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Background Infective dermatitis associated with human T-cell lymphotropic virus type-1 (HTLV-1), (IDH), is a chronic eczema occurring in HTLV-1 infected children. Rare cases of adulthood IDH have been reported and no study until now aimed to compare juvenile and adulthood IDH. Methodology/Principal findings Twelve cases of adulthood IDH followed for a mean time of 7.5 years were analyzed according to clinicopathological and molecular aspects, comparing them to juvenile IDH cases. Diagnosis was based on the modified major criteria used for juvenile IDH. Proviral load (PVL) assessment was performed by real-time PCR technique. Adulthood IDH presented similar clinicopathological and molecular aspects compared to juvenile IDH. The morphology of lesions and areas of involvement were similar, except for the involvement of the ankles and inframammary folds in the adulthood form. HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP) occurred in six adulthood IDH patients, with almost equal frequency. However, at least in two patients, HAM/TSP appeared prior to IDH, differently from what was observed in juvenile IDH. Conclusions/Significance Adulthood IDH is similar to juvenile IDH according to clinicopathological aspects and PVL levels. Therefore, the same modified major diagnostic criteria for juvenile IDH can be applied to both forms., Author summary HTLV-1 infection is most prevalent in developing countries, and in Brazil, it is most prevalent in poor black communities. The majority of infected people are asymptomatic, but a minority can develop adult T-cell leukemia/lymphoma, HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP) and/or infective dermatitis associated with HTLV-1 (IDH), the subject of this study. IDH is an exudative chronic eczema that always involves the scalp and retroauricular regions and is characterized by its prompt response to antimicrobial therapy. First described in Jamaican children in the sixties, only in 2001 adulthood onset of IDH was reported, and since then little literature has been produced concerning it. Thus, we aimed to provide a thorough description of this relatively new subtype of IDH, investigating its clinical, pathological and molecular aspects, comparing them to juvenile IDH. Apart from minor differences, such as involvement of extra body sites and earlier onset of HAM/TSP, adulthood IDH is very similar to its juvenile counterpart, and the same diagnostic criteria can be applied to both.

According to the WHO, sexual health is not merely the absence of disease. Sexual dysfunction may be present in 40.8% of psoriasis patients, furthermore, 68% prevalence was found in Brazilian women with psoriasis. The moderate prevalence of psoriatic lesions in the genital area (35%–42%) does not explain the alarming prevalence of sexual dysfunction. Other factors, such as anxiety, depression, and also psoriasis treatment may contribute to its development. Likewise, atherosclerosis of the pelvic vasculature is involved in the pathogenesis of erectile dysfunction. Risk factors for erectile dysfunction tend to be confused with the comorbidities seen in psoriasis patients. We also highlight that it may serve as a marker of cardiovascular risk.

Background Human T-cell lymphotropic virus type-1 (HTLV-1) may cause severe diseases such as HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and infective dermatitis associated with HTLV-1 (IDH). The clinical characteristics and progression of 25 early onset HAM/TSP associated or not to IDH were described. Methods Following-up 37 IDH patients with neurological examinations, 54% developed HAM/TSP. To these cases were added 5 cases of juvenile HAM/TSP. The patients were HTLV-1+ and were submitted to dermatological and neurological examinations. Diagnosis of HAM/TSP was performed according to Osame et al (1990) and Castro-Costa et al (2006) criteria. Results Twenty-one patients were classified as definite HAM/TSP by both criteria, 3 as probable HAM/TSP by Osame et al, and another as probable HAM/TSP according to Castro-Costa et al Median age at onset of neurological manifestations was 9 years for the IDH/HAM/TSP group and 16 years for the HAM/TSP group (P = .045). In 12 patients, the onset of neurological manifestations occurred when they were less than 10 years of age. In the group IDH/HAM/TSP, the neurological symptoms always begun during the period of activity of IDH. The progression of HAM/TSP evaluated in 17 cases was heterogeneous, and 3 had rapid progressive course. Conclusions The juvenile HAM/TSP may occur very early and also presents marked female predominance. Progression of IDH to HAM/TSP before 19 years of age is frequent (54%). Rapid progressive form may also occur in early HAM/TSP. As juvenile IDH and HAM/TSP are due to vertical transmission through breastfeeding, it is very important to avoid this pathway of infection.

Introduction: Hemangiosarcoma is a rare vascular malignant neoplasm. Diagnosis is based on both clinical and histopathological features. Case Presentation: An 85-years-old white man presented with a 1-year history of progressive lesions on face and scalp. Physical examination revealed violaceous infiltrative plaques, with ulceration and bleeding, on the face and scalp, associated to numerous violaceous satellites papules. Histopathological examination revealed a neoplastic proliferation represented by slit-like anastomosing vascular channels that dissociated collagen fibers in the dermis, lined by atypical endothelium with moderately hyperchromatic and pleomorphic nuclei. Diagnostic conclusion was cutaneous angiosarcoma. Conclusions: Cutaneous angiosarcoma most often affects males (2:1), mostly Caucasian patients over 70 years old. It may originate from blood or lymphatic vessels. The most common form affects the face and scalp. There are varying degrees of cellular atypia, anastomosing slit-like channels, dissociation of collagen fibers, occasional hyaline globules and areas of hemorrhage. Immunohistochemistry confirmed blood vascular origin with diffuse positivity for CD34 and focally diffuse for factor VIII. We emphasize the need of clinicopathological correlation for early recognition and treatment.

BACKGROUND: Psoriasis is an immune-mediated disease that manifests predominantly in the skin, although systemic involvement may also occur. Although associated comorbidities have long been recognized and despite several studies indicating psoriasis as an independent risk factor for cardiovascular events, little has been done in general medical practice regardind screening. In the United States, less than 50% of clinicians are aware of these recommendations. OBJECTIVE: To identify the prevalence of these comorbidities in 296 patients followed up at a university dermatology clinic. METHODS: Systematically investigated comorbidity frequencies were compared with general practitioners' registry frequencies. Clinical features correlated with comorbidities were also investigated. RESULTS: High prevalences of systematically investigated comorbidities such as hypertension (30%) and dyslipidemia (26.5%) were documented. Conversely, data from general practitioners' records showed that 33% of dyslipidemia cases were undiagnosed and indicated possible underdiagnosis of some comorbidities. Furthermore, an association was found between: the number of comorbidities and psoriasis duration, age and high body mass index an association was found between the number of comorbidities and psoriasis duration, age, high body mass index, waist circumference or waist-to-hip ratio. (p

Psoriasis is a chronic and systemic, immune-mediated, inflammatory disease, mainly manifested by skin and / or joints lesions, presenting with a wide degree of clinical severity, but generally with great impact on patients' quality of life. Despite advances in the understanding of its pathogenesis, especially regarding the participation of T-lymphocytes and the key role of TNF, the triggering factor of the disease at the molecular level remains unknown, as well as the function of other cell populations. By presenting antigens to T-lymphocytes, monocytes assume an important role in both innate and adaptive immune response. In the last two decades, by using flow cytometry with antibodies against CD14 (receptor for lipopolysaccharide) and CD16 (low affinity receptor for IgG), human blood monocytes were classified into three subpopulations: classical (CD14++CD16-), intermediate (CD14++CD16+), and non-classical (CD14+CD16++). Under normal conditions the population of classical monocytes corresponds to about 85%, while intermediate to 5.4%, and nonclassical to 9.2%. However, intermediate and nonclassical subsets are increased in various inflammatory situations, such as moderate to severe asthma, colorectal cancer, and rheumatoid arthritis. Despite psoriasis being considered a disease of inflammatory nature, scarce studies evaluating the frequency of subpopulations of monocytes in psoriatic patients are found on current medical literature, and they are restricted to peripheral blood analysis. This study aims to identify the frequency of monocyte subpopulations in blood levels as well as lesional skin of plaque psoriasis patients, and to evaluate their association to cytokines, and clinical disease severity.

Background Psoriasis is a chronic, inflammatory skin disorder characterized by well-demarcated erythematous-scaly lesions, affecting 0-6.6% of the world population. Facial lesions are neglected in most descriptions in current literature, although some studies indicate that it could be a predictor of psoriasis severity. Objective To compare the severity of psoriasis, measured by the Psoriasis Area and Severity Index (PASI) and Dermatology Life Quality Index (DLQI), in patients with and without facial lesions. Material and methods This is a cross-sectional study, whose sample size was 63 individuals for each group. Subjects were included in the HUPES Complex Dermatology Service (Salvador/BA - Brazil) from April 2014 to January 2016. Demographic information was obtained using a prestructured form. Assessments of psoriasis severity and quality of life were done using PASI and DLQI, respectively. Results Facial lesions were detected on 63 (50.0%) of the 126 patients included. The pinna was the most frequently affected region (63.5%). Mean PASI in the group with facial involvement was 14.0 vs. 6.0, when facial involvement was absent. DLQI means for these groups were, respectively, 7.5 and 4.7. Conclusions Facial involvement in a psoriasis patient was a marker of disease severity, and its identification should influence the therapeutic decision.

Data on chronic plaque psoriasis severity and its potential clinical and lifestyle implications in the Brazilian population are limited. The primary aim of this study was to assess the clinical severity of plaque psoriasis in Brazil. Further objectives included evaluating potential associations between disease severity and demographic, lifestyle, and clinical characteristics, health-related quality of life (HRQOL), and work productivity.This observational (non-interventional) cross-sectional study was conducted in 26 dermatologic clinics across 11 Brazilian states. Psoriasis severity was assessed using investigator judgment and Finlay's Rule of Tens: a Psoriasis Area and Severity Index (PASI) score10, a Body Surface Area (BSA) 10%, or a Dermatology Life Quality Index (DLQI) score10.Among 1125 patients, 205 (18.2%) had moderate-to-severe disease. On multiple regression analyses, psoriasis severity was significantly (directly) associated with the presence of physical inactivity and comorbid pain, anxiety, and depression; and significantly (inversely) associated with HRQOL and work productivity.Cross-sectional studies cannot assess temporal trends, and observational studies cannot conclusively determine causality or exclude biases and confounding due to unmeasured variables.Among Brazilian patients with moderate-to-severe psoriasis, disease severity had far-reaching adverse impacts on lifestyle, comorbidities, HRQOL, and work productivity.

Psoriasis is an autoimmune disease associated with the production of pro-inflammatory cytokines. The identification of these molecules in the pathogenesis of psoriasis facilitated the use of monoclonal antibodies to block their actions as a treatment for severe psoriasis. An increased inflammatory response has been documented in patients with obesity, a condition that is associated with the occurrence and severity of psoriasis. Osteopontin (OPN), TNF and CXCL9 levels are enhanced in patients with psoriasis, although OPN has been documented in the adipose tissue of obese patients without psoriasis. The prevalence of obesity is much higher in psoriasis patients compared with the general population. Thus, we aimed to evaluate the relationship between cytokine levels and psoriasis in the context of obesity. We compared OPN and CXCL9 plasma levels among 117 psoriasis patients and 27 healthy body mass index-matched subjects using ELISA. We also analyzed the TNF, CCL2 and CCL5 levels in a smaller subgroup of patients and matched controls. Median OPN, CCL5 and CXCL9 levels were significantly higher in psoriasis patients compared with the controls, independent of obesity. There was no difference between the median CCL2 levels in the psoriasis patients and the controls (P

Fifteen families with clustering of infective dermatitis associated with HTLV-1 (IDH) and/or HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP) were observed among 28 families of IDH index cases, 93% of them occurring in two generations. With the exception of two mothers of children with IDH, all the mothers with HAM/TSP had at least one child with HAM/TSP. This is the first report of such clustering involving many families.

Inherited epidermolysis bullosa (EB) is a heterogeneous group of genetic disorders that present with skin and, in some cases, mucosal fragility, predisposing patients to the development of blisters and/or erosions after minimal trauma or friction. Children with a recurrent history of these kinds of lesions or neonates that present them in the absence of another reasonable explanation should be investigated. Diagnosis must be based on clinical and histopathological findings. To date, management of inherited EB basically consists in avoiding traumas that trigger lesions, as well as preventing infection and facilitating healing of the wounds with the systematic use of bandages. A epidermólise bolhosa hereditária (EBH) compreende um grupo heterogêneo de desordens genéticas que têm em comum a fragilidade cutânea e, em alguns casos mucosa, predispondo ao desenvolvimento de bolhas e/ou erosões após fricção ou trauma mínimo. Crianças com história recorrente deste tipo de lesão ou neonatos que as apresentem na ausência de outra explicação plausível devem ser investigados. O diagnóstico deve se basear em achados clínicos e histopatológicos. Até o presente momento, o manejo da EBH consiste basicamente em evitar os traumas desencadeadores das lesões, bem como evitar a infecção e facilitar a cicatrização das feridas com o uso sistemático de curativos.

Psoriasis is a systemic, chronic, immunologically mediated disease, with significant genetic and environmental influences. It affects from 1 to 3% of the world population. Recently, the relation between psoriasis and different comorbidities, particularly metabolic syndrome, has become extremely relevant. Uveitis is characterized by a process of intraocular inflammation resulting from various causes. Considering psoriasis and uveitis as immune-mediated diseases, this study aims to evaluate the possible association of psoriasis and/or psoriatic arthritis with uveitis and its subtypes. Few studies have evaluated the association of uveitis and psoriasis without joint involvement. It seems that psoriasis without arthropathy is not a risk factor for the development of uveitis. Uveitis tends to develop more frequently in patients with arthropathy or pustular psoriasis than in patients with other forms of psoriasis. Ophthalmic examination should be performed periodically in patients with psoriasis and uveitis. If ophthalmopathy is diagnosed, the patient should receive adequate treatment with anti-inflammatory drugs or immunomodulators to prevent vision loss. Psoríase é uma doença sistêmica, crônica, imunologicamente mediada, com importante influência genética e ambiental, que afeta 1 à 3% da população mundial. Nos últimos anos, a relação da psoríase com diferentes comorbidades, em especial a síndrome metabólica, tornou-se extremamente relevante. A uveíte é caracterizada por um processo de inflamação intra-ocular resultante de várias causas. Considerando a psoríase e a uveíte como doenças imunologicamente mediadas, o presente trabalho visa avaliar a possível associação da psoríase e/ou artrite psoriática com a uveíte e seus subtipos. Poucos são os estudos que avaliam a associação de uveíte e psoríase sem comprometimento articular. Parece que a psoríase sem artropatia não seria um fator de risco para desenvolvimento de uveíte. A uveíte tende a desenvolver mais frequentemente em pacientes com artropatia ou psoríase pustulosa que em outras formas de psoríase. Avaliação oftalmológica deve ser feita periodicamente em pacientes com psoríase, proporcionando ao paciente um diagnóstico precoce da oftalmopatia e a instituição de tratamento adequado com anti-inflamatórios não hormonais ou drogas imunomoduladoras, no intuito de evitar a perda da visão nos pacientes com psoríase e uveíte.

Background. Infective dermatitis associated with human T-cell lymphotropic virus type 1 (HTLV-1; IDH) is a chronic recurrent eczema affecting HTLV-1–infected children. The epidemiological and dermatological characteristics of IDH are described, and their principal diagnostic criteria are reevaluated. Methods. Forty-two patients were included: 40 patients serologically positive for HTLV-1 and 2 seronegative patients who tested positive in polymerase chain reaction (PCR) assays. Results. The mean age at onset of the disease was 2.6 ± 2.4 years (range, 2 months–11 years). The mean duration of breast-feeding was 24.2 months. The lesions were erythematous, scaly, and crusted, always affecting the scalp and retroauricular regions. Crusting of the nostrils was observed in 64.3% of the patients. Of the 36 patients followed up, 23 had the active disease. The age at which IDH disappeared in the others was 10–20 years. Conclusions. The onset of IDH may occur earlier than reported in the literature. The scalp and retroauricular regions are always affected, and lesions are invariably present in ≥3 areas. Crusting of the nostrils cannot be considered an obligatory factor for the diagnosis of IDH. The recurring nature of IDH was a characteristic found in all cases. Patients with classic IDH lesions who are serologically negative should be investigated by PCR. Therefore, the indispensable criteria for diagnosis are (1) presence of erythematous-scaly, exudative, and crusted lesions involving ≥3 areas, including the scalp and retroauricular regions; (2) recurring nature of the lesions; and (3) a finding of HTLV-1 infection by serology or molecular biology.

A psoríase pustulosa generalizada é tipo raro de psoríase descrita pela primeira vez, em 1910, por Von Zumbusch. A psoríase pustulosa generalizada na criança tende a ter um curso mais benigno que no adulto, entretanto, em sua forma grave, pode por em risco a vida do paciente. Talvez, pela raridade da doença, nenhuma opção terapêutica se mostrou consistentemente eficaz e segura até o momento. Relatos isolados sugerem ser o retinoide a droga de escolha nesses casos, porém trata-se de fármaco que, na primeira infância, traz mais dificuldades no manejo. Relata-se exemplo dessa rara doença em lactente, quadro extenso e grave, com resposta satisfatória ao uso de dapsona, com boa tolerabilidade. Generalized pustulous psoriasis is a rare type of psoriasis first described in 1910 by Von Zumbusch. This disease tends to have a more benign development in children when compared to its development in adults. However, in its serious form it may represent a risk to the patient's life. Maybe, due to being such a rare disease no therapeutic option has so far consistently proved to be both effective and safe. Isolated reports suggest that retinoid is the best choice of drug in these cases. However, it is difficult to manage this drug in early infancy. The present report describes a serious case of this rare disease in an infant with a satisfactory response and good tolerance to the use of dapsone.

Skin lesions are frequent in human T-cell lymphotropic virus type 1 (HTLV-1) infection and may constitute an alert for the diagnosis of this condition. The most severe skin diseases related to this virus are adult T-cell leukemia/lymphoma (ATLL), an aggressive form of leukemia/lymphoma that fails to respond to chemotherapy, and infective dermatitis associated with HTLV-1 (IDH), a severe and recurrent form of eczema occurring in childhood. ATLL affects the skin in 43-72% of cases. In this review, the clinical, histopathological and immunohistochemical aspects of ATLL and IDH will be discussed, as well as the differential diagnoses, giving particular focus to the primary cutaneous ATLL. IDH may progress to HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and to ATLL. Adult onset IDH and reactional and inflammatory dermatoses found in carriers and also in patients with HAM/TSP will be considered. Other dermatological diseases that occur more frequently in HTLV-1-infected individuals such as xerosis, acquired ichthyosis, seborrheic dermatitis and infectious and parasitic dermatoses will also be discussed.

Salvador (BA, Brazil) is an endemic area for human T-cell lymphotrophic virus type 1 (HTLV-1). The overall prevalence of HTLV-1 infection in the general population has been estimated to be 1.76%. HTLV-1 carriers may develop a variety of diseases such as adult T-cell leukemia/lymphoma, HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and infective dermatitis associated with HTLV-1 (IDH). IDH is a chronic and severe form of childhood exudative and infective dermatitis involving mainly the scalp, neck and ears. It has recently been observed that 30% of patients with IDH develop juvenile HAM/TSP. The replication of HTLV-1 has been reported to be greater in adult HAM/TSP patients than in asymptomatic HTLV-1 carriers. In the current study, the proviral load of 28 children and adolescents with IDH not associated with HAM/TSP was determined and the results were compared to those obtained in 28 HTLV-1 adult carriers and 28 adult patients with HAM/TSP. The proviral load in IDH patients was similar to that of patients with HAM/TSP and much higher than that found in HTLV-1 carriers. The high levels of proviral load in IDH patients were not associated with age, duration of illness, duration of breast-feeding, or activity status of the skin disease. Since proviral load is associated with neurological disability, these data support the view that IDH patients are at high risk of developing HAM/TSP.

OBJECTIVES: To review the literature on diseases linked with infection by human T-cell lymphotropic virus type I (HTLV-I) in childhood and adolescence, with focus on clinical aspects, diagnosis, pathogenesis, progression and treatment. SOURCES: Medical literature published during the last 20 years identified using PubMed and MEDLINE and from specialized medical books, with emphasis on infective dermatitis associated with HTLV-I (IDH), on the juvenile form of HTLV-associated myelopathy/tropical spastic paraparesis (HAM/TSP), on adult T-cell leukemia/lymphoma (ATL) and on HTLV-I-associated uveitis. Keywords used to search databases were: HTLV-I-associated infective dermatitis, HTLV-I-associated myelopathy/tropical spastic paraparesis, adult T-cell leukemia/lymphoma, HTLV-I-associated uveitis. SUMMARY OF THE FINDINGS: IDH is a chronic, relapsing and infected dermatitis of childhood which always involves the scalp and which may progress to HAM/TSP and ATL. HAM/TSP is a chronic and incapacitating myelopathy of adults. There are 17 well-documented cases of HAM/TSP in children and adolescents in the literature, 12 of whom are patients with IDH. In contrast with the adult form of the disease, the juvenile form is rapid and progressive. ATL is a type of T-cell leukemia/lymphoma that affects adults and is generally fatal. Eleven of the 24 published reports of ATL in children and adolescents were diagnosed in Brazil. CONCLUSIONS: These diseases are likely to be more common in childhood and adolescence than the literature would suggest. It is advisable that serological testing be performed for HTLV-I in children and adolescents suffering from chronic and relapsing eczema, with signs and symptoms of myelopathy or with a diagnosis of T-cell leukemia/lymphoma. It is important that pediatricians know how to recognize the pediatric manifestations of this infection in order to correctly diagnose them and offer their patients appropriate guidance and treatment.

Human T cell lymphotropic virus type 1 (HTLV-1)-associated infective dermatitis (IDH) is a chronic and recurrent eczema occurring during childhood and adolescence. HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic myelopathy of adulthood presenting with slowly progressive spastic paraparesis and sphincter dysfunction with mild sensory involvement. There are few reports describing an association between IDH and HAM/TSP. The objective of this study was to evaluate the occurrence of HAM/TSP in patients with IDH and in seropositive members of their families and to determine the blood levels of antibodies against HTLV-1 in patients with HAM/TSP. Twenty patients with IDH and their seropositive mothers and siblings underwent clinical neurological and laboratory evaluations. The diagnosis of HAM/TSP was made in accordance with the World Health Organization criteria. Nine individuals had HAM/TSP (6 of the patients with IDH 2 mothers and 1 seropositive brother). In 3 families 11 individual had HAM/TSP. The serum antibody titers of the patients with HAM/TSP varied from 1:3.125 to 1:78.125. A strong association was observed between IDH and HAM/TSP. The familial clustering of both diseases suggests a genetic background. Serological screening for HTLV-1 in children with symptoms of myelopathy is essential in areas where HTLV-1 is endemic. (authors)

HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) is a chronic myelopathy characterized by a slowly progressive spastic paraparesis and sphincter disturbances beginning in adulthood. Only eight well-documented cases occurring in childhood and adolescence have been described. Infective dermatitis associated to the HTLV-I (IDH) is a chronic eczema of childhood occurring in vertically infected carriers. Here we describe a 7-year-old boy with HAM/TSP and IDH. The neurological manifestations were spastic gait, hyperreflexia of lower limbs, clonus and bilateral Babinski's sign. High levels of HTLV-I antibodies in the serum and in the cerebrospinal fluid were observed. The association of these two diseases and the early onset of HAM/TSP are probably related to a strong humoral anti-HTLV-I response.

Psoriasis is a chronic systemic inflammatory disease mainly affecting the skin. Population-based surveys have shown a higher prevalence of non-alcoholic fat liver disease (NAFLD) in patients with psoriasis compared with the general population, especially in those with a greater psoriasis area and severity index (PASI). It is speculated that similar pathogenic bases may play a role in this association, highlighting insulin resistance and the release of inflammatory cytokines as the most likely causes. In the present work, we review basic aspects of the relationship between psoriasis and NAFLD.

Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Laboratório de Imunoregulação e Microbiologia. Salvador, BA, Brasil Universidade Federal da Bahia. Hospital Universitário Prof. Edgard Santos. Medicina Interna. Salvador, BA, Brasil Universidade Federal da Bahia. Hospital Universitário Prof. Edgard Santos. Medicina Interna. Salvador, BA, Brasil Katholieke Universiteit. Rega Institute. Clinical and Epidemiological Virology. Leuven, Belgium Fundação Oswaldo Cruz. Centro de Pesquisas Gonçalo Moniz. Laboratório de Imunoregulação e Microbiologia. Salvador, BA, Brasil Universidade Federal da Bahia. Hospital Universitário Prof. Edgard Santos. Patologia. Salvador, BA, Brasil We describe a patient with human T cell lymphotropic virus type 1 (HTLV-1)-associated infective dermatitis who developed HTLV-1-associated myelopathy/tropical spastic paraparesis and adult T cell leukemia/lymphoma at 16 years of age. Long inverse polymerase chain reaction was used to demonstrate monoclonal integration of proviral DNA in the lymphomatous skin lesion.

Background Infective dermatitis associated with HTLV-1 (IDH) is a recurrent and severe childhood infected form of eczema, that usually begins at 18 months in vertically-infected children. IDH may progress to adult T-cell leukemia/ lymphoma (ATL). Abnormal T cells (ably), including flower cells, were found in the peripheral blood smears of 30% (9/31) of IDH patients in Bahia, Brazil. Monoclonal integration of provirus assessed by inverted long PCR (ILPCR) was detected in 3 of these patients (2 with flower cells and one with ably cells but without flower cells in peripheral blood smear). The aim of this study was to identify the integration sites of the HTLV-1 provirus in host human genome in the IDH patients with monoclonal integration Materials and methods The sequences of the PCR products of these 3 patients obtained by ILPCR were mapped on to the human genome using the Basic Local Alignment Search Tool http://www.ncbi.nih.gov/blast.cgi. Results In the two patients with flower cells, integration sites were detected, respectively, in chromosome 1 (1p34.2) and chromosome 3 (3p11.1). In chromosome 1, the provirus was located within PPM1H gene coding region and in chromosome 3, within alpha satellite DNA sequences. The patient with integration in chromosome 1 also presents HAM/TSP. In the IDH patient without flower cells, integration site was identified at chromosome 12 (12q14) and was located near the transcriptional start site of GUCA2B gene. Discussion In IDH patients with monoclonal integration, the provirus integration sites can be associated with transcriptionally active regions of the human host genome.

Estudos recentes demonstram uma relação entre obesidade e inflamação crônica, confirmada através da associação de níveis elevados de fator de necrose tumoral alfa (TNF-±), interleucina seis (IL-6) e proteína C reativa, com aumento do índice de massa corporal (IMC). O estado inflamatório, nos indivíduos obesos, poderia contribuir para o desenvolvimento ou agravamento da psoríase. Fenômenos análogos já foram descritos, em outras doenças inflamatórias crônicas, como a artrite reumatóide e doença de Chrön. Estudos epidemiológicos mostram uma prevalência elevada de comorbidades cardiovasculares, secundárias às alterações metabólicas, associadas à psoríase e obesidade. Permanecem ainda não elucidados alguns aspectos desta associação, como: o impacto da obesidade (nas formas clínicas da dermatose, na associação com comorbidades e na resposta ao tratamento). Recent studies have found a relationship between obesity and chronic inflammation, confirmed by the association of high levels of tumor necrosis factor (TNF-_), interleukin six (IL-6,) and reactive C-protein with an increase in body mass index (BMI). In obese individuals, this inflammatory condition could contribute to the development or aggravation of psoriasis. Analogous phenomena have already been described in other inflammatory chronic diseases, such as rheumatoid arthritis and Crohn's disease. Epidemiological studies have identified a high prevalence of cardiovascular comorbidities, secondary to the metabolic alterations associated with psoriasis and obesity. A few aspects of this association remain unclear, such as the impact of obesity in the clinical forms of dermatoses, in the response to treatment, and its relationship with comorbidities.

BACKGROUND: Infective dermatitis associated with HTLV-1 (IDH) is a severe childhood form of eczema that may progress to adult T-cell leukemia/lymphoma (ATL). OBJECTIVE: In this study, the presence of clinical and laboratory parameters suggestive of ATL was evaluated in a cohort of 30 patients with IDH. STUDY DESIGN: Over a period of 33 months, the patients were submitted to three-monthly clinical evaluations, routine laboratory exams, full blood count and blood smears, and to six-monthly blood sampling for HTLV-1 proviral load determination. HTLV-1 proviral load was quantified using real-time TaqMan PCR assay. RESULTS: Abnormal cells (Ably) were found in the peripheral blood smears of nine patients (30%), flower cells being detected in five of these cases (16.6%). The presence of Ably and flower cells was not associated with a higher proviral load in those patients. CONCLUSIONS: This is the first report on the presence of flower cells in HTLV-1-infected children and adolescents. Furthermore, these cells have not previously been reported in IDH patients. The cases with flower cells probably represent precursory ATL cases, these patients being at a greater risk of developing ATL.

Background Instruments for field diagnosis of eczema are increasingly used, and it is essential to understand specific limitations to make best use of their strengths. Our objective was to assess the validity of ISAAC and UK Working Party criteria for field diagnosis of eczema in children. Methods We performed a cohort study in urban Brazil. Parents/guardians of 1,419 children answered ISAAC phase II questionnaire. Children were examined for skin lesions (UKWP protocol). Two dermatologists examined most cases of eczema (according to ISAAC or UKWP), and a sample without eczema. Results Agreement between repeat questionnaires on the filter question was poor (kappa = 0.4). Agreement between the 2 dermatologists was fair (kappa = 0.6). False positive reports included scabies in 39% of ISAAC cases and 33% of UKWP cases. Sensitivity and PPV were low (ISAAC: 37.1% and 16.1%; UKWP: 28.6% and 23.8%). Specificity and NPV were high (ISAAC: 90.0% and 96.6%; UKWP: 95.3% and 96.2%). One-year prevalence of eczema was 11.3% (ISAAC), 5.9% (UKWP) and 4.9% (adjusted dermatologist diagnosis). Point prevalence of scabies (alone or not) was 43%, 33% and 18%, in eczemas according to ISAAC, to UKWP and to dermatologists. The reasons why children with eczema were not identified by ISAAC or UKWP were wrongly denying dry skin, itchy rash or personal history of atopic diseases. A limitation is that questionnaire was already validated in Brazil, but not field tested in this specific setting. Conclusions Studies using UKWP or ISAAC criteria should include a validation arm, to contribute to the understanding of potential limitations of their use in different contexts and to explore solutions. We list specific recommendations.

Psoriasis is a chronic immune-mediated systemic disease that is influenced by genetic and environmental factors, is associated with comorbidities, and has a negative impact on the quality of life of affected individuals. The prevalence of psoriasis varies among different ethnic groups, but this topic has not been studied in Brazil to date. In this review, we evaluate the epidemiology and treatment of psoriasis from a Brazilian perspective. We focused on studies that involved Brazilian subjects. The prevalence of psoriasis in Brazil is estimated to be 2.5%, but no population study has been performed previously. Environmental factors, such as tropical climate, in association with genetic factors, such as miscegenation, may exert a beneficial impact on the course and frequency of psoriasis in Brazil. A number of studies have advanced our understanding of the cardiovascular, ophthalmic, and oral comorbidities that are associated with psoriasis. Concerns about biological therapy, such as endemic leprosy, human T-cell lymphotropic virus (HTLV), and tuberculosis infections, are discussed. The nonavailability of treatment options for psoriasis in the public health system contradicts the Brazilian Society of Dermatology guidelines, stimulating the judicialization of access to medicines in psoriasis care.

A participação do sistema de histocompatibilidade humano (HLA: human leukocyte antigens) na patogênese das doenças auto-imunes é bem conhecida. Situado no braço curto do cromossomo 6, o sistema HLA se destaca por seu polimorfismo e por sua capacidade de conferir susceptibilidade ou proteção a diferentes enfermidades. Em Dermatologia, esse sistema desempenha papel importante na patogenia e história natural de várias doenças. A força e o tipo de associação variam com a dermatose e, algumas vezes, com o grupo étnico-racial estudado. O surgimento de métodos moleculares para tipificação dos alelos HLA e as recentes atualizações de sua nomenclatura têm contribuído para o melhor entendimento desse sistema. Infelizmente, essas informações não têm sido veiculadas de maneira adequada na literatura clínica, o que dificulta o entendimento da associação do HLA com as doenças cutâneas. Nesta revisão, são discutidos alguns aspectos do sistema HLA, métodos de detecção, nomenclatura e sua associação com vitiligo, pênfigo, psoríase, lúpus eritematoso, escabiose, leishmaniose cutânea, hanseníase, paracoccidioidomicose e dermatite atópica. The participation of the human histocompatibility system (HLA: human leukocyte antigens) in the pathogenesis of autoimmune diseases is well known. Situated on the short arm of chromosome 6, the HLA system is very polymorphic and has the capacity to confer susceptibility or resistance to different diseases. In Dermatology, this system has an important participation in the pathogenesis and natural course of various diseases. The strength and type of association differ with conditions and sometimes with the ethnic-racial group studied. The discovery of molecular methods to typify HLA alleles and recent updates in its nomenclature has contributed to a better understanding of this system. Unfortunately, this information has not been adequately transmitted in the literature, hindering identification of the association of the HLA with skin diseases. In this review, some aspects of the HLA system are discussed, such as methods of detection, nomenclature and association with vitiligo, pemphigus, psoriasis, lupus erythematosus, scabies, cutaneous Leishmaniasis, leprosy, paracoccidiodomycosis and atopic dermatitis.

A dermatite infecciosa associada ao HTLV-I (DIH) é um tipo de eczema infectado e recidivante que incide em crianças que adquirem verticalmente a infecção pelo HTLV-I. Inicia-se após os 18 meses de idade. No entanto, existe relato recente de início na vida adulta. As lesões são eritemato-descamativas, infectadas e freqüentemente crostosas. Localizam-se, com maior freqüência, no couro cabeludo, regiões retroauriculares, pescoço e região inguinal, mas podem ser generalizadas. Vêem-se também físsuras retroauriculares, pápulas eritêmato-descamativas e foliculares. Prurido discreto a moderado, secreção nasal crônica e blefaroconjutivite podem ser observados. A DIH sempre se associa à infecção por Staphylococcus aureus e/ou Streptococcus beta hemoliticus. O diagnóstico diferencial clínico deve ser feito com as dermatites atópica e seborréica e, do ponto de vista anatomopatológico, com a psoríase e o linfoma cutâneo de células T. São discutidos os aspectos clínico-imunopatológicos, o diagnóstico diferencial e a evolução da DIH. Considerando a freqüência da DIH em Salvador, aconselha-se que seja feita sorologia para o HTLV-I em todos os casos de eczema severo em crianças e que essa possibilidade diagnóstica também seja considerada em adultos. Infectious dermatitis associated with HTLV-I (IDH) is a form of infected and recurrent dermatitis which affects children vertically infected with HTLV-I. IDH starts after 18 months of age. The lesions are erythematous-scaly and frequently crusty and are more frequently located on the scalp and on the retroauricular regions, but the lesions may be generalized. Pustules, erythematous-scaly and follicular papulae and retroauricular fissures may also be observed. Children present mild to moderate pruritus, lesions in the nostrils, and blepharoconjunctivitis. IDH is always associated with Staphylococcus aureus infection and/or Streptococcus beta haemolyticus infection. The differential diagnosis with atopic and seborrheic dermatitis can be made clinically. Histologically it is important to make a differential diagnosis with psoriasis and cutaneous T-cell lymphoma. We discuss here the clinical-immuno-pathological aspects, the differential diagnosis, and the evolution of IDH. Considering the frequency of IDH in Salvador, Bahia, we suggest that children with severe eczema be submitted to routine serology for HTLV-I and that IDH be included in the differential diagnosis of dermatitis in adulthood.

The treatment effect against psoriasis of an antigen (delipidated, deglycolipidated form of M. vaccae-PVAC) was investigated. One hundred and sixty-five patients were enrolled in three arms (50 or 15 microg or placebo), each receiving a total of two intradermal injections (days 0 and 21). At week 12, a 75% decrease in psoriasis area and severity index was similar among the studied groups (13, 9 and 18%, p=0.429). The overall incidence of adverse events was significantly higher in the PVAC treated groups when compared to placebo (98.2, 87.3 and 70.9%; p0.001) largely due to local reactions that were limited for the most part to grades 1 and 2 in severity and were self-limiting. Despite its overall safety, PVAC was not clearly indicated to be superior to placebo in the treatment of psoriasis in this study.

Infective dermatitis associated with human T cell lymphotropic virus type I (HTLV-I) infection is a chronic, relapsing eczema of childhood.Children, their mothers, and their siblings underwent serological testing for HTLV-I. Epidemiological data were collected from all seropositive children and their family members, and clinical and dermatological examinations were performed. Laboratory studies, including skin culture, and histopathological analyses were also performed. The diagnosis of infective dermatitis associated with HTLV-I (IDH) was made according to previously established criteria.All of the patients with cases that demonstrated clinical aspects of IDH were positive for HTLV-I. The median age of the children at the time of the first visit was 8.0 years (range, 2-14 years). The median duration of breastfeeding for 19 children was 22.5 months (range, 1-48 months). The lesions were erythematous, scaly, exudative, and crusted in all cases. The scalp, retroauricular areas, neck, and groin were the regions that were commonly affected. Cultures were positive for Staphylococcus aureus for 95% of the patients. The children were followed-up for a median of 3.0 years (range, 0.1-7 years), and 5 children developed HTLV-I-associated myelopathy/tropical spastic paraparesis. All of the children except 1 were treated with sulfamethoxazole-trimethoprim, and their lesions either improved greatly or completely disappeared.The present study demonstrates the severity of IDH in Bahia and confirms that its diagnosis is based almost exclusively on clinical aspects of the disease. Serological testing for HTLV-I and careful follow-up is recommended for all children with chronic, relapsing, severe eczema in regions where HTLV-I is endemic.