Leprosy knowledge among primary care physicians in Southern Brazil: are we underdiagnosing?

BACKGROUND: Leprosy is a chronic infection with high morbidity in Brazil. Primary care physicians' lack of knowledge about the disease may play a significant role in underdiagnosis. This study aimed to assess primary care physicians' ability to identify typical leprosy skin lesions and their knowledge of the subject. METHODS: This cross-sectional study relied on a questionnaire in which participating doctors chose one main diagnostic hypothesis and two differential diagnoses for each skin lesion presented. Five leprosy lesions were included. Questions regarding management, follow-up, and diagnostic workup for the disease were also included. The questionnaire was sent to primary care physicians working in Curitiba, in the Southern Brazilian state of Paraná, and dermatologists, who constituted the control group. RESULTS: Thirty-two primary care physicians and 26 dermatologists agreed to participate in the study. Primary care physicians accurately identified a mean of 1.8 ± 1.2 of the five leprosy skin lesions, while dermatologists accurately identified 2.5 ± 0.9 (P = 0.009). The main misdiagnosed leprosy forms were the lepromatous and histoid variants. Among primary care physicians, 56.2% claimed to have little knowledge of the subject and a large share of participants was unaware of recent updates in treating paucibacillary forms, even within the dermatologist subgroup. CONCLUSIONS: Primary care physicians in Curitiba have little information regarding the diagnosis, treatment, and follow-up of leprosy. Even dermatologists had difficulties with treatment and patient management, emphasizing the constant need for education on this subject.

Ingenol mebutate treatment for actinic cheilitis: clinical, histopathological and p53 profile of 14 cases.

INTRODUCTION: Actinic cheilitis (AC) is part of a spectral disease of keratinocyte carcinomas considered by some authors an early stage of in situ squamous cell carcinoma. Treatment options for AC can be lesion and field-directed therapies. Ingenol mebutate (IM) induces rapid and direct cell death and immune responses being able to destruct dysplastic cells. MATERIALS AND METHODS: This study enrolled patients with AC to receive IM gel 0.015% for self-application on the lower lip for 3 consecutive days. A biopsy was performed before and after treatment for histopathological and immunohistochemical evaluation. Local skin reactions (LSR) were evaluated. The level of significance considered was 5%. RESULTS: Fourteen patients were enrolled. All LSR had a complete resolution for up to 2 weeks. The most common adverse events were burning sensation, angular cheilitis, and pain. There was an improvement of more than 80% in patients' subjective evaluation. There was no statistically significant histopathological response since all patients remained with mild dysplasia. No reduction in the P53 expression was observed in the current study. CONCLUSIONS: Despite being a safe therapeutic method, the absence of histopathological or immunohistochemical response suggests that clinical improvement may not be accompanied by histopathological cure for AC treated with IM.

Dermoscopy in the diagnosis of juvenile xanthogranuloma.

Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis. It manifests clinically as a red-yellow papule, usually showing spontaneous remission. The diagnosis is based on clinical and histological findings. We report the use of dermoscopy ("setting sun" pattern) as an adjuvant tool in the diagnosis of juvenile xanthogranuloma in a female patient presenting with a 2-month history of a pre-auricular papule.

Dermoscopy in the diagnosis of juvenile xanthogranuloma

Abstract: Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis. It manifests clinically as a red-yellow papule, usually showing spontaneous remission. The diagnosis is based on clinical and histological findings. We report the use of dermoscopy ("setting sun" pattern) as an adjuvant tool in the diagnosis of juvenile xanthogranuloma in a female patient presenting with a 2-month history of a pre-auricular papule.

Indication guidelines for Mohs micrographic surgery in skin tumors.

Mohs micrographic surgery is a technique used to excise skin tumors based on comprehensive surgical mapping, in which the surgeon removes the tumor, followed by a complete histological evaluation of the tumor's margins. The correlation of the presence of a tumor in histological examinations and its precise location on the surgical map result in a complete removal of the tumor with maximum normal tissue preservation. The present article seeks to provide general practitioners and healthcare specialists with guidelines regarding recommendations for Mohs micrographic surgery to treat skin tumors, based on the most reliable evidence available in medical literature on the subject. This bibliographic review of scientific articles in this line of research was conducted based on data collected from MEDLINE/PubMed. The search strategy used in this study was based on structured questions in the Patient, Intervention, Control, and Outcome (PICO) format. MeSH terms were used as descriptors. The indications of this technique are related to recurrence, histology, size, definition of tumor margins, and location of tumors. These guidelines attempt to establish the indications of Mohs surgery for different types of skin tumors.

Indication guidelines for Mohs micrographic surgery in skin tumors

Abstract: Mohs micrographic surgery is a technique used to excise skin tumors based on comprehensive surgical mapping, in which the surgeon removes the tumor, followed by a complete histological evaluation of the tumor's margins. The correlation of the presence of a tumor in histological examinations and its precise location on the surgical map result in a complete removal of the tumor with maximum normal tissue preservation. The present article seeks to provide general practitioners and healthcare specialists with guidelines regarding recommendations for Mohs micrographic surgery to treat skin tumors, based on the most reliable evidence available in medical literature on the subject. This bibliographic review of scientific articles in this line of research was conducted based on data collected from MEDLINE/PubMed. The search strategy used in this study was based on structured questions in the Patient, Intervention, Control, and Outcome (PICO) format. MeSH terms were used as descriptors. The indications of this technique are related to recurrence, histology, size, definition of tumor margins, and location of tumors. These guidelines attempt to establish the indications of Mohs surgery for different types of skin tumors.

Polymorphism of the E-cadherin gene CDH1 is associated with susceptibility to vitiligo.

Vitiligo is a depigmenting disorder characterized by loss of functional melanocytes from the epidermis. Experimental data suggest that defective melanocyte adhesion may underlie the pathogenesis of the disease. In particular, association between vitiligo and genetic variants of the DDR1 gene involved in melanocyte adhesion has been recently published. A subsequent, independent study revealed lower expression of DDR1 in vitiligo lesions. Here, we expand this investigation by testing for association between vitiligo and polymorphisms of CDH1, IL1B and NOV (formerly CCN3), genes belonging to the DDR1 adhesion pathway, in two population samples of distinct design. Our results reveal that alleles of marker rs10431924 of the CDH1 gene are associated with vitiligo, especially in the presence of autoimmune comorbidities.

Vitiligo--Part 2--classification, histopathology and treatment.

In an unprecedented effort in the field of vitiligo, a global consensus resulted on a suggested new classification protocol for the disease. The main histopathological finding in vitiligo is the total absence of functioning melanocytes in the lesions, while the inflammatory cells most commonly found on the edges of the lesions are CD4+ and CD8+ T lymphocytes. Physical and pharmacological treatment strategies aim to control the autoimmune damage and stimulate melanocyte migration from the unaffected edges of lesions and the outer hair follicle root sheath to the affected skin; moreover, surgical treatments can be combined with topical and physical treatments.

Vitiligo - Part 2 - classification, histopathology and treatment

In an unprecedented effort in the field of vitiligo, a global consensus resulted on a suggested new classification protocol for the disease. The main histopathological finding in vitiligo is the total absence of functioning melanocytes in the lesions, while the inflammatory cells most commonly found on the edges of the lesions are CD4+ and CD8+ T lymphocytes. Physical and pharmacological treatment strategies aim to control the autoimmune damage and stimulate melanocyte migration from the unaffected edges of lesions and the outer hair follicle root sheath to the affected skin; moreover, surgical treatments can be combined with topical and physical treatments.

Vitiligo--part 1.

Vitiligo is a chronic stigmatizing disease, already known for millennia, which mainly affects melanocytes from epidermis basal layer, leading to the development of hypochromic and achromic patches. Its estimated prevalence is 0.5% worldwide. The involvement of genetic factors controlling susceptibility to vitiligo has been studied over the last decades, and results of previous studies present vitiligo as a complex, multifactorial and polygenic disease. In this context, a few genes, including DDR1, XBP1 and NLRP1 have been consistently and functionally associated with the disease. Notwithstanding, environmental factors that precipitate or maintain the disease are yet to be described. The pathogenesis of vitiligo has not been totally clarified until now and many theories have been proposed. Of these, the autoimmune hypothesis is now the most cited and studied among experts. Dysfunction in metabolic pathways, which could lead to production of toxic metabolites causing damage to melanocytes, has also been investigated. Melanocytes adhesion deficit in patients with vitiligo is mainly speculated by the appearance of Köebner phenomenon, recently, new genes and proteins involved in this deficit have been found.

Vitiligo - Part 1

Vitiligo is a chronic stigmatizing disease, already known for millennia, which mainly affects melanocytes from epidermis basal layer, leading to the development of hypochromic and achromic patches. Its estimated prevalence is 0.5% worldwide. The involvement of genetic factors controlling susceptibility to vitiligo has been studied over the last decades, and results of previous studies present vitiligo as a complex, multifactorial and polygenic disease. In this context, a few genes, including DDR1, XBP1 and NLRP1 have been consistently and functionally associated with the disease. Notwithstanding, environmental factors that precipitate or maintain the disease are yet to be described. The pathogenesis of vitiligo has not been totally clarified until now and many theories have been proposed. Of these, the autoimmune hypothesis is now the most cited and studied among experts. Dysfunction in metabolic pathways, which could lead to production of toxic metabolites causing damage to melanocytes, has also been investigated. Melanocytes adhesion deficit in patients with vitiligo is mainly speculated by the appearance of Köebner phenomenon, recently, new genes and proteins involved in this deficit have been found.

Reativação de tuberculose no tratamento com imunobiológicos / Reactivation of tuberculosis in treatment with immunobiological

O uso de agentes biológicos vem se mostrando uma boa opção no tratamento da psoríase de difícil controle. Os inibidores do fator de necrose tumoral alfa (TNF-alfa) demonstraram resultados positivos tanto em índices de resposta terapêutica quanto em velocidade de início de ação. No entanto, pelo fato de o TNF-alfa ter uma importante participação na formação do granuloma e, consequentemente na defesa contra o Mycobacterium tuberculosis, tal tratamento pode resultar na reativação de doença latente. Assim sendo, o screening para tuberculose é necessário antes e durante o uso destas drogas na prática clínica.

The use of biologics agents has been a good option in the trteatment of resistant psoriasis. The tumor necrosis factor-alpha (TNF-alpha) blockers demonstrated positives results, both in efficacy and onset of action. However TNF-alpha plays an important role in host defense against tuberculosis, this treatment can result in reactivation of latent disease. Thus, screening for tuberculosis is necessary before and during the use of these drugs in clinical practice.

Genetic risk factors for human susceptibility to infections of relevance in dermatology.

BACKGROUND: In the pre-microbiological era, it was widely accepted that diseases, today known to be infectious, were hereditary. With the discovery of microorganisms and their role in the pathogenesis of several diseases, it was suggested that exposure to the pathogen was enough to explain infection. Nowadays, it is clear that infection is the result of a complex interplay between pathogen and host, therefore dependant on the genetic make-up of the two organisms. Dermatology offers several examples of infectious diseases in different stages of understanding of their molecular basis. In this review, we summarize the main advances towards dissecting the genetic component controlling human susceptibility to infectious diseases of interest in dermatology. Widely investigated diseases such as leprosy and leishmaniasis are discussed from the genetic perspective of both host and pathogen. Others, such as rare mycobacterioses, fungal infections and syphilis, are presented as good opportunities for research in the field of genetics of infection.

Genetic risk factors for human susceptibility to infections of relevance in dermatology / Fatores de risco genético para a suscetibilidade humana à infecções de relevância em dermatologia

BACKGROUND: In the pre-microbiological era, it was widely accepted that diseases, today known to be infectious, were hereditary. With the discovery of microorganisms and their role in the pathogenesis of several diseases, it was suggested that exposure to the pathogen was enough to explain infection. Nowadays, it is clear that infection is the result of a complex interplay between pathogen and host, therefore dependant on the genetic make-up of the two organisms. Dermatology offers several examples of infectious diseases in different stages of understanding of their molecular basis. In this review, we summarize the main advances towards dissecting the genetic component controlling human susceptibility to infectious diseases of interest in dermatology. Widely investigated diseases such as leprosy and leishmaniasis are discussed from the genetic perspective of both host and pathogen. Others, such as rare mycobacterioses, fungal infections and syphilis, are presented as good opportunities for research in the field of genetics of infection.

INTRODUÇÃO: Durante a era pré-microbiológica, era comum a visão de que doenças, hoje sabidamente infecciosas, eram hereditárias. Com a descoberta dos microorganismos e seu papel na patogênese de diversas patologias, chegou-se a propor que a exposição ao patógeno era condição suficiente para explicar infecção. Hoje, está claro que infecção é o resultado de uma complexa interação entre patógeno e hospedeiro, dependendo portanto, em última análise, do make-up genético de ambos os organismos. A dermatologia oferece diversos exemplos de doenças infecciosas em diferentes graus de entendimento de suas bases moleculares. Nesta revisão, resumimos os principais avanços na direção da dissecção do componente genético controlando suscetibilidade do ser humano a doenças infecciosas de importância na dermatologia. Doenças amplamente estudadas, como a hanseníase e a leishmaniose, são discutidas sob o ponto de vista da genética tanto do hospedeiro quanto do patógeno. Outras, como micobacterioses raras, micoses e sífilis, são apresentadas como boas oportunidades para pesquisa na área de genética de infecção.

Topography of basal cell carcinoma and their correlations with gender, age and histologic pattern: a retrospective study of 1042 lesions.

BACKGROUND: Basal cell carcinoma accounts for 75% of skin cancer. Sun exposure and genetics are related to its etiology. It's expected that biological and behavioral differences provide different patterns of involvement between sexes. OBJECTIVES: To evaluate the topography of lesions and their correlations with gender, age and histological type. METHODS: Retrospective study of basal cell carcinoma patients treated between 1999 and 2008 in the Skin Cancer Clinic of Santa Casa de Misericordia of Curitiba. We evaluated sex, age, location, histological type, margins commitment, sun exposure and family skin cancer history. RESULTS: We found 1042 lesions in 545 patients (61% women), being more numerous in men (p<0.01). Their ages ranged between 27 and 95 years (median=65). Men had more sun exposure (p<0.01). The lesions were more frequent extra-cephalic recently (p<0.01). The margin involvement was higher in the head (p<0.01). The superficial type was less frequent on the head (p<0.01) and was associated with younger ages in women (p<0.01). The head housed 74% of lesions and the legs 2%. Women had a predilection for the legs, nose and upper lip and men to trunk, ears and scalp (p <0.05). The surgeries in the medial epicanthus and scalp occurred at younger ages (p=0.01). CONCLUSIONS: We identified significant associations between the topography of lesions, gender, age and histological type, demonstrating the possible pathophysiological diversity and differential risk factors operation. In the period studied we found no trend of increase in the proportion of young or women among patients.