Follicular dendritic cell sarcoma aggravated by hyaline-vascular Castleman's disease in association with paraneoplastic pemphigus: study of the tumor and successful treatment

Abstract The authors have successfully treated and monitored a case of paraneoplastic pemphigus in association with follicular dendritic cell sarcoma aggravated by hyaline-vascular Castleman's disease. The patient was a 56-year-old female who presented with recalcitrant erosive lichen planus of the oral cavity, tongue, and genital mucosa, along with polymorphous eruptions throughout her body. Histological examination of the cutaneous lesions, indirect immunofluorescence on rat bladder epithelium, and western blot of human keratinocyte proteins identified anti-epidermal antibodies in the patient's serum. Positron emission tomography and computed tomography scans found a mass in her retroperitoneal region. Pathology and immunohistochemistry investigation further corroborated the diagnosis of follicular dendritic cell sarcoma originated from hyaline-vascular Castleman's disease. Complete remission was achieved and the patient has been monitored for four years.

Manifestación oral del pénfigo vulgar. Reporte de un caso / Oral manifestation of vulgar pemphigus. A case report

RESUMEN El pénfigo es una enfermedad autoinmune potencialmente mortal, que causa ampollas y erosiones en la piel y en la membrana mucosa. Las lesiones epiteliales son el resultado de autoanticuerpos que reaccionan con las glicoproteínas desmosomales y están presentes en la superficie celular del queratinocito. La reacción autoinmune contra estas glicoproteínas causa una pérdida de adhesión celular, resultando en la formación de ampollas intraepiteliales. Del 80 al 90 % de los pacientes con pénfigo vulgar, desarrollan trastornos cutáneos y en el 60 % de los casos alteraciones en la mucosa que es el primer o único signo. El diagnóstico de las lesiones en cavidad bucal es fundamental, ya que pueden prevenir su afectación a la piel. Si se establece el tratamiento en su etapa inicial, la enfermedad es más fácil de controlar y aumenta la posibilidad de una remisión temprana del trastorno y mejor calidad de vida. Este reporte de caso mostró a una paciente de 35 años, la que comenzó a presentar lesiones ulceradas en toda la orofaringe, con sensación de ardor e incapacidad para la ingestión de alimentos. El diagnóstico fue pénfigo vulgar (AU).

ABSTRACT Pemphigus is a potentially deadly autoimmune disease causing blisters and erosions in the skin and the mucous membrane. The epithelial lesions are the result of antibodies reacting to desmosomal glycoproteins, and are present in the keratinocytes cellular surface. The autoimmune reaction to these glycoproteins causes a cellular adhesion loss resulting in the formation of intraepithelial blisters. From 80 to 90 % of the patients with vulgar pemphigus develop skin disorders, and 60 % of the cases show mucosa changes as the first or unique sign. The diagnosis of the lesions in oral cavity is essential because it could prevent the skin damage. If the treatment begins in an initial stage, it is easier to control the disease and the possibility of the disorder's early remission and a better life quality increases. This is the report of the case of a female patient, aged 35 years, who presented ulcerated lesions in the entire oropharyngeal region, with itching sensation and inability for food consumption. The diagnosis was vulgar pemphigus (AU).

Fogo selvagem: endemic pemphigus foliaceus

Abstract: Fogo selvagem or endemic pemphigus foliaceus is an autoimmune acantholytic anti-cadherin bullous disease that primarily affects seborrheic areas, which might disseminate. Brazil has the world's largest number of patients, mainly in the Central-West region, but the disease has also been reported in other South American countries. It affects young people and adults who have been exposed to rural areas, with occurrence of familial cases. Anti-desmoglein-1 autoantibodies are directed against desmosomal structures, with loss of adhesion of the upper layers of the epidermis, causing superficial blisters. The etiology is multifactorial and includes genetic, immune, and environmental factors, highlighting hematophagous insect bites; drug-related factors are occasionally involved. Flaccid blisters readily rupture to yield erosive-crusty lesions that sometimes resemble seborrheic dermatitis, actinic keratosis, and chronic cutaneous lupus erythematosus. The clinical presentation varies from localized to disseminated lesions. Clinical suspicion should be confirmed with histopathological and immunofluorescence tests, among others. The progression is usually chronic, and therapy varies according to clinical presentation, but generally requires systemic corticosteroid therapy associated with adjuvant immunosuppressive treatment to decrease the adverse effects of corticosteroids. Once the disease is under control, many patients remain stable on low-dose medication, and a significant proportion achieve remission.

Pemphigus foliaceus with pustular presentation in a patient with psoriasis

Abstract Pemphigus foliaceus is a chronic autoimmune disease of the skin, clinically characterized by scaly and crusty cutaneous erosions involving the seborrheic areas. The patient can eventually become erythrodermic. There are reports of atypical cases of pemphigus foliaceus with pustules and neutrophils, and clinical differentiation from generalized pustular psoriasis of von Zumbusch is difficult. We report the case of a 55-year-old man with a history of psoriasis vulgaris that has developed pemphigus foliaceus with pustules, triggered by withdrawal of systemic corticosteroids. This is the first report associating this atypical form of pemphigus with psoriasis, suggesting that an overlap with generalized pustular psoriasis can occur.

Enfermedades ampollosas autoinmunes del grupo de los pénfigos / Autoimmune blistering diseases of the pemphigus group

En muchas enfermedades dermatológicas se presentan ampollas, pero no todas son de etiología autoinmune. Para el estudio de las enfermedades ampollosas se deben tener en cuenta las manifestaciones clínicas, la historia de cómo y cuándo empezaron las ampollas, las características epidemiológicas e histológicas (por ejemplo, el nivel de la piel en el que se producen las ampollas) y la presencia o no de infiltrados inflamatorios. Para corroborar la etiología autoinmune de la enfermedad ampollosa es importante contar con los resultados de pruebas como la inmunofluorescencia directa e indirecta, el inmunoblotting, el ensayo inmunoenzimático (ELISA), la inmunoprecipitación y la microscopía electrónica. La información sobre los títulos séricos de autoanticuerpos ayuda a orientar mejor el tratamiento inmunosupresor.

Blisters may appear in many dermatological diseases, but they are not necessarily of autoimmune etiology. For the study of blistering diseases, it is necessary to take into account the clinical aspects, the history of when and how blisters appeared, the epidemiological and histological information (for instance, the skin level at which blisters are located), and whether inflammatory infiltrates are present. In order to corroborate the autoimmune etiology of blisters, it is important to have the results of confirmatory tests such as direct and indirect immunofluorescence, immune blotting, enzyme-linked immune-assay (ELISA), immune precipitation, and electronic microscopy. Information on autoantibodies serum titers may help to conduct a more precise immunosuppressive therapy.

Pênfigo e suas variações / Pemphigus and its variations

As doenças nas quais o sistema imunológico causa lesões no próprio organismo chamam-se doenças autoimunes, a exemplo do pênfigo, que é considerado um conjunto de doenças vesicobolhosas que podem acometer membranas mucosas e pele.Objetivo: apresentar uma revisão de literatura atualizada acerca do pênfigo, englobando sua conceituação e etiologia, características clínicas, formas de diagnóstico, tratamento e prognóstico.Conclusão: o pênfigo é uma enfermidade crônica autoimune que com frequência se manifesta como lesões bolhosas iniciais na mucosa bucal, afetando posteriormente outras mucosas e a pele. Além disso, apresenta uma diversidade de lesões que podem ser incluídas no seu diagnóstico diferencial, as quais devem ser detalhadamente conhecidas pelo cirurgião dentista...

Diseases in which the immune system causes lesions in the body are called autoimmune diseases, such as pemphigus, which is considered a set of vesiculobullous diseases that can affect the mucous membranes and skin.Aim: to present a review of current literature on pemphigus, including its concept and etiology, clinical features, methods of diagnosis, treatment and prognosis.Conclusion: the pemphigus is a chronic autoimmune disease that often manifests itself as early bullous lesions in the oral mucosa, subsequently affecting other mucous membranes and skin. Moreover, it presents a variety of lesions that may be included in their differential diagnosis, which must be thoroughly known by the surgeon-dentist...

Pênfigo vulgar: relato de caso / Pemphigus vulgaris: a case report

O pênfigo é uma doença vesicobolhosa caracterizada pela presença de auto-anticorpos contra moléculas de adesão intraepidérmicas. As quatro formas mais conhecidas são: pênfigo vulgar, pênfigo vegetante, pênfigo foliáceo e o eritematoso. O pênfigo vulgar é doença órgão específica, em que auto-anticorpos IgG se dirigem contra desmogleína 3, culminando no processo de acantólise. Em grande porcentagem dos casos se manifesta inicialmente na mucosa, das quais a maisafetada é a oral. O tratamento mais utilizado é a corticoterapia oral, muitas vezes em altas doses e por períodos prolongados. Como efeitos colaterais dessa terapêutica, frequentemente ocorrem: hipertensão arterial, diabete, osteoporose, infecções e distúrbios hidroeletrolíticos. O diagnóstico é feito através da clínica, visualização das lesões e biópsia.

Pénfigo vulgar post-radioterapia en paciente con carcinoma mamario / Póst-radiotherapy pemphygus vulgaris in patients with breast carcinoma

Se presenta una paciente de 38 años, con antecedentes personales de carcinoma de mama, variedad ductal, que recibió tratamiento quirúrgico con vaciamiento ganglionar, quimioterapia y radioterapia localizada en la región mamaria izquierda; tras este último tratamiento desarrolla un pénfigo vulgar localizado en la región irradiada. El diagnóstico de pénfigo vulgar fue confirmado por histopalogía y estudios inmunológicos.

Pênfigo foliáceo em eqüino: relato de caso / Equine pemphigus foliaceous: case report

Diagnosticou-se pênfigo foliáceo em um eqüino macho, castrado, quatro anos de idade da raça Árabe apresentando dermatite generalizada caracterizada por placas escamo-crostosas e presença de dermatite pustular acantolítica subcorneal

Pemphigus foliaceus was diagnosed in a four-year-old Arabian equine gelding, with generalized dermatitis characterized by scaling and crusting plaques and acantholytic subcorneal pustular dermatitis

Inflammatory Myofibroblastic Tumor on Intercostal Nerve Presenting as Paraneoplastic Pemphigus with Fatal Pulmonary Involvement

Inflammatory myofibroblastic tumors (IMTs) are benign neoplasms that can occur at different anatomic sites with nonspecific clinical symptoms. A 48-yr-old woman presented with a 2-month history of a relapsed oral ulcer, progressive dyspnea, and a thoracic pain induced by breathing. A tumorous mass was noticed in the right costodiaphragmatic recess on chest computed tomography and magnetic resonance imaging, and the patient underwent a right costotransversectomy with excision of the tumor, which originated from the 12th intercostal nerve. Histology and immunohistochemistry showed that the tumor was an IMT of the intercostal nerve. The patient's postoperative course was not favorable; dyspnea persisted after surgery, and a progressive pulmonary compromise developed. The cause of the respiratory failure was found to be bronchiolitis obliterans, which in this case proved to be a fatal complication of paraneoplastic pemphigus associated with an IMT. This case of IMT of the spinal nerve in the paravertebral region is unique in terms of its location and presentation in combination with paraneoplastic pemphigus, which is rare. A brief review of the heterogeneous theories concerning the pathogenesis, clinicopathological features, and differential diagnosis of this disease entity is presented.

Study of desmoglein 1 and 3 antibody levels in relation to disease severity in Indian patients with pemphigus.

OBJECTIVES: To conduct a cross-sectional study to compare Dsg1 and Dsg3 antibody levels independently with severity of disease activity in pemphigus vulgaris (PV) and pemphigus foliaceus (PF). METHODS: Blood samples from 44 patients with pemphigus (PV-38, PF-6) were analyzed using ELISA. The severity of skin and mucosal disease was graded using a score from 0 to 3. RESULTS: A statistically significant correlation between increase in Dsg 3 antibody titres with severity of oral involvement and Dsg 1 titres with severity of skin involvement was found in both PV and PF patients (p < 0.01). However, we were unable to demonstrate a relationship between increased titres of Dsg1 and Dsg 3 antibodies with oral and skin involvement respectively. CONCLUSION: This study suggests that the severity of skin and oral disease in pemphigus is determined by the quantities of Dsg1 and Dsg3 antibodies respectively.

Evidence for the participation of nitric oxide in pemphigus

Pemphigus is an inflammatory autoimmune disorder of the skin. Nitric oxide (NO) is an inflammatory mediator linked to a variety of physiological and pathophysiological phenomena that include skin tumors, psoriasis, urticaria, and atopic dermatitis. Inflammatory cells present in pemphigus lesions are important sources of NO production. We investigated whether NO is involved in pemphigus. A prospective cohort study was conducted at the Dermatology Service of the Hospital Universitário Walter Cantídio of the Federal University of Ceará. All patients seen at the outpatient clinic between August 2000 and July 2001, with a clinically and histologically confirmed diagnosis of pemphigus were included. The median age was 42.5 years (range: 12-69 years) with a male to female ratio of 3:2. Total serum nitrite levels, used as a marker for NO production, were determined by the Griess reaction. Skin biopsies from pemphigus and breast surgery (control) patients were used for the detection of the inducible NO synthase (iNOS) by immunohistochemistry. Twenty-two (22) patients with pemphigus and eight (8) controls who did not differ in demographic characteristics were included. Total serum nitrite levels were significantly higher (>7 æmol/L) in pemphigus patients compared to controls (<6 æmol/L), regardless of the severity of the clinical activity of pemphigus (P < 0.0001). All pemphigus biopsies presented increased immunostaining for iNOS that was not detected in normal skin samples. These data are the first to demonstrate that pemphigus patients display increased serum NO levels that are associated with increased iNOS expression in the affected skin.

Manejo conservador de la enfermedad periodontal crónica en presencia de lesiones vesiculobulosas: reporte de dos casos de pénfigo vulgar / Conservative management of chronic periodontal disease in the presence of vesiculobullous lesions: report of two cases of pemphigus vulgaris

Se reportan dos casos clínicos de pénfigo vulgar, confirmados a través del examen histopatológico, con localización en la región mucogingival de cavidad bucal, en pacientes con presencia de enfermedad periodontal crónica moderada avanzada. El tratamiento periodontal a emplearse en pacientes con lesiones bucales vesiculobullosas como el pénfigo vulgar, puede ser considerado conservador, evitando así la consecuente descamación epitelial de la superficie mucosa y la posterior ulceración de la misma al realizarse tratamientos orientados a las periodontopatías

Estudo de lesões orais associadas a doenças dermatologicas / Study of oral lesions associated to dermatologic diseases

No presente trabalho, nos propusemos a avaliar a freqüência das lesões orais em pacientes portadores de doenças dermatológicas, visando despertar o cirurgião dentista para a necessidade de estabelecer um diagnóstico preciso. A amostra do nosso estudo constou de 101 pacientes procedentes do Serviço de Dermatologia do Hospital Universitário Onofre Lopes (HUOL) da UFRN com dermatopatologias diagnosticadas os quais foram examinados clinicamente com intuito de identificar alterações bucais. A idade dos pacientes variou entre 20 e 60 anos de idade, havendo predileção pelo sexo feminino (1.8:1). Do total de casos avaliados, 43 porcento eram de lúpus eritematoso, 35 porcento de líquen plano, 11 porcento de pênfigo vulgar, 8 porcento de eritema multiforme e 3 porcento eram do grupo penfigóide. As manifestações orais, em valores absolutos, foram mais freqüentes nos pacientes portadores de líquen plano (17) seguido pelo pênfigo (3), grupo penfigóide (2) e lúpus eritematoso (2). O líquen plano reticular foi a apresentação clínica mais comum e a mucosa jugal o sítio mais acometido. Os portadores de pênfigo e grupo penfigóide exibiram preferencialmente lesões de aspecto ulcerativo distribuídas na região de mucosa jugal e orofaringe, enquanto o lúpus eritematoso apresentava lesões circunscritas e eritematosas. Não foram observadas alterações bucais nos casos de eritema multiforme. De acordo com os resultados obtidos concluímos que o líquen plano foi a dermatopatologia mais prevalente em nosso estudo em relação à freqüência das alterações orais apresentadas, a partir da qual podemos inferir a necessidade do conhecimento do cirurgião dentista neste contexto e a importância do seu papel para que seja estabelecido um diagnóstico precoce e orientado o tratamento adequado com uma abordagem multidisciplinar

Lesiones estomatológicas en un caso de pénfigo vulgar / Stomatological lesions in a pemphigus vulgaris case

El pénfigo vulgar es una enfermedad mucucutáneo autoinmune, predominantemente ampollar, de pronóstico grave. Las ampollas pueden manifestarse en diferente localizaciones de la mucosa bucal, rápidamente se rompen dejando erosiones dolorosas, dificultando la alimentación y la fonación. Las lesiones cutáneas se ubican preferentemente en zonas de roce (pliegues submamarios, zona inguinal). Los métodos complementarios de diagnóstico utilizados para las lesiones orales son la citología exfoliativa, la biopsia y la inmunofluorescencia. El tratamiento con esteroides es de elección, tanto para las manifestaciones locales como sistémicas

Aspectos clínicos, diagnóstico e tratamento do pênfigo vulgar - revisão de literatura / Clinical features, diagnosis and treatment of pemphigus vulgaris - literature review

O pênfigo vulgar é uma doença auto-imune, vesículo-bolhosa, crônica e grave, que acomete pele e mucosas. A presença de anticorpos antidesmossômicos resulta na dissolução da substância cimentante itercelular pela destruição dos desmossomos que mantêm unidas as células epiteliais gerando uma separação intra-epitelial característica, que ocorre logo acima da camada de células basais. O exame físico mostra erosões e ulcerações superficiais distribuídas em qualquer parte da mucosa oral, podendo-se observar, também, a presença de epitélio livre sobre suas ulcerações. Há sintomatologia dolorosa dificultando a alimentação. O diagnóstico clínico deve ser confirmado pela biópsia parcial incluindo tecido perilesional, cujo espécime é submetido à técnica de rotina por H&E e à imunofluorescência. Embora não exista cura, a doença pode ser controlada pelo uso de corticosteróides sistêmicos em combinação ou não com outras drogas imunossupressoras. O pênfigo vulgar é uma condição importante, se não tratada, pode resultar no óbito do paciente. Freqüentemente, as primeiras manifestações dessa doença ocorrem na boca, devendo o cirurgião-dentista estar capacitado a diagnosticá-la precocemente

Pênfigo vulgar e penfigóide benigno de mucosa: considerações gerais e relato de casos / Pemphigus vulgaris and mucous membrane pemphigoid: general considerations and case report

O pênfigo vulgar e penfigóide benigno de mucosa são doenças mucocutâneas, vesículo-bolhosas que acometem a mucosa de revestimento da cavidade bucal. Não são condições comuns e são capazes de provocar sinais e sintomas que variam de leve a moderado, podendo muitas vezes, exigir controles terapêuticos prolongados por toda vida do paciente. Sua etiologia permanece obscura, mas sabe-se que possuem etiopatogenia de caráter imunológico. Este trabalho é um relato de dois casos clínicos, onde buscamos fazer considerações gerais de importância para o Cirurgião Dentista no que diz respeito a suas condutas frente a pacientes com suspeitas de serem portadores destas patologias