Treatment of keloids and hypertrophic scars. Position statement of the Brazilian expert group GREMCIQ.

Keloids (K) and hypertrophic scars (HS) are abnormal responses to wound healing that occur as the result of dermal inflammation. Despite the advances on their treatment, many patients still suffer from the negative effects of excessive scarring; its approach is impaired by the lack of objective data on different treatments and the large genetic variability among patients and the difficulties in producing multicentre studies. Their incidence among the Brazilian population is high, as the result of an admixture of Amerindians, Europeans and Africans ancestral roots. With the aim of producing multicentre studies on K and HS, a panel of senior Brazilian dermatologists focused on their treatment was invited to contribute with the K and HS Treatment Brazilian Guidelines. In the first part of this study, different treatment modalities for keloids and HS are fully reviewed by the panel. The second part of the study presents a consensus recommendation of treatment for different types of lesions. More than a literature review, this article aims to show the pitfalls and pearls of each therapeutic option, as well as a therapeutic approach by the Panel of Experts on keloids and Scars on a highly mixed population, providing simple guidelines.

Blefarocalasia: una presentación rara de cutis laxo / Blepharochalasis: A rare presentation of cutis laxa

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Acne in women.

This review focuses on the subject of acne in women, a disease that is increasingly common and that can also affect men. Adult acne differs from the type of acne that occurs in teenagers, and it may persist beyond adolescence or have its onset at an older age (adult-onset acne or late acne). Acne can have a negative impact on the quality of life of patients at any age, leading to a negative body image and decrease in self-esteem, and in older patients it can result in discrimination in the workplace and in other social environments. Acne in women must be understood as a specific problem, and here we discuss the pathogenesis, clinical presentation, psychology and treatment of this very prevalent problem.

An index for staging facial lipoatrophy and evaluation of the efficacy of the treatment with polymethylmethacrylate in HIV/AIDS patients: a pilot study.

BACKGROUND: Treatment of facial lipoatrophy of HIV/AIDS patients is mandatory by law in Brazil due to its negative impact on their quality of life. The index for facial lipoatrophy (ILA) is used as one of the inclusion criteria for patient treatment. OBJECTIVES: To define a correct diagnosis and staging of facial lipoatrophy, by employing the ILA. PATIENTS AND METHODS: This is an observational study of a series of case reports from patients submitted to facial lipoatrophy evaluation through ILA and treated with polymethylmethacrylate (PMMA) fillers. Facial lipoatrophy was classified in grades from I to IV, corresponding to mild, moderate, severe and very severe stage, according to ILA. Response to the treatment was defined as excellent (≥ 91%), good (71-90%), moderate (51-70%) and insufficient (≤ 50%). RESULTS: A total of 20 patients were included in this study: 18 men and two women. Median age was 49 years (35-61) and average ILA was 9.9 (7.2-16.8). Ten patients presented facial lipoatrophy grade II (moderate), 5 grade III (severe) and 5 grade IV (very severe). The average volume of PMMA used was 13 mL (5.5-22 mL). All patients showed good or excellent response, with a median of 86% (74-100%). The most typical adverse effect was local oedema but there were no late adverse effects. CONCLUSION: The ILA is an excellent method for evaluation of facial lipoatrophy and also for the assessment of the response to therapy. Facial filling with PMMA showed efficacy and safety in the treatment of facial lipoatrophy in HIV/AIDS patients.

Advances in tropical diseases.

There are six diseases that WHO considers as the major threat in developing countries, leprosy, filariasis, malaria, schistosomiasis, Chagas disease and leishmaniasis; and of these only malaria does not present skin lesions. These diseases are among the so called tropical diseases found in countries of tropical climate, usually infections and infestations considered exotic and rare in European and North American countries. It is extremely important for doctors of all countries to be able to provide correct pre travel counseling and to make early diagnosis and treatment, thus avoiding dissemination of these dieases to non endemic areas. The authors review some important tropical diseases seen in Brazil, as paracoccidiodomycosis, lobomycosis, myiasis, tungiasis, and cutaneous schistosomiasis and discuss new information about them.

Histogenesis of Abrikossoff tumour of the oral cavity.

BACKGROUND: Abrikossoff or granular cell tumour (GCT) is a relatively rare neoplasia, benign in most of the cases. It may occur in any part of the human body, but it has an oral location in 70% of the cases. Its origin has been discussed for decades, and it is not yet definitively determined. Immunohistochemical techniques suggest its origin in the Schwann cells, while more recent studies with new markers indicate an origin related to neuroendocrine cells. OBJECTIVE: Contribute to the clarification of histogenesis of oral Abrikossoff tumour studying immunohistochemical marking of 11 oral Brazilian cases. MATERIALS AND METHODS: Samples of tissues from the oral mucosa, tongue and lips placed in paraffin blocks, from eleven patients with a histopathological diagnosis of benign GCT were studied. Four different anti-serums (S-100, vimentin, PGP9.5 and ENE) were used for immunoperoxydase technique. RESULTS: A clear positivity for S-100 protein and vimentin was observed, with markers indicating origin from the Schwann cells. Less intense positivity was found in some cases, for ENE and PGP9.5, which suggests a neuroendocrine origin. CONCLUSIONS: The results obtained suggest an origin from Schwann cells, but also arise the possibility of neuroendocrine origin. New methods and more specific immunohistochemical markers are needed to elucidate the origin of the Abrikossoff tumour.

Immunophenotypic aspects of cylindroma and nodular hidradenoma.

BACKGROUND: Some adnexal tumours have many controversies about their histogenesis. OBJECTIVES: To evaluate the eccrine and/or apocrine differentiation phenotype in cases of cylindroma and clear cell hidradenoma with CD15 and p63 antibodies. METHODOLOGY: Slides and blocks of six cases of cylindroma and seven cases of nodular hidradenoma (clear cells) were analyzed by the technique of immunohistochemistry with CD15 and p63 antibodies. RESULTS: In all cases of cylindroma we obtained negative results for CD15 antibody and positive for p63 antibody. In five of seven cases of nodular hidradenoma (clear cell), we could easily observe clear cells between 20% and 50% of tumour cells. In the two other cases, cystic lesions were present and occasional clear cells could be seen. The reaction with CD15 antibody was positive in granular and cytoplasmic pattern in six of seven cases, especially in cells with suggestive clear cytoplasm in lower proportion than this clear cells could be seen in haematoxylin and eosin. The positivity for p63 antibody, nuclear pattern, was observed in six of seven cases, in the major part of tumour cells. In only one case, the positivity was in 20% of cells. Limitation Samples are in small number because these are relatively rare tumours. CONCLUSIONS: The present study suggests eccrine origin for both tumours: cylindroma and clear cell hidradenoma.

Lobomycosis. Literature Review and Future Perspectives / Revisión de la literatura y perspectivas futuras

Lobomycosis is a cutaneous infection of tropical and subtropical regions caused by the fungus Lacazia loboi, which still has a controversial taxonomical position. The first description of the disease and fungus was made in 1930 by Jorge Lobo.1t is a chronic disease with predominance of lesions similar to keloids, in exposed areas, limited to skin and semimucosa. There is no systemic involvement and patients maintain a good general health. Diagnosis is confirmed by direct or histopathologic exam and, until present, the fungus has not been cultivated. Surgery is the treatment of choice for isolated lesions, but there are frequent recurrences. Good therapeutic responses have been reported with clofazimine, with or without itraconazole, and with 5-fluorocytosine. This disease should be recognized by dermatologists worldwide because, although restricted to the Amazon region, it has been observed in other locations. Research development and achievement of new knowledge in molecular biology and genetic engineering of lobomycosis are of utmost importance because they may, in the future, lead to the culture of the fungus in the laboratory and to a better understanding of its pathogenesis, transmission mechanism, and new methods of diagnosis, prevention and treatment (AU)

La lobomicosis es una infección cutánea de las regiones tropicales y subtropicales que está causada por el hongo Lacazia loboi, y cuya posición taxonómica continúa siendo controvertida. Jorge Lobo realizó la primera descripción de la enfermedad y del hongo en 1930. Se trata de una enfermedad crónica con predominio de lesiones que se asemejan a queloides, en áreas fotoexpuestas, limitadas a la piel y a la semimucosa. No existe afectación sistémica y los pacientes presentan buen estado general. El diagnóstico se confirma por el examen directo o el estudio histológico y, hasta el momento, el hongo no se ha podido cultivar. La escisión quirúrgica es el tratamiento de elección para lesiones aisladas, pero las recurrencias son frecuentes. Se han descrito respuestas favorables con clofazimina, con o sin itraconazol, y con 5-fluorocitosina. Aunque es una enfermedad propia de la región del Amazonas, los dermatólogos de todo el mundo deben reconocerla, ya que también se ha observado en otras regiones. El desarrollo de la investigación y la consecución de nuevos conocimientos en biología molecular e ingeniería genética sobre la lobomicosis son de gran importancia ya que, en un futuro, permitirán el cultivo del hongo en el laboratorio, 10 que facilitará una mejor comprensión de su patogenia, mecanismo de transmisión y nuevos métodos de diagnóstico, prevención y tratamiento (AU)

Oral lichen planus and dermal dendrocytes.

INTRODUCTION: Oral lichen planus (OLP) is a relatively common inflammatory disease with a wide range of clinical forms. Its pathogenesis has not been fully elucidated although it is known to be mediated by lymphocytes with the participation of cytokines and other inflammatory cells, including type I and type II dermal dendrocytes (DD) (factor XIIIa+ DD and CD34+ DD, respectively). OBJECTIVES: To describe the presence and tissue distribution of these cells, through immunohistochemistry, in 23 specimens from patients with clinical and histopathological criteria of OLP. RESULTS: Factor XIIIa+ DD were mainly located in the superficial dermis (p < 0.0001) as opposed to the deep submucosa. These cells were abundant throughout the dermal-epidermal junction and closely related to lymphocyte infiltration. Moreover, factor XIIIa+ DD were also found in the epithelium and deep dermis. CD34+ DD were distributed mostly to the deep dermis directly below the lymphocyte infiltrate with few cells in the subepithelial region. CONCLUSIONS: DD were present in OLP, with distinct tissue distributions. Factor XIIIa+ DD were predominant in the superficial dermis while CD34+ DD could be found mostly in the deep dermis. These findings suggest that DD, and those positive for factor XIIIa+ in particular in view of their ability to express intercellular adhesion molecule-1 (ICAM-1) and tumor necrosis factor alpha (TNF-alpha), may play an important role in pathogenesis of OLP.

Liquen plano oral y dendrocitos dérmicos / Oral lichen Planus and Dermal Dendrocytes

Introducción: El liquen plano oral (LPO) es una enfermedad inflamatoria relativamente frecuente que se presenta con un amplio abanico de formas clínicas. Todavía no se ha determinado completamente su patogenia, aunque se sabe que los linfocitos actúan de mediadores con la participación de citoquinas y otras células inflamatorias, entre ellas los dendrocitos dérmicos (DD) tipo I y tipo II (DD positivos para el factor XIIIA y CD34, respectivamente).Objetivos. Describir la presencia y distribución de estas células en el tejido, mediante técnicas inmunohistoquímicas, en 23 muestras procedentes de pacientes que reunían los criterios clínicos e histopatológicos de LPO. Resultados: Los DD factor XIII+ estaban localizados principalmente en la dermis superficial (p < 0,0001) y no en la submucosa profunda. Dichas células se encontraban en abundancia en toda la unión dermoepidérmica y se relacionaban estrechamente con la infiltración linfocitaria. Los DD factor XIIIa+ se encontraban además en el epitelio y la dermis profunda. En cambio, los DD CD34+ se distribuyeron principalmente en la dermis profunda, directamente por debajo del infiltrado linfocitario, con pocas células en la zona subepitelial. Conclusiones: Los DD estaban presentes en el LPO, con diferentes distribuciones en los tejidos. Así, los DD factor XIIIa+ predominaban en la dermis superficial, mientras que los DD CD34+ se encontraban principalmente en la dermis profunda. Esto apunta a que los DD, y sobre todo los DD factor XIIIa+ debido a su capacidad para expresar moléculas de adhesión intercelulares-1 (ICAM-1) y el factor de necrosis tumoral alfa (TNF-α), pueden desempeñar una función destacada en la patogénesis del LPO (AU)

Introduction: Oral lichen planus (OLP) is a relatively common inflammatory disease with a wide range of clinical forms. Its pathogenesis has not been fully elucidated although it is known to be mediated by lymphocytes with the participation of cytokines and other inflammatory cells, including type I and type II dermal dendrocytes (DD) (factor xiIIa+ DD and CD34+ DD, respectively). Objectives: To describe the presence and tissue distribution of these cells, through immunohistochemistry, in 23 specimens from patients with clinical and histopathological criteria of OLP. Results: Factor xiIIa+ DD were mainly located in the superficial dermis (p < 0.0001) as opposed to the deep submucosa. These cells were abundant throughout the dermal-epidermal junction and closely related to lymphocyte infiltration. Moreover, factor xiIIa+ DD were also found in the epithelium and deep dermis. CD34+ DD were distributed mostly to the deep dermis directly below the lymphocyte infiltrate with few cells in the subepithelial region. Conclusions: DD were present in OLP, with distinct tissue distributions. Factor xiIIa+ DD were predominant in the superficial dermis while CD34+ DD could be found mostly in the deep dermis. These findings suggest that DD, and those positive for factor xiIIa+ in particular in view of their ability to express intercellular adhesion molecule-1 (ICAM-1) and tumor necrosis factor α (TNF-α), may play an important role in pathogenesis of OLP (AU)

Lobomycosis. Literature review and future perspectives.

Lobomycosis is a cutaneous infection of tropical and subtropical regions caused by the fungus Lacazia loboi, which still has a controversial taxonomical position. The first description of the disease and fungus was made in 1930 by Jorge Lobo. It is a chronic disease with predominance of lesions similar to keloids, in exposed areas, limited to skin and semimucosa. There is no systemic involvement and patients maintain a good general health. Diagnosis is confirmed by direct or histopathologic exam and, until present, the fungus has not been cultivated. Surgery is the treatment of choice for isolated lesions, but there are frequent recurrences. Good therapeutic responses have been reported with clofazimine, with or without itraconazole, and with 5-fluorocytosine. This disease should be recognized by dermatologists worldwide because, although restricted to the Amazon region, it has been observed in other locations. Research development and achievement of new knowledge in molecular biology and genetic engineering of lobomycosis are of utmost importance because they may, in the future, lead to the culture of the fungus in the laboratory and to a better understanding of its pathogenesis, transmission mechanism, and new methods of diagnosis, prevention and treatment.

Methotrexate and liver function: a study of 13 psoriasis cases treated with different cumulative dosages.

BACKGROUND: The need and frequency of hepatic biopsies during methotrexate (MTX) therapy are still controversial. OBJECTIVES: The purpose of this investigation is to assess MTX liver toxicity in patients with psoriasis through percutaneous liver biopsy, and compare liver morphology changes with increasing cumulative dosages (1, 2, 3 and 4 g) of MTX. RESULTS: Cumulative dosages of 1 to 2 g MTX did not cause significant liver toxicity. From a cumulative dosage of 3 to 4 g, there is fibrosis formation, inflammation enhancement in the portal area and fibrous septa, configuring regenerative nodes. CONCLUSION: In patients with no risk factors for liver disease, with normal physical examination and liver tests, biopsy can be done after a cumulative MTX dosage of approximately 1 to 1.5 g and repeated for each gram. In patients with risk factors, liver biopsy should be done before use of MTX, or within the first 2 months of treatment at the most, and repeated for each gram of cumulative dosage.

Pemphigus vulgaris: environmental factors. Occupational, behavioral, medical, and qualitative food frequency questionnaire.

BACKGROUND: There have been reports suggesting the involvement of environmental factors in the disease process of pemphigus. Factors suggested include exposure to pesticides or certain drugs. OBJECTIVE: To analyze the association of pemphigus with environmental exposure to various agents, including smoking, recreational and occupational insults, drugs, and food. DESIGN AND SETTING: In-person interviews of pemphigus patients and control subjects were conducted by trained medical investigators using a structured questionnaire. Questions included occupational, behavioral, medical, and qualitative food frequency details. The multicenter study was conducted at outpatient services of teaching hospitals in Bulgaria, Brazil, India, Israel, Italy, Spain, and the USA. PARTICIPANTS: A total of 126 pemphigus patients (55 men, 71 women; age, 54 +/- 17 years) and 173 healthy controls (87 men, 86 women; age 50 +/- 19 years) were interviewed in the period between October 1, 1999 and March 31, 2000. The diagnosis of pemphigus was based on clinical, histologic, immunohistologic, and immunohistochemical criteria. The disease duration was 2-27 years (8.4 +/- 7.2 years). Individuals with skin diseases other than pemphigus were selected as control subjects. MAIN OUTCOME MEASURE: Information on drugs, foods, and occupational, environmental, constitutional, and other possible risk factors was analyzed by t-tests and chi-squared tests as applicable. A multivariate logistic regression model was applied to the data to study simultaneously the independent relationship between each risk factor and pemphigus vulgaris. RESULTS: The risk for pemphigus vulgaris was lower for ex-smokers and current smokers than for patients who had never smoked. Exposure to pesticides and occupational exposure to metal vapor were associated with an increased risk of pemphigus. Pemphigus patients had more pregnancies than controls. There were differences in environmental factors between countries, with exposure to gardening materials and pesticides being highest among patients from Bulgaria, followed by Israel. Disease characteristics also exhibited differences between countries. Bulgarian patients less frequently had oral mucous membrane lesions: 66% compared to 92% for Israeli patients and 83% for Italians. The distribution of the disease in skin and mucous membranes was similar among patients from all countries. Exclusive skin involvement was seen in 50% of patients, mucous membranes alone in 23% of patients, and both skin and mucous membranes in 27% of patients. CONCLUSIONS: The beneficial effect of smoking on pemphigus might be explained by its effect on the immune system. In addition, smoking has an antiestrogenic effect, while pesticides have an estrogenic effect. The lower numbers of smokers among patients, the higher exposure rates to pesticides, and the higher number of female patients who had been pregnant may point to the contribution of estrogens to the disease process. It remains to be determined whether measures, such as avoiding exposure to pesticides or metal vapor, may be beneficial in the clinical context. As the present study was a survey, more definitive studies should be conducted to validate the results.

Leprosy. Recognition and treatment.

Leprosy is a slowly progressive, chronic infectious disease caused by the bacillus Mycobacterium leprae. It is a very serious, multilating and stigmatizing disease in many parts of the world and early diagnosis and therapy is the most important strategy for its control. The skin and peripheral nerves are the most affected organs. It is highly infective, but has low pathogenicity and low virulence with a long incubation period. The geographical distribution of leprosy has varied greatly with time and it is now endemic only in tropical and subtropical regions such as India and Brazil. The diagnosis of leprosy is made from the clinical picture, but must be complimented by skin bacilloscopy and histopathology. Leprosy has a number of distinct clinical presentations. Indeterminate leprosy is frequently the initial form consisting of a few lesions that either evolves into the other forms or resolves spontaneously. Lepromatous leprosy is the more contagious form and affects mainly the skin. In addition, some peripheral nerves may be thickened and other symptoms maybe present. The tuberculid form affects the skin and nerves, although usually there are few lesions. There is also a form borderline between the lepromatous and tuberculoid forms. Current treatment of leprosy involves use of 3 drugs: rifampicin (rifampin); clofazimine; and dapsone. Multidrug therapy aims to effectively eliminate M. leprae in the shortest possible time to prevent resistance from occurring. The duration of therapy was recently reduced from 24 to 12 months. Other treatment options are under evaluation in both preclinical and clinical trials and a number show promise. The combination of rifampicin, ofloxacin and minocycline given as a single dose has been recommended for the treatment of paucibacillar leprosy. Only when physicians, other health workers, and the population in endemic countries become fully aware of, and able to recognize, the disease in its initial phase, will it be possible for therapy to be instituted at the very beginning with either the standard scheme or the newer ones. Intervention at such an early stage will avoid the onset of the more serious signs and symptoms, meaning that leprosy will eventually become a less important public health problem. Therefore, efforts must be made to alert populations at risk and all health workers of the importance of an early diagnosis and treatment in leprosy infection.