Childhood pemphigus vulgaris is a challenging diagnosis

Pemphigus Vulgaris (PV) is an uncommon autoimmune and blistering mucocutaneous disease. Childhood Pemphigus Vulgaris (CPV) is a pediatric variant of PV, which affects children below 12 years, being very rare among children under 10 years of age. CPV has similar clinical, histological, and immunological features as seen in PV in adults. The mucocutaneous clinical presentation is the most common in both age groups. Vesicles and erosions arising from the disease usually cause pain. A few CPV cases have been reported in the literature. This study reports a case of an 8-year-old male patient with oral lesions since the age of 3 years, and the diagnosis of pemphigus was achieved only 2 years after the appearance of the initial lesions. CPV remains a rare disease, making the diagnosis of this clinical case a challenge due to its age of onset and clinical features presented by the patient. Therefore, dentists and physicians should know how to differentiate CPV from other bullous autoimmune diseases more common in childhood.

Gastrointestinal cytomegalovirus disease in a patient with pemphigus vulgaris treated with corticosteroid and mycophenolate mofetil

Pemphigus vulgaris is an autoimmune disease characterized by the formation of suprabasal intra-epidermal blisters on the skin and mucosal surfaces. Infectious diseases are the main cause of death in patients with pemphigus due to the disrupture of the physiological skin barrier, immune dysregulation, and the use of immunosuppressive medications leaving the patient prone to acquire opportunistic infections. We report the case of a 67-year-old woman diagnosed with pemphigus vulgaris, who was irregularly taking prednisone and mycophenolate mofetil. She was hospitalized because of a 1-month history of watery diarrhea and oral ulcers. Unfortunately, the patient died suddenly on the ward. The autopsy revealed a bilateral saddle pulmonary embolism, Gram-positive cocci bronchopneumonia, and gastrointestinal cytomegalovirus infection, causing extensive gastrointestinal mucosal ulcers.

Use of rituximab in pemphigus patients with chronic viral hepatitis: Report of three cases.

Use of rituximab in patients with chronic viral hepatitis can worsen pre-existing hepatitis or reactivate occult infection. There are no reports of use of rituximab in pemphigus patients with co-existing viral hepatitis. Herein, we report three pemphigus patients with co-existing chronic viral hepatitis (hepatitis C (n = 2), hepatitis B (n = 1)), who were treated successfully with rituximab under close supervision and concurrent antiviral drug administration. There was no derangement of the liver function tests or increase in viral load in any of the patients. By incorporating good collaboration with a hepatologist and close follow-up, such patients can be managed successfully with biologic therapies when the conventional treatment modalities have failed.

Prevalence of Metabolic Syndrome and its components in a Brazilian sample of pemphigus patients

BACKGROUND: Pemphigus foliaceus and pemphigus vulgaris are endemic in the northeastern region of São Paulo State, Brazil. They are treated mainly with systemic corticosteroids, which may provoke osteoporosis; atherosclerosis, higher blood pressure, insulin resistance, glucose intolerance, hyperlipidemia and abdominal obesity. These side effects of corticoids also constitute criteria for the diagnosis of metabolic syndrome. OBJECTIVE: The prevalence of metabolic syndrome and each component of metabolic syndrome in Pemphigus foliaceus and pemphigus vulgaris groups was compared with Brazilian casuistic samples. METHODS: Data of 147 patients (pemphigus foliaceus 48.9% and pemphigus vulgaris 51.1%) were compiled from medical records regarding metabolic syndrome and its components, and included in the analysis. RESULTS: There was no significant difference regarding the prevalence of metabolic syndrome in pemphigus groups compared with the Brazilian casuistic samples. The analysis of each component of metabolic syndrome showed a higher prevalence of: higher blood pressure in male subjects with pemphigus vulgaris, and in pemphigus foliaceus in both genders; diabetes mellitus in both genders for pemphigus vulgaris and pemphigus foliaceus; obesity in females for pemphigus vulgaris and pemphigus foliaceus, and hypertriglyceridemia in both genders for pemphigus vulgaris and pemphigus foliaceus groups that were statistically significant compared to the Brazilian reports. Furthermore, the study noted a higher incidence of cardiovascular events in both genders in pemphigus foliaceus and pemphigus vulgaris groups than in Brazilian casuistic samples. CONCLUSION: The components of metabolic syndrome are more numerous in pemphigus when compared with Brazilian casuistic samples. Future studies are necessary to assure that metabolic syndrome may be associated with pemphigus per se, including a greater casuistic sample of patients who have not ...

A case of Paraneoplastic Pemphigus associated with Castleman's disease / Um caso de Penfigo Paraneoplastico (PNP) associado a doenca de Castleman

We present a case of PNP associated with Castleman's Disease. We have also reviewed the literature and described the characteristics of the two associated diseases. Gene clonal rearrangement was done to help diagnosis. We used, in addition, stereotactic radiosurgery which, as far as we know, has never before been employed to treat PNP associated with Castleman's Disease. This produced a good response, suggesting that it might be a good alternative treatment for PNP associated with Castleman's Disease when it is too difficult to operate.

Apresentamos um caso de PNP associada à doença de Castleman.Também revisamos a literatura, e referenciamos as características das duas doenças associadas. Um rearranjo genético clonal foi feito para ajudar o diagnóstico. Além disso, usamos a radiocirurgia que até então nunca havia sido utilizada para tratar PNP associada à doença de Castleman. Esta produziu uma boa resposta, sugerindo que pode ser uma boa alternativa para o tratamento de PNP associada com a doença de Castleman quando é muito difícil fazer uma cirugia convencional.

Anagen effluvium.

Disturbances of hair follicle cycling lie at the heart of most hair growth disorders, and have dramatic effects on visible hair growth and shedding. The two common disorders due to aberration in hair follicle cycling are telogen and anagen effluvium. Though a lot of literature addresses the problem of telogen effluvium, there are not many reviews on anagen effluvium or anagen hair loss. Anagen effluvium is considered synonymous with chemotherapy-induced alopecia and other causes are rarely considered. In this review, we try to discuss the etiopathogenesis, clinical presentation, differentials, and management issues in anagen effluvium. Anagen effluvium is the abrupt loss of hairs that are in their growing phase (anagen) due to an event that impairs the mitotic or metabolic activity of hair follicle. Chemotherapy, radiation and toxic chemicals, and sometimes inflammatory diseases like alopecia areata and pemphigus are also capable of diminishing the metabolic activity of hair follicles resulting in anagen hair loss. Although it is reversible, and hair regrowth occurs after a delay of 1-3 months; sometimes it can lead to permanent alopecia and can be psychologically devastating with negative impact on individual perceptions of appearance, body image, sexuality, and self-esteem. For some patients, the emotional trauma may be so severe that it may lead to discontinuing or refusing therapy that might otherwise be beneficial. In such cases, a psychosomatic approach as well as empathic consideration of the patients concerns and fears as well as the provision of practical medical-aesthetic and styling tips are equally important and can be integrated in management.

Endemic pemphigus in the peruvian Amazon: epidemiology and risk factors for the development of complications during treatment / Pênfigo endêmico na Amazônia peruana: epidemiologia e fatores de risco para o desenvolvimento de complicações durante o tratamento

BACKGROUND: Pemphigus is an autoimmune blistering disease. According to a report, in areas of endemic pemphigus foliaceus (EPF) in Peru there are cases of pemphigus vulgaris with epidemiologic, clinical and histopathologic characteristics similar to those of "endemic pemphigus vulgaris" (EPV) in Brazil. OBJECTIVES: To determine the clinical and epidemiologic characteristics of endemic pemphigus and the risk factors of patients for developing complications during treatment. METHODS: A study was carried out from July 2003 to March 2008. The study population was 60 patients with EPF and 7 patients with EPV evaluated in hospitals and clinics in the Peruvian Amazon and Lima. A multivariate analysis was carried out using binary logistic regression. RESULTS: The average age of EPF patients was 31.4 years; 55% were men; 60% presented the generalized clinical variant. Non-compliance with the treatment was seen in 57.1% of the patients. Thirty-five percent presented complications (e.g. pyodermitis and pyelonephritis) during treatment. The risk factors for developing complications during treatment were non-compliance with the treatment and having the generalized clinical form. In the EPV group, the average age was 21.7 years; 71.4% were men. All patients presented with the mucocutaneous clinical variant and the initial presentation consisted of oral mucosa lesions; 71.4% presented complications during treatment, pyodermitis being the most frequent. CONCLUSIONS: Non-compliance with the treatment and the generalized clinical form are risk factors for the development of complications during treatment of patients with EPF. Peru indeed has EPV cases with epidemiologic characteristics similar to EPF. Living in a rural area may represent a risk factor for the development of complications during treatment of patients with EPV.

FUNDAMENTOS: O pênfigo é uma doença auto-imune bolhosa. Segundo um relatório, em áreas de pênfigo foliáceo endêmico no Peru há casos de pênfigo vulgar com características epidemiológicas, clínicas e histopatológicas semelhantes às do "pênfigo vulgar endêmico" no Brasil. OBJETIVOS: Determinar as características clínicas e epidemiológicas do pênfigo endêmico e os fatores de risco para o desenvolvimento de complicações durante o tratamento. MÉTODOS: Um estudo foi realizado de julho de 2003 a março de 2008. 60 doentes de pênfigo foliáceo endêmico e 7 de pênfigo vulgar endêmico foram avaliados em hospitais e clínicas na Amazônia peruana e em Lima. Uma análise multivariante foi feita usando regressão logística binária. RESULTADOS: A idade média dos doentes de pênfigo foliáceo endêmico foi 31,4 anos; 55% eram homens, 60% apresentavam a forma clínica generalizada. 57,1% nao cumpriram o tratamento. 35% apresentaram complicações (por exemplo, piodermites e pielonefrite). Os fatores de risco foram não cumprir o tratamento e ter a forma clínica generalizada. No grupo pênfigo vulgar endêmico, a idade média foi 21,7 anos; 71,4% eram homens. Todos os pacientes apresentavam a variante clínica mucocutânea e a apresentação inicial consistia de lesões da mucosa bucal; 71,4% apresentaram complicações durante o tratamento, piodermites sendo a mais freqüente. CONCLUSÕES: Não cumprir o tratamento e ter a forma clínica generalizada são fatores de risco para o desenvolvimento de complicações durante o tratamento de pênfigo foliáceo endêmico. Peru realmente tem casos de pênfigo vulgar endêmico com características epidemiológicas semelhantes às do pênfigo foliáceo endêmico. Viver numa área rural pode ser um fator de risco para o desenvolvimento de complicações.

Refractory pemphigus vulgaris associated with herpes infection: case report and review / Pênfigo vulgar refratário com infecção herpética associada: relato de caso e revisão da literatura

BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune disease characterized by blistering of the skin and mucosa, which develops due to the interaction between predisposing genetic and environmental factors. Infections caused by members of the Herpesviridae family have been suggested as a possible triggering factor for PV. OBJECTIVE AND METHODS: In this report, we investigate the presence of herpesviruses in refractory lesions on the right upper eyelid. The lesion has persisted despite the treatment with corticosteroids. Polymerase chain reaction (PCR) and DNA sequence analysis have been used to detect the DNA of HSV 1/2, VZV, EBV, CMV, HHV-6, HHV-7, and HHV-8. RESULTS: The sample collected from the right upper eyelid has tested positive for HSV 1/2. Sequence analysis has confirmed the PCR results and allowed the identification of the HSV strain as belonging to type 1. After treatment with acyclovir, the lesion of the right upper eyelid has cleared and not relapsed. CONCLUSION: When patients present PV lesions which are refractory to corticosteroid therapy, herpetic infection should be considered.

INTRODUÇÃO: Pênfigo vulgar (PV) é uma doença auto-imune caracterizada por bolhas na pele e mucosas, que se desenvolve devido a interações entre predisposição genética e fatores ambientais. Infecções por vírus da família herpesviridae são sugeridos como possíveis gatilhos para PV. OBJETIVOS E MÉTODOS: Neste relato investigamos a presença de viroses herpéticas em lesão refratária da pálpebra superior direita de uma paciente portadora de PV. A lesão persistiu mesmo após tratamento com corticoesteróides. Reação em cadeia da polimerase (PCR) e análise de sequenciamento de DNA foram usados para detectar o DNA do HSV1/2, VZV, EBV, CMV, HHV-6, HHV-7, e HHV-8. RESULTADOS: A amostra coletada da pálpebra superior direita foi positiva para HSV1/2. O seqüenciamento confirmou o resultado do PCR e identificou a cepa do HSV pertencendo ao tipo 1. Após o tratamento com aciclovir a lesão foi cicatrizada e não recidivou. CONCLUSÃO: Quando pacientes portadores de PV forem refratários à corticoterapia, infecção herpética deverá ser considerada.

Enfermedades virales y autoinmunes en pacientes con pénfigo foliáceo endémico y su influencia en la inmunología de la enfermedad / Viral and autoimmune disease in endemic penfigus foliaceus patients and their influence over the disease immunology

Objetivo: Determinar la presencia de enfermedades virales y autoinmunes en pacientes con pénfigo foliáceo endémico (PFE) y su influencia sobre la inmunología de la enfermedad. Material y métodos: Estudio prospectivo realizado en la provincia de Coronel Portillo (Ucayali-Perú) de octubre del 2001 a septiembre del 2005. La muestra estuvo constituida por 15 pacientes con PFE captados en el Hospital Regional de Pucallpa así como en comunidades y/o distritos que cumplen con las características de foco endémico (Nueva Requena, Campo Verde, Yarinacocha) a los que se les realizó inmunofluorescencia indirecta, ELISA para anticuerpos anti desmogleína 1 y anti desmogleína 3. Para confirmar el diagnóstico previo de fiebre del dengue se evaluó la presencia de anticuerpos lg G mediante ELISA de captura para virus del dengue. Resultados: Para las enfermedades virales la prevalencia fue de 26.7 por ciento, se presentó un caso de herpes simple, un caso de erupción variceliforme de Kaposi y dos casos de fiebre del dengue. Las enfermedades autoinmunes se presentaron en tres casos, dos de psoriasis vulgar y otro de vitíligo no segmentario localizado. La presencia de enfermedades virales y/o autoinmunes no influyeron significativamente en los valores índices de anticuerpos antidesmogleína 1 e inmunofluorescencia indirecta. Conclusiones: Existe una baja frecuencia de enfermedades virales y/o autoinmunes en pacientes con PFE, y cuando se presentan no influyen significativamente en la inmunología de la enfermedad.

Study of upper gastrointestinal tract involvement in pemphigus by esophago-gastro-duodenoscopy.

INTRODUCTION: Involvement of upper gastrointestinal tract in pemphigus vulgaris is not uncommon. AIM: To study the involvement of upper gastrointestinal tract (UGIT) with the help of esophago-gastro-duodenoscopy (EGD) in patients of vesiculobullous dermatoses with emphasis on pemphigus vulgaris. METHODS: Forty-two patients (M-22, F-20) with vesiculobullous dermatoses, diagnosed on the basis of clinical features and skin histopathology as pemphigus vulgaris (PV)-40 patients and pemphigus foliaceus (PF)-2 patients were included in the study. The EGD was performed and mucosa of the esophagus, stomach and first part of the duodenum were examined. Mucosal biopsies were taken from the lower esophagus in 26 patients of PV and studied after H and E staining. RESULTS: On EGD, esophageal involvement was seen in 67% patients of PV (27/40). Of these, Grade I esophagitis was observed in seven, Grade II in 11, Grade III in four and Grade IV involvement was seen in five patients of PV. Three PV patients had associated esophageal candidiasis. Involvement of esophageal mucosa was also observed in one out of two patients of PF. Gastric mucosa was involved in 52% and duodenal mucosa in 20% of PV patients. Acantholysis was observed in seven out of 26 (27%) esophageal biopsies of PV patients. Two patients of PV vomited a tube-like structure, indicative of 'esophagitis dissecans superficialis'. The involvement of the gastric mucosa in patients with history of oral corticosteroid intake (60%) was compared to the group without history of oral corticosteroids (30%). CONCLUSION: Among PV patients under study, significant involvement of oral (87%), esophageal (67%), gastric (52%) and duodenal mucosa (20%) was observed.

Prevalencia de disfunción tiroidea primaria en pacientes con pénfigo vulgar / Prevalence of primary thyroid disorders in patients with pemphigus vulgaris

Introducción. Los pénfigos representan un grupo de enfermedades autoinmunes caracterizadas por la formación de ampollas. Dentro de este grupo, el pénfigo vulgar es la forma clínica más frecuente. Por otro lado, la patología tiroidea tiene elevada prevalencia en la práctica médica y está asociada con un amplio rango de trastornos cutáneos. Hay publicaciones aisladas de la asociación entre patología tiroidea y pénfigo vulgar. Por esta razón, se decidió establecer la prevalencia de disfunción tiroidea primaria y autoinmunidad tiroidea en pacientes con pénfigo vulgar. Objetivos. (i) Determinar la prevalencia de disfunción tiroidea primaria y autoinmunidad tiroidea en pacientes con pénfigo vulgar. (ii) Relacionar los hallazgos con lo referido en la bibliografía. Material y métodos. Se efectuó un estudio observacional, transversal, prospectivo y controlado, entre marzo del 2002 y marzo del 2004, en 28 pacientes con diagnóstico de pénfigo vulgar que concurrieron a la Sección Ampollares de la División Dermatología y fueron evaluados posteriormente por endocrinólogos de la División Endocrinología, en el Hospital de Clínicas José de San Martín. La evaluación tiroidea fue realizada en 28 pacientes consecutivos con pénfigo vulgar y 40 voluntarios sanos (controles) apareados por sexo y edad, y consistió en palpación tiroidea, prueba de funcionalidad tiroidea mediante dosaje en sangre de las hormonas T3, T4, TSH, y prueba de autoinmunidad tiroidea mediante dosaje en sangre de anticuerpos antiperoxidasa...

Prevalence of thyroid autoimmunity in patients with pemphigus vulgaris

El pénfigo vulgar (PV)es una enfermedad ampollar clásica de etiología autoinmune que se caracteriza por la presenciade lesiones intraepiteliales. Para establecer la prevalencia de anormalidades tiroideas en el PV, realizamos unestudio prospectivo en 15 pacientes consecutivos que consultaron a la División Dermatología debido a PV y enun grupo de 15 voluntarios sanos (Grupo Control). La función tiroidea se evaluó a través de la medición de T3,T4 y TSH y la presencia de bocio se determinó por medio de la palpación tiroidea. La autoinmunidad se investigóusando un ensayo IRMA para la medición de anticuerpos antitiroperoxidasa (ATPO). En cada grupo había9 mujeres y 6 hombres que fueron apareados por edad y sexo, con edades comprendidas entre 25 y 65 años(promedio 48.2 años) en el grupo PV, y entre 25 y 69 años (promedio 45.4 años) en el grupo control. Se encontróque 7 pacientes (46.6%) del grupo PV y uno (6.6%) del grupo control presentaron alteraciones tiroideas,(p<0.015). La presencia de ATPO positivos se observó en 6 pacientes con PV y en un voluntario del grupocontrol. Un paciente con PV presentó un bocio difuso asociado a un hipotiroidismo subclínico con ATPO negativos.De los 7 casos con ATPO positivos, solo un paciente del grupo PV presentó una tiroiditis de Hashimotomanifiesta. Todos los demás casos solo tuvieron ATPO positivos sin evidencias clínicas de tiroiditis crónica. ElPV se asocia frecuentemente con ATPO positivos, aunque la mayoría de los pacientes no presentan enfermedadestiroideas que se expresen clínicamente..

Pemphigus in Guilan: Clinical and epidemiologic features of 126 hospitalized patients

Pemphigus is a serious blistering disease with considerable mortality, which heavily burdens on health care system because of the long time hospitalization. It is rare in North America but is considered as the most common immunobullous disease in eastern countries such as Malaysia and China. The purpose of this study was to define the epidemiologic features of pemphigus in north of Iran. One-hundred and twenty-six cases of pemphigus diagnosed based on clinicohistological findings that were hospitalized in Rasht, in north of Iran, between 1995-2001 were studied. Epidemiological data collected from their hospital records were entered in SPSS software and statistical analyses were performed by [t] test, chi-square and Pearson correlatrion tests. Pemphigus vulgaris was the most common type [83%], followed by foliaceous [6%] and vegetans [1%]. The mean age of patients was 45.6 years, with 47.2 in vulgaris and 37.8 in foliaceous group [P<0.05]. There was no sex difference in mean age in vulgaris group, whereas the mean age of foliaceous group was 30.2 for females and 51.8 years for males [P<0.05]. Female: male ratio was 1.5:1, with 1.44: 1 for vulgaris and 1.85:1 for foliaceous groups, respectively. The oral mucosa was the first site of involvement in 63% of vulgaris cases, and 72.4% of them had widespread mucocutaneous disease at the time of hospitalization. Frequency of illness in spring [32%] was higher than other seasons. The mean time for 80% improvement of lesions in hospital was significantly higher in cases with widespread mucocutaneous lesions than cases with only skin or mucosal illness [P<0.05]. These findings clearly demonstrate the need for continuous training of involved health workers [dermatologists, dentists, otolaryngologists, general physicians] for early diagnosis and referral of disease, which not only could decrease the morbidity and mortality, complications rates for patients but also hospitalization costs for patients and health care system

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

Pénfigo vulgar bucal precedido de lesiones cutáneas: reporte de un caso / Oral pemphigus vulgaris preceded by cutaneous lesions

El pénfigo vulgar forma parte de un grupo de enfermedades que se manifiestan por ampollas en piel y membranas mucosas, apareciendo con muy alta frecuencia en la mucosa bucal. Generalmente se presenta primero en la boca, por lo que es de suma importancia que el odontólogo esté familiarizado con la enfermedad con el fin de diagnosticarla y tratarla precozmente, previniendo que las lesiones cutáneas aparezcan y haciendo que el tratamento sea más sencillo. Se presenta una revisión bibliográfica y se reporta el caso de una paciente que asistió a la consulta del Servicio de Clínica Estomatológica de la Facultad de Odontología, UCV, con manifestaciones bucales de pénfigo vulgar. El objetivo principal de este artículo es ofrecer al odontólogo una información detallada de esta patología, ya que es necesario que esté capacitado para diagnosticarla y tratarla en conjunto con el patólogo clínico

Coexistencia de pénfigo vulgar y liquen plano en mucosa orofaríngea / Coexistence of pemphigus vulgaris and lichen planus in the oropharyngeal mucosa

Se presenta un caso donde se asocia la presencia en mucosa oral de pénfigo vulgar y liquen plano en una mujer de 32 años de edad, confirmado por histopatología e inmunofluorescencia. La paciente fue tratada con metilprednisona y azatioprina con buena respuesta terapéutica a partir de los 7 meses de instituida la misma. La paciente no presentó hasta el momento lesiones cutáneas ni tampoco indicios de enfermedad tumoral, por lo que excluimos el diagnóstico de pénfigo paraneoplásico y afirmamos la simple coexistencia de pénfigo vulgar y liquen plano. Se discute la probable causa de esta asociación