescence, which will allow diagnostic confirmation.

Assuntos

RESUMO

Pemphigus vulgaris (PV) is a chronic autoimmune mucocutaneous disease. In most cases, the initial manifestation occurs in the mouth as multiple ulcerations preceded by blisters that rupture and later spread to other mucous membranes and the skin. Esophageal impairment is rare. We report a case of PV with esophageal involvement in a 53-year-old woman who sought medical care, complaining of diffuse painful lesions in the oral cavity for approximately 1 month, with no improvement with nystatin. Upper digestive endoscopy (UDE) was performed with findings of vesiculobullous lesions in the proximal and middle esophagus, which were biopsied and for which histopathology confirmed PV in the esophageal mucosa. We draw attention to UDE as important for the diagnosis, and it should be indicated in patients with vesiculobullous lesions of the mouth, especially if there are esophageal complaints.