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Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the mucous membrane and skin. Typically, oral lesions appear before skin lesions, and in a majority of the cases only oral lesions are present. The dentist may then be the first to recognize and diagnose this disease. It is unusual for PV to present over the gingiva as a primary site of involvement. Diagnosis is based on clinical presentation and confirmed by histopathological study. Early diagnosis and management can prevent the uneven life- threatening effects of this potentially chronic mucocutaneous disorder. The case serves to enhance our awareness of the gingiva as a site at which systemic disease can manifest itself.

Background: Cutaneous manifestations are fairly common in rheumatoid arthritis (RA) and they can help in early diagnosis, prompt treatment, and hence reduced morbidity from the disease. Aims: The objective of the present study was to find out the different patterns of dermatoses in a group of patients with RA from Eastern India. Methodology: Consecutive patients fulfilling the American Rheumatism Association 1987 revised criteria for the classification of RA and who had different dermatoses were included in this cross-sectional study done over a period of 8 years in a tertiary care hospital in Eastern India. Thorough clinical examination and appropriate laboratory investigations were performed as needed. Data were recorded in a predesigned schedule, and appropriate statistical analysis was done. Results: We studied 111 evaluable patients with an age range of 19–71 years and a female to male ratio of 7:1. The mean disease duration of RA was 6.5 years. Cutaneous infections as a group was the most common mucocutaneous manifestation (34.2%) followed by xerosis including ichthyotic skin changes (27%), pigmented purpuric dermatoses (14.4%), leg ulcer (9.9%), periungual telangiectasia (9.9%), rheumatoid nodules (RNs) (8.1%), purpura and ecchymoses (7.2%), small vessel vasculitis in (7.2%), corn and callosities (6.3%), palmar erythema (4.5%), and neutrophilic dermatosis (4.5%). Raynaud's phenomenon was found in 3.6% patients and panniculitis in (3.6%) patients. Rheumatoid factor (RF) and anti-cyclic citrullinated peptides antibody were positive in 74.8% and 88.3% patients, respectively. No statistically significant difference of incidence of leg ulcer, small vessel vasculitis, RN, or Raynaud's phenomenon could be noted between RF positive and negative groups. Limitations: Being an institution-based study, the study findings may not reflect the true situation in the community which remained a limitation of this study. Conclusion: While some of the features of this study were analogous to Western data, other features showed discordance which may be due to ethnic variations among the patients with RA.

Nasal angiomyolipoma (AML) are extremely rare tumors and so far

BACKGROUND: Treatment of cutaneous leishmaniasis (CL) and mucosal leishmaniasis (ML) in travelers is still controversial. Over the last decade, national and international consortia have published recommendations for treating CL in travelers. These guidelines harmonize many issues, but there are some discrepancies. METHODS: Leishmania parasites causing CL can now be genotyped by polymerase chain reaction techniques for detecting Leishmania DNA. Therefore, treatment recommendations can now be species based rather than based on geographical exposure. To review the evidence on which the recommendations were based, "LeishMan" (Leishmaniasis Management), a group of experts from 13 institutions in eight European countries, performed a PubMed MEDLINE) literature search and considered unpublished evidence and the experts' own personal experiences. The Oxford evidence grading system was used to evaluate the information. RESULTS AND CONCLUSION: In this article, the authors provide practical treatment recommendations for imported CL and ML in Europe, drawn up from the review by the European experts.

Leishmaniasis is endemic in Sardinia but only cutaneous and visceral cases have been reported to date. We report a case of mucocutaneous leishmaniasis as presentation of HIV infection in a Sardinian patient who had never visited endemic areas. Serological and clinical diagnosis was cytologically and histopathologically confirmed. The patient had a good response to treatment with liposomal amphotericin combined with highly active antiretroviral therapy without recurrences after four years. Our case report highlights the need to better assess the circulation of species, risk factors and clinical spectrum of Leishmania infection in the Italian Mediterranean islands.

Background: Tuberous sclerosis complex (TSC) is an autosomal dominant neurocutaneous disease resulting in a wide array of clinical manifestations, primarily affecting the skin and central nervous system. Mucocutaneous features play a very important role in the recognition of this syndrome. Aims: To review the prevalence and patterns of cutaneous manifestations in tuberous sclerosis, in a group of patients from eastern India. Methods: Observational clinical study on twenty-seven consecutive patients of tuberous sclerosis collected during a period of four years. Results: Most were between 10-20 years of age; the male to female ratio was 2:1. Family history was found in two-thirds. The classical triad of tuberous sclerosis was present in only nine (33.3%) patients. Adenoma sebaceum was the most common cutaneous feature (100%), followed by hypomelanotic macules (92.6%), connective tissue nevi (66.6%), and Koenen′s tumors (33.3%). Oral mucosal fibromas were seen in six (22.22%) patients. Fibromatous plaque over forehead and scalp was seen in three patients. Limitation of the study was small size of study sample. Conclusion: Prominent mucocutaneous changes are extremely common manifestation of TSC, which may provide crucial diagnostic clues for primary care physicians.

BACKGROUND: American tegumentary leishmaniasis has an annual incidence of 1 to 1.5 million cases. In some cases, the patient's immune response can eliminate the parasite, and the lesion spontaneously resolves. However, when this does not occur, patients develop the disseminated form of the disease. OBJECTIVE: To investigate the association between clinical, laboratory and pathological findings in cases of American tegumentary leishmaniasis. METHODS: A retrospective study of the medical records of 47 patients with American cutaneous leishmaniasis. Clinical, laboratory and epidemiological data were collected, and semi-quantitative histopathological analyses were performed using the Spearman correlation coefficient (p

The heat-shock protein 70 gene (hsp70) has been exploited for Leishmania species identification in the Old and New World, using polymerase chain reaction (PCR) followed by restriction fragment length polymorphism analysis. Three new Leishmania-specific hsp70 PCRs were recently described, and we applied 2 of these on 89 clinical samples from a total of 73 Peruvian patients with either cutaneous or mucocutaneous leishmaniasis. The new PCRs on average showed a 2- to 3-fold improved sensitivity in the tested sample types (lesion biopsies, aspirates, and scrapings), for both genus detection and species typing, and were most successful in biopsies. Leishmania braziliensis, L. peruviana, and L. guyanensis were encountered. About one third of the L braziliensis parasites contained 2 hsp70 alleles. This study is a paradigm for the implementation of a globally applicable upgraded tool for the identification of Leishmania directly on human specimens from cutaneous and mucocutaneous lesions in the New World. (C) 2012 Elsevier Inc. All rights reserved.

In South Brazil, American tegumentary leishmaniasis is predominant at Paraná, specially on the North and West areas, where it is localized one of the disease’s production circuits. It is believed that 3 to 5% of the cases evolve to the mucosal form, and that 1% of these can evolve to death. There is a long time that first choice drugs to treat this disease are the antimony compounds as pentavalent salts. The purpose of this study was to describe epidemiological and treatment characteristics of patients with mucosal leishmaniasis, who were diagnosed during the period from 2001 to 2007 in cities located on the coverage area of the 13 Heath Regional, which is situated in Cianorte, Paraná. The data were collected at the outpatient medical records and the System of Notifications and Information of Diseases epidemiological investigation records. During the mentioned period, 505 cases of American tegumentary leishmaniasis were notified, 30 of them (6%) as the mucosal form. In this study, the patients included were those with diagnosis of mucosal leishmaniasis that had complementary exams performed before and during the treatment. The results showed the need of patients’ careful monitoring by health teams, due to the possible evolution of the disease and the presence of unwanted drug effects. En la región Sur de Brasil, la leishmaniasis tegumentaria americana predomina en el estado de Paraná, principalmente al Norte y Oeste del país, donde está localizado uno de los circuitos de producción de la enfermedad. Se estima que de 3 a 5% de los casos de leishmaniasis cutánea desenvuelvan lesión mucosa, y que cerca de 1% de ellas puedan acabar en muerte. Por mucho tiempo, las drogas escogidas para tratar la enfermedad son los compuestos antimoniales bajo la forma de sales pentavalentes. El objetivo de este estudio fue describir características epidemiológicas y de tratamiento de los pacientes con leishmaniasis mucosa, diagnosticados en el periodo de 2001 a 2007, en municipios localizados en el área de estudio de la 13ª Regional de la Salud, localizada en el municipio de Cianorte, Paraná. Las informaciones fueron colectadas del registro médico y de los papeles de investigación epidemiológicos del SINAN (Sistema de Notificación e Información de Agravios). En el periodo estudiado, fueron notificados 505 casos de leishmaniasis tegumentaria americana, 30 de los cuales (6%) en forma mucosa. Fueron incluidos en el estudio pacientes diagnosticados con leishmaniasis mucosa, que poseían exámenes complementares realizados antes y durante el tratamiento. Los resultados evidenciaron la necesidad de cuidadosamente acompañar los enfermos en los equipos de salud, dada la posible evolución y la presencia de efectos medicamentosos indeseables en los pacientes afectados por la condición. Na região Sul do Brasil, a leishmaniose tegumentar americana predomina no Estado do Paraná, especialmente ao Norte e Oeste, onde um dos circuitos de produção da doença é localizado. Estima-se que 3 a 5% dos casos de leishmaniose cutânea desenvolvam lesão mucosa, e que cerca de 1% destas podem evoluir para óbito. De longa data, as drogas de escolha para tratamento da doença são os compostos antimoniais sob a forma de sais pentavalentes. O objetivo deste estudo foi descrever características epidemiológicas e de tratamento dos pacientes com leishmaniose mucosa, diagnosticados no período de 2001 a 2007, em municípios localizados na área de abrangência da 13ª Regional de Saúde, com sede no município de Cianorte, no Paraná. As informações foram coletadas do prontuário ambulatorial e das fichas de investigação epidemiológicas do Sistema de Notificação e Informação de Agravos. No período estudado, foram notificados 505 casos de leishmaniose tegumentar americana, 30 deles (6%) na forma mucosa. Foram incluídos no estudo pacientes com diagnóstico de leishmaniose mucosa, que apresentavam exames complementares realizados antes e durante o tratamento. Os resultados evidenciaram a necessidade de acompanhamento cuidadoso dos doentes nas equipes de saúde, dado a possível evolução e presença de efeitos medicamentosos indesejáveis nos pacientes afetados pela condição.

Skin is one of the most frequently involved organs in human immunodeficiency virus (HIV) infection, and mucocutaneous manifestations may be one of the earliest markers of AIDS. The prevalence of cutaneous abnormalities in HIV approaches nearly 90%. Mucocutaneous manifestations may also act as a prognostic marker of HIV infection. Children are increasingly being affected by HIV infection and it is important to realize the presence of the infection early in the disease process as their immune status is not mature enough to handle the stress of various infections. Skin manifestations can serve as early markers and prognostic indicators of HIV infection. This review highlights the epidemiology, transmission, pathogenesis, and the mucocutaneous manifestations of HIV infection in children.

Dengue viral infection is a cause of considerable morbidity and mortality and may be associated with a variety of mucocutaneous manifestations that may provide important early clues to the diagnosis of this condition. Cutaneous and mucosal findings like confluent erythema, morbilliform eruptions, and hemorrhagic lesions may figure prominently in the clinical features of dengue. The differential diagnoses include a large number of bacterial and viral exanthems as well as drug rash.

Chikungunya fever (CF) is an arboviral acute febrile illness transmitted by the bite of infected Aedes mosquitoes. After a quiescence of more than three decades, CF has recently re-emerged as a major public health problem of global scale. CF is characterized by an acute onset of high fever associated with a severe disabling arthritis often accompanied by prominent mucocutaneous manifestations. The disease is usually self-limiting, but the joint symptoms and some of the cutaneous features may persist after the defervescence. A wide range of mucocutaneous changes has been described to occur in association with CF during the current epidemic. Besides a morbilliform erythema, hyperpigmentation, xerosis, excoriated papules, aphthous-like ulcers, vesiculobullous and lichenoid eruptions, and exacerbation of pre-existing or quiescent dermatoses had been observed frequently. These unusual features may help in the clinical differential diagnosis of acute viral exanthems mimicking CF.

Se presenta el caso de un hombre de 74 anos procedente del area rural de Caicedonia, Valle del Cauca, con diagnosticos de hipertension arterial no controlada, insuficiencia renal cronica estadio IV y anemia grave. Tenia antecedentes de leishmaniasis cutanea en los parpados del ojo derecho, el pabellon auricular izquierdo y las extremidades, diagnosticada 15 anos atras en el departamento del Guaviare. Recibio tratamiento incompleto con antimoniales en esa epoca. Consulto al Centro Internacional de Entrenamiento e Investigaciones Medicas (CIDEIM) por un cuadro progresivo de ocho anos de evolucion de lesiones mucosas ulceradas en el labio superior, la mucosa nasal y los parpados del ojo derecho (figura 1). Se hizo un diagnostico histopatologico de leishmaniasis, confirmado mediante reaccion en cadena de la polimerasa (figura 2). Debido a las enfermedades concomitantes del paciente, el tratamiento con antimoniales (Glucantime®) estaba contraindicado. Se administro tratamiento supervisado intrahospitalario con miltefosine (Impavido®, capsulas de 50 mg) a una dosis diaria de 1,8 mg/kg por 28 dias, de acuerdo con las guias nacionales. Se realizo control clinico y de laboratorio durante el tratamiento y despues de finalizado, sin evidencia de ningun tipo de complicacion. El paciente asistio a controles medicos hasta la octava semana despues del tratamiento, en los cuales presento mejoria clinica de las lesiones (figura 3). Se remitio para continuar el manejo complementario por medicina interna, oftalmologia y cirugia plastica. La leishmaniasis mucocutanea es una grave complicacion evitable de la leishmaniasis cutanea. Este caso muestra fallas en el diagnostico y tratamiento oportunos y, en general, en el programa de control de esta enfermedad. Por otra parte, el miltefosine surge como una opcion terapeutica a los antimoniales para el tratamiento de pacientes en quienes esten contraindicados o presenten alto riesgo de toxicidad.

Bean syndrome or blue rubber bleb syndrome is a rare disorder of vascular channels with mucocutaneous manifestations. The clinical severity varies among patients from being totally symptom free to fatal bleeding resulting in anemia. As the histopathologic findings are not pathognomonic, diagnosis is usually made from the clinical presentation, though the imaging techniques reveal extension of the lesion. We report an asymptomatic case of blue rubber bleb syndrome who reported for dental treatment with oral and cutaneous manifestations.

Se relatan los estudios ecológico-epidemiológicos realizados en 1975 en la parte alta del valle de Tambopata (Alto Tambopata), en relación con la leishmaniasis tegumentaria o espundia y con la esperanza de que los resultados de esta investigación puedan posteriormente orientar campañas preventivas contra la espundia. Se practicó la inoculación intradérmica del antígeno leishmanina, la reacción de Montenegro en un total de 556 personas, obteniéndose 116 casos positivos. Se relacionó la intensidad de la reacción con las localidades donde se practicó la reacción de Montenegro. Se realizó la búsqueda de infección leishmaniásica en mamíferos selváticos que fueron capturados y en los mamíferos domésticos de la zona, de los cuales tres perros, una mula y un asno tenían lesiones cutáneas comparables a las que da lugar la leishmaniasis tegumentaria en la especie humana. En los cultivos realizados a partir de las lesiones cutáneas en ocho casos humanos de leishmaniasis tegumentaria se obtuvo en cuatro de ellos el aislamiento del agente etiológico de la espundia. A partir de los estudios entomológicos se recolectó en conjunto 868 especímenes de titiras, correspondiente a nueve especies del género Lutzomyia; L. auraensis representó 79,8% del total de titiras capturadas y L. migoni el 12,0%. Tanto por la ocurrencia de las lesiones metastásicas en las mucosas buco- naso-faríngeas, así como por la presencia de personas con reacción de Montenegro positiva y sin evidenciar infección leishmaniásica en el Alto Tambopata, presente o pasada, ésta no difiere de las que ocurren en otros países sudamericanos, donde la espundia es endémica. Ecological and epidemiological studies performed in the highlands of the Tambopata Valley (High Tambopata) during 1975, about "espundia" or tegumentary Leishmaniasis are hereby described, hoping that the findings of this research could be useful to guide preventive actions against "espundia". The intradermical inoculation of the leishmanina antigen, Montenegro reaction was used in 556 humans, and 116 positive cases were found. The intensity of the reaction was related to the sites where the Montenegro reaction was performed. Leishmania infection was sought in jungle mammals that were captured, as well as in domestic mammals living in the area; of these, three dogs, one mule and one donkey had cutaneous injuries, resembling those of tegumentary leishmaniasis in human beings. In four out of eight cultures from human cases of tegumentary leishmaniasis performed, the ethiological agent of "espundia" was isolated. As a result of entomological studies, 868 specimens of "titiras" were captured, corresponding to nine species of the Genus Lutzomyia; the findings point out that 79.8% of the insects were L. auraensis and 12% L. migoni. The occurrence of metastasic injuries in the oral, nasal and pharingeal mucosae, as well as the presence of positive Montenegro reaction, without evidence of any past or present Leishmaniasis infection in Alto Tambopata shows that "espundia" does not differ from the disease that occurs in other South American countries where "espundia" is endemic.

Background: Lichen planus (LP) is a mucocutaneous disease that is relatively common among adult population. LP can present as skin and oral lesions. This study highlights the prevalence of oral, skin, and oral and skin lesions of LP. Aims: The aim of this study was to evaluate the prevalence of oral, skin, and oral and skin lesions of LP from a population of patients attending the Department of Oral Medicine and Radiodiagnosis, Pushpagiri College of Dental Sciences, Tiruvalla, Kerala, India. Materials and Methods: A cross-sectional study was designed to evaluate the prevalence of oral, skin, and oral and skin lesions of LP. This is a ongoing prospective study with results of 2 years being reported. LP was diagnosed on the basis of clinical presentation and histopathological analysis of mucosal and skin biopsy done for all patients suspected of having LP. Statistical analysis was carried out using SPSS (Statistical package for social sciences) software version 14. To test the statistical significance, chi-square test was used. Results: Out of 18,306 patients screened, 8,040 were males and 10,266 females. LP was seen in 118 cases (0.64%). Increased prevalence of LP was observed in middle age adults (40–60 years age group) with lowest age of 12 years and highest age of 65 years. No statistically significant differences were observed between the genders in skin LP group (P=0.12) and in oral and skin LP groups (P=0.06); however, a strong female predilection was seen in oral LP group (P=0.000036). The prevalence of cutaneous LP in oral LP patients was 0.06%. Conclusion: This study showed an increased prevalence of oral LP than skin LP, and oral and skin LP with a female predominance.

A case of mucocutaneous nasal histoplasmosis in an immunocompetent host is described below. A 30-year-old male had a broadened nose with swelling and repeated blockage of nasal passages for the past six months. Diagnosis was made on the basis of histological demonstration of characteristic yeast cells of Histoplasma capsulatum var. capsulatum occurring within histiocytes and extracellularly in stained smears of fine needle aspirates and biopsy from the lesions in ala of the nose and perioral region. The patient showed appreciable regression of lesions after three weeks of itraconazole therapy but was not available for re-assessment.

We have recently published a case of nosocomial dengue infection that was transmitted by mucocutaneous exposure to blood from a febrile traveler who had recently returned from Peru (6). During phlebotomy, a healthcare worker was splashed in the face with the traveler’s blood. Both the traveler and the healthcare worker were subsequently found to have dengue fever with dengue virus type 3. This route of infection is biologically plausible because infection through mucosal surfaces (intranasal and oral routes) has been shown pos