Monkeypox: What the Dermatologist Needs to Know.

Monkeypox, an endemic disease in some African countries, has provoked public health activeness on a global scale that even the World Health Organization (WHO), invoking international health regulations, declared it a public health emergency of international concern (PHEIC). The WHO called attention of member states to exert maximum surveillance over the disease, its patients, and contact persons in order to standardize control measures. A need was directed to provide complete knowledge about the disease, allowing the administration of prior diagnoses as well as isolation and more effective epidemiologic control measures. An updated review on monkeypox emphasized upon having the fundamental aspects of the lesions caused by the disease, and appropriate management of patients upon clinical and epidemiologic evaluation.

Heparin-Induced Bullous Hemorrhagic Dermatosis: A Report of an Exceptionally Exuberant Case and Literature Review.

INTRODUCTION: Heparin-induced bullous hemorrhagic dermatosis (HBHD) is an unusual cutaneous adverse reaction to heparin characterized by acute onset of hemorrhagic bullae. METHODOLOGY: An exuberant case of HBHD is reported, and a revision of previously reported cases indexed on PubMed is provided. Cases were tabulated to the following parameters: sex, age, type of heparin and route of administration, associated medical procedure, number of days between heparin start and onset of lesions, site of the lesions, level of blister on histopathology, inflammatory infiltrate on histopathology, direct immunofluorescence findings, heparin-related intervention, number of days to resolution of skin lesions, associated hemorrhagic event, and outcome. CASE REPORT: A 21-year-old woman with end-stage renal disease was receiving intravenous unfractioned heparin (UFH) during hemodialysis section for the past 3 months. Four hours after using for the first time an arteriovenous fistula punctured on her right wrist, the patient noticed the onset of vesicles and blisters on the right forearm containing citrus or serohemorrhagic exudate, which became overt hemorrhagic in 24 hours. Histopathology depicted a nonacantholytic subcorneal blister containing erythrocytes and plasma without any significant dermal inflammatory infiltrate. RESULTS: Sixty cases of HBHD were included. HBHD affected predominantly men, with a male/female rate of 2.75. The age range was from 21 to 94 years, with an average of 70.8 and a median of 72 years. Nine patients used UFH, and 54 patients used low molecular weight heparins (3 patients used both). The lapse of time between the start of heparin and the onset of skin lesion varied from 6 hours to 240 days, with an average of 17.3 days and a median of 7 days. Limbs were affected in most of the cases. The level of the blister was subcorneal in 10 patients, intraepidermal in 30, subepidermal in 8, and both intraepidermal and subepidermal in 1. In 33 cases, there was no significant dermal inflammatory infiltrate. Pure lymphocytic inflammatory infiltrate was present in 10 cases. Eosinophils were found within the dermal inflammatory infiltrate in 2 cases, neutrophils in 2, and a mixture of eosinophils and neutrophils in other 2. Direct immunofluorescence was performed in 18 cases, all of them with negative results. DISCUSSION: We hypothesize that mechanical trauma, skin fragility, and the anticoagulation effect of heparin might contribute concomitantly to the development of the lesions and speculate that subepidermal blisters in HBHD could be formed by rupture of the floor of a former intraepidermal blister.

Adverse effects of polychemotherapy for leprosy in 13 years of follow-up at a university hospital

Abstract Leprosy is one of the neglected diseases in the world and Brazil is the second country with more cases. A retrospective study was conducted based on the medical records of 196 leprosy patients diagnosed during the course of 13 years at a university hospital. The aim was to describe the adverse effects of polychemotherapy, as well the most prevalent and most vulnerable populations. In the study, dapsone was the most implicated drug, especially in women, and the risk increased with age. The authors conclude that with this patient profile, greater vigilance should be taken regarding possible adverse effects, especially anemia.

Adverse effects of polychemotherapy for leprosy in 13 years of follow-up at a university hospital.

Leprosy is one of the neglected diseases in the world and Brazil is the second country with more cases. A retrospective study was conducted based on the medical records of 196 leprosy patients diagnosed during the course of 13 years at a university hospital. The aim was to describe the adverse effects of polychemotherapy, as well the most prevalent and most vulnerable populations. In the study, dapsone was the most implicated drug, especially in women, and the risk increased with age. The authors conclude that with this patient profile, greater vigilance should be taken regarding possible adverse effects, especially anemia.

The Importance of the Biopsy Technique in the Diagnosis of Histoid Leprosy.

Histoid leprosy (HL) was originally described by Wade in 1963 and is regarded as a rare variant of lepromatous leprosy (LL). These characteristic clinical lesions are firm, deeply adhered nodules with features reminiscent of dermatofibromas or keloids in a background of apparently healthy skin. The main histopathological findings described are the presence of spindle cell histiocytes immersed in a richly collagenized background, usually forming a nodular pattern of infiltration with sharply delimitation and positive staining for acid-fast bacilli. The classical form of HL lesions should be devoid of foam histiocytes and globi. However, we and other authors noticed that in most of the cases, despite characteristic clinical features, histopathology depicts a mixture of LL and HL patterns. Therefore, we present a case with clinical features similar to HL in which an excisional scalpel biopsy of a nodule demonstrated features of classical LL in the center of the lesion and features of HL in the periphery, highlighting that a proper biopsy technique could enhance the ability of the dermatopathologist to histopathologically diagnose cases of HL. In cases in which HL is clinically suspected, we advocate replacing the usual 4-mm incisional punch biopsy by a broader elliptical scalpel biopsy, encompassing the totality of the lesion whenever possible to achieve a reliable representation of the pathologic process.

Erythema Nodosum Leprosum: Update and challenges on the treatment of a neglected condition.

Erythema Nodosum Leprosum (ENL) occurs due to the immunological complication of multibacillary leprosy and is characterized by painful nodules and systemic compromising. It is usually recurrent and/or chronic and has both physical and economic impact on the patient, being a very important cause of disability. In addition, ENL is a major health problem in countries where leprosy is endemic. Therefore, adequate control of this condition is important. The management of ENL aims to control acute inflammation and neuritis and prevent the onset of new episodes. However, all currently available treatment modalities have one or two drawbacks and are not effective for all patients. Corticosteroid is the anti-inflammatory of choice in ENL but may cause dependence, especially for chronic patients. Thalidomide has a rapid action but its use is limited due the teratogenicity and neurotoxicity. Clofazimine and pentoxifylline have slow action and have important adverse effects. Finally, there is no pattern or guidelines for treating these patients, becoming more difficult to evaluate and to control this condition. This review aims to show the main drugs used in the treatment of ENL and the challenges in the management of the reaction.

Modified simple decompression in the treatment of cubital tunnel syndrome: avoiding ulnar nerve subluxation.

OBJECTIVE: In this study, we propose a modification to the simple decompression technique that contains the ulnar nerve in the cubital fossa, thus preventing subluxation during forearm flexion movements. METHODS: Five consecutive patients with leprosy-associated cubital tunnel syndrome underwent surgery with the modified technique between July 2011 and October 2012. RESULTS: The most common symptoms were neuropathic pain and sensory changes (both 60%). On the McGowan scale, three patients maintained their preoperative score and two patients improved by two points, while on the Louisiana State University Health Sciences Center scale, two patients maintained the same scores, two improved by two points, and one improved by one point. Four patients were able to discontinue corticosteroid use. The mean follow-up time was 25.6 months (range 2-48 months). There were no recurrences or subluxations in the long-term. CONCLUSION: This alternative technique resulted in excellent functional results, as well as successful withdrawal from corticosteroids. Furthermore, it resulted in no ulnar nerve subluxations.

Modified simple decompression in the treatment of cubital tunnel syndrome: avoiding ulnar nerve subluxation / Descompressão simples modificada no tratamento da síndrome do túnel cubital: evitando subluxação do nervo ulnar

ABSTRACT Objective In this study, we propose a modification to the simple decompression technique that contains the ulnar nerve in the cubital fossa, thus preventing subluxation during forearm flexion movements. Methods Five consecutive patients with leprosy-associated cubital tunnel syndrome underwent surgery with the modified technique between July 2011 and October 2012. Results The most common symptoms were neuropathic pain and sensory changes (both 60%). On the McGowan scale, three patients maintained their preoperative score and two patients improved by two points, while on the Louisiana State University Health Sciences Center scale, two patients maintained the same scores, two improved by two points, and one improved by one point. Four patients were able to discontinue corticosteroid use. The mean follow-up time was 25.6 months (range 2-48 months). There were no recurrences or subluxations in the long-term. Conclusion This alternative technique resulted in excellent functional results, as well as successful withdrawal from corticosteroids. Furthermore, it resulted in no ulnar nerve subluxations.

RESUMO Objetivo Neste manuscrito apresentamos uma modificação da técnica de descompressão simples do nervo ulnar no túnel cubital que impede a subluxação do nervo em movimentos de flexão do antebraço. Métodos Foram incluídos cinco pacientes consecutivos acometidos por síndrome do túnel cubital (Hanseníase) submetidos à cirurgia entre 2011 e 2012. Resultados Os sintomas mais comuns foram dor neuropática e alterações sensitivas (60%). No pós-operatório, três pacientes mantiveram o mesmo escore e dois melhoraram dois pontos na escala de McGowan, enquanto na escala Louisiana State University Health Sciences Center, dois pacientes mantiveram o mesmo escore, dois melhoraram dois pontos e um melhorou um ponto. Os corticosteróides foram descontinuados em quatro pacientes. O tempo médio de seguimento foi 25,6 meses (variação 2-48 meses). Não foram observadas recorrência ou subluxação no longo prazo. Conclusões A técnica alternativa apresentou excelentes resultados funcionais e foi bem sucedida na retirada dos corticosteróides. Ademais, subluxações do nervo ulnar não foram observadas.

Type 2 leprosy reaction with Sweet's syndrome-like presentation.

Leprosy is a chronic disease characterized by manifestations in the peripheral nerves and skin. The course of the disease may be interrupted by acute phenomena called reactions. This article reports a peculiar case of type 2 leprosy reaction with Sweet's syndrome-like features as the first clinical manifestation of leprosy, resulting in a delay in the diagnosis due to unusual clinical presentation. The patient had clinical and histopathological features reminiscent of Sweet's syndrome associated with clusters of vacuolated histiocytes containing acid-fast bacilli isolated or forming globi. Herein, it is discussed how to recognize type 2 leprosy reaction with Sweet's syndrome features, the differential diagnosis with type 1 leprosy reaction and the treatment options. When this kind of reaction is the first clinical presentation of leprosy, the correct diagnosis might be not suspected clinically, and established only with histopathologic evaluation.

Type 2 leprosy reaction with Sweet's syndrome-like presentation

Abstract Leprosy is a chronic disease characterized by manifestations in the peripheral nerves and skin. The course of the disease may be interrupted by acute phenomena called reactions. This article reports a peculiar case of type 2 leprosy reaction with Sweet's syndrome-like features as the first clinical manifestation of leprosy, resulting in a delay in the diagnosis due to unusual clinical presentation. The patient had clinical and histopathological features reminiscent of Sweet's syndrome associated with clusters of vacuolated histiocytes containing acid-fast bacilli isolated or forming globi. Herein, it is discussed how to recognize type 2 leprosy reaction with Sweet's syndrome features, the differential diagnosis with type 1 leprosy reaction and the treatment options. When this kind of reaction is the first clinical presentation of leprosy, the correct diagnosis might be not suspected clinically, and established only with histopathologic evaluation.

Cutaneous involvement by cytomegalovirus in a renal transplant recipient as an indicator of severe systemic infection.

Cytomegalovirus is an opportunistic virus that commonly affects immunosuppressed patients. Cutaneous involvement by this virus is rare and occurs in significantly immunocompromised hosts, with a poor prognosis. Skin ulcers may represent the first sign of systemic infection by cytomegalovirus in these patients. Herein, a case of a systemic infection by Cytomegalovirus presenting as genital and oral ulcers in a kidney-transplant recipient is reported.

Cutaneous involvement by cytomegalovirus in a renal transplant recipient as an indicator of severe systemic infection

Abstract Cytomegalovirus is an opportunistic virus that commonly affects immunosuppressed patients. Cutaneous involvement by this virus is rare and occurs in significantly immunocompromised hosts, with a poor prognosis. Skin ulcers may represent the first sign of systemic infection by cytomegalovirus in these patients. Herein, a case of a systemic infection by Cytomegalovirus presenting as genital and oral ulcers in a kidney-transplant recipient is reported.

Do you know this syndrome? / Voce conhece esta sindrome?

Berardinelli-Seip syndrome is a rare autosomal recessive disease characterized by inadequate metabolism and inefficient storing of lipids in fat cells, generating accumulation of fat in organs such as the liver, spleen, pancreas, heart, arterial endothelium and skin. Classically, patients manifest generalized lipoatrophy at birth or until 2 years of age, and in adolescence usually develop marked insulin resistance with rapid progression to diabetes and dyslipidemia. We report the case of a 17-year-old Berardinelli-Seip syndrome patient with eruptive xanthoma associated with severe hypertriglyceridemia. It is worth noting Eruptive xanthoma as a dermatological manifestation that is not generally highlighted in the reports of cases of this genetic metabolic disorder.

Síndrome de Berardinelli-Seip é doença genética autossômica recessiva rara, caracterizada por ineficiência em metabolizar e estocar material lipídico adequadamente nos adipócitos, gerando acúmulo de gordura em órgãos não habituais, como fígado, baço, pâncreas, coração, endotélio arterial e pele. Classicamente, os portadores nascem ou manifestam lipoatrofia generalizada até os 2 anos e, geralmente na adolescência, desenvolvem marcada resistência insulínica com rápida progressão para diabetes e dislipidemia. Relatamos um caso de portadora da síndrome de Berardinelli-Seip, de 17 anos, com xantoma eruptivo associado à hipertrigliceridemia grave. Ressalta-se o xantoma eruptivo como manifestação dermatológica não enfatizada nos casos relatados sobre esse distúrbio metabólico genético.

Educação em saúde e busca ativa de casos de hanseníase em uma escola pública em Ananindeua, Pará, Brasil / Health education and active search for cases of leprosy in a public school in Ananindeua, Pará, Brazil / Educación para la salud y búsqueda activa de casos de lepra en una escuela pública en Ananindeua, Pará, Brasil

Objetivo: Descrever ação para o controle da hanseníase por meio da educação em saúde e da busca ativa de casos na faixa etária escolar, em uma instituição pública de ensino do município de Ananindeua, Pará. Métodos: Trata-se de estudo descritivo, conduzido em uma instituição pública de ensino, incluindo estudantes do ensino fundamental. Em outubro e novembro de 2010 foram realizadas palestras na escola sobre hanseníase e outras dermatoses e, em seguida, foi feita uma triagem com os alunos que apresentaram alguma mancha na pele. No total de 532 alunos envolvidos, foram identificados 55 alunos como casos suspeitos para hanseníase. Esses alunos foram conduzidos para consulta dermatológica, sendo realizados o exame clínico e a aplicação de questionários sociodemográficos. Resultados: A face (49%) e os membros superiores (45%) foram as principais regiões do corpo com lesões. Na maioria dos casos investigados tratava-se de pitiríase alba (42%) e pitiríase versicolor (31%). Entretanto, diagnosticou-se um caso de hanseníase (2%), na forma clínica tuberculóide. Conclusão: Considerando a abrangência do estudo, o número de casos de hanseníase encontrado é significativo e reafirma a alta taxa de detecção de hanseníase em menores de 15 anos no município. Ações de vigilância epidemiológica devem ser intensificadas, no sentido de detectar a doença mais precocemente, contribuindo para a redução de estigmas e da restrição à participação social.

Objectives: To describe action for leprosy control through health education and case active search among school age children in a public school in the municipality of Ananindeua, Pará state. Methods: This is a descriptive study conducted in a public school including elementary school students. In October and November 2010, lectures on leprosy and other skin lesions were given in this school and then screening was performed with students that presented skin spots. From the total of 532 students involved, 55 were identified as suspected to leprosy. These students were taken to dermatological appointment including clinical examination and application of socio-demographic questionnaire. Results: The face (49%) and upper limbs (45%) were the main body areas presenting lesions. In most of the cases investigated, pityriasis alba (42%) and pityriasis versicolor (31%) were diagnosed. However, one case of leprosy (2%) was also found, in tuberculoid clinical form. Conclusion: Considering the scope of the study, the number of leprosy cases found is significant and confirms the high detection rate of leprosy in children under age 15 in the city. Epidemiological surveillance actions should be intensified in order to detect the disease in its early stage, contributing to enhancement in social participation by reducing the disease-related stigma.

Objetivo: Describir la acción para el control de la lepra mediante la Educación para la Salud y la búsqueda activa de casos en niños en edad escolar en una escuela pública en la ciudad de Ananindeua, Pará. Métodos: Se trata de un estudio descriptivo, realizado en una escuela pública, con los estudiantes de primaria. En octubre y noviembre de 2010 se realizaron conferencias sobre lepra y otras enfermedades de la piel y luego se examinó a los estudiantes que tenían alguna mancha en la piel. Del total de 532 alumnos, 55 estudiantes fueron identificados como sospechosos de tener lepra. Esos estudiantes fueron derivados a consulta dermatológica, examen clínico y aplicación de cuestionarios socio-demográficos. Resultados: La cara (49%) y las extremidades superiores (45%) fueron las principales áreas del cuerpo con lesiones. En la mayoría de los casos se constató la pitiriasis alba (42%) y pitiriasis versicolor (31%). Sin embargo, se diagnosticó un caso de lepra (2%), en la forma clínica tuberculoide. Conclusión: Teniendo en cuenta el alcance del estudio, el número de casos de lepra encontrado es significativo y reafirma la alta tasa de detección de lepra en los niños menores de 15 años en la ciudad. Las acciones de vigilancia epidemiológica deben ser intensificadas, con el fin de detectar precozmente la enfermad, contribuyendo para la reducción del estigma y de la restricción de la participación social.

Do you know this syndrome? Berardinelli-Seip syndrome.

Berardinelli-Seip syndrome is a rare autosomal recessive disease characterized by inadequate metabolism and inefficient storing of lipids in fat cells, generating accumulation of fat in organs such as the liver, spleen, pancreas, heart, arterial endothelium and skin. Classically, patients manifest generalized lipoatrophy at birth or until 2 years of age, and in adolescence usually develop marked insulin resistance with rapid progression to diabetes and dyslipidemia. We report the case of a 17-year-old Berardinelli-Seip syndrome patient with eruptive xanthoma associated with severe hypertriglyceridemia. It is worth noting Eruptive xanthoma as a dermatological manifestation that is not generally highlighted in the reports of cases of this genetic metabolic disorder.

Hanseníase em menores de 15 anos: a importância do exame de contato / Leprosy in children under 15 years: the importance of early diagnosis / Enfermedad de Hansen en menores de 15 años: la importancia del examen de contacto

Descrever dois casos de hanseníase em menores de 15 anos, sendo um de paciente com 18 meses de idade e outro de 13 anos, diagnosticados por modos de detecção diferentes, ressaltando a importância de examinar os contatos. DESCRIÇÃO DO CASO: Um dos casos foi diagnosticado precocemente por meio do exame de contatos intradomiciliares, enquanto o outro foi diagnosticado por demanda espontânea após quatro anos de aparecimento das lesões e, apesar de ser contato de um ex-paciente, não foi examinado na época. COMENTÁRIOS: Em países endêmicos, a alta detecção da hanseníase em menores de 15 anos revela a persistência na transmissão do bacilo e as dificuldades dos programas de saúde para o controle da doença. O maior tempo para diagnóstico ocasiona sequelas e deformidades e, dessa forma, a busca dos contatos constitui importante método para o diagnóstico precoce da doença na infância, quando os sinais clínicos nem sempre são fáceis de serem identificados e há grande diversidade de formas clínicas em que a doença pode se apresentar.

To describe two cases of leprosy in children under 15 years old, being one patient aged with 18 months and other 13 years, diagnosed by different modes of detection, emphasizing the importance of examining the contacts. CASE DESCRIPTION: One of the cases was diagnosed early by examining household contacts, while the other was diagnosed by spontaneous demand, after four years of the onset of lesions, although he had been a former patient contact who was not examined at the time. COMMENTS: In endemic countries, the high detection of leprosy in children under 15 years old reveals the persistence of the bacillus transmission and the difficulties encountered by public health programs to control the disease. Delay in leprosy diagnosis leads to sequels and deformities and, thus, the search for contacts is important as an effective method for early diagnosis of the disease in childhood, where clinical signs are not always easy to be identified due to the great variety of clinical forms in which the disease may occur.

Describir dos casos de enfermedad de Hansen en menores de 15 años, siendo uno de paciente con 18 meses de edad y otro de 13 años, diagnosticados por modos de detección distintos, subrayando la importancia de examinar a los contactos. DESCRIPCIÓN DEL CASO: Uno de los casos tuvo diagnóstico temprano mediante examen de contactos intradomiciliares, mientras que el otro fue diagnosticado por demanda espontánea después de cuatro años de surgimiento de las lesiones y, a pesar de ser contacto de un ex-paciente, no fue examinado en la época. COMENTARIOS: En países endémicos, la alta detección de enfermedad de Hansen en menores de 15 años revela la persistencia en la transmisión del bacilo y las dificultades de los programas de salud para el control de la enfermedad. El mayor tiempo para diagnóstico ocasiona secuelas y deformidades y, de ese modo, la búsqueda por los contactos constituye importante método para el diagnóstico temprano de la enfermedad en la infancia, cuando las señales clínicas no siempre son fáciles de identificarse y hay gran diversidad de formas clínicas en que la enfermedad puede presentarse.