Cutaneous tuberculosis in Rio de Janeiro, Brazil: description of a series of 75 cases.

BACKGROUND: Brazil is one of the highest tuberculosis (TB) burden countries of the world. Cutaneous tuberculosis (CTB) is a rare form of extrapulmonary manifestation of tuberculosis. This study aimed to describe the clinico-evolutive, laboratory and therapeutic aspects of CTB cases among patients from a cohort with TB in Rio de Janeiro, Brazil. METHODS: Cases of diagnosed CTB with microbiologic confirmation or clinical response to anti-tuberculous treatment associated with positive smear or histopathological findings between the years 2000 and 2016 were selected. RESULTS: Seventy-five patients with CTB were included, most were women (58.7%) with a median age of 42 years. CTB diagnosis was based on culture in only 42.7% of the cases. Scrofuloderma represented 50.7% of the cases, followed by erythema induratum of Bazin (EIB) (18.7%), tuberculous gumma (13.3%), lupus vulgaris (8%), TB verrucosa cutis (4%), orificial TB (2.7%) and associated forms (2.7%). Other TB presentations were pulmonary (22.7%), mammary (6.6%) and osteoarticular (4%). All patients who completed the treatment (97.3%) had their lesions healed. Only two patients (2.6%) needed to change the therapy due to adverse reactions. Fifty percent of EIB patients presented recurrence. CONCLUSIONS: These data highlight the diversity of CTB presentations and the importance of the skin to assist in early identification and treatment of TB. More studies are necessary to improve the knowledge on EIB for a better approach towards these patients, mainly in cases of recurrence.

Cutaneous tuberculosis and HIV infection at a referral centre in Rio de Janeiro, Brazil.

BACKGROUND: Cutaneous tuberculosis (CTB) is a rare extrapulmonary form of tuberculosis (TB). Despite the increase in the number of cases of TB and HIV, few cases of CTB have been reported. OBJECTIVE: To describe CTB cases among patients with HIV infection from a cohort with tuberculosis. METHODS: We describe a series of 15 CTB and HIV cases, based on secondary data from 2000 to 2016. Diagnosis was based on isolation of Mycobacterium tuberculosis in culture or clinical response to anti-tuberculous treatment associated with positive smear or histopathologic findings from affected skin or an adjacent lymph node. FINDINGS: Scrofuloderma was present in 12 (80%) patients and solitary gumma in three (20%) patients. One case of scrofuloderma was associated with papulonecrotic tuberculid. Seven (46.6%) patients had pulmonary TB. Diagnosis was based on culture in nine patients (60%). The median CD4 cell count was 262 cells/µL. All patients were cured at the end of treatment (median time 6 months). Three patients presented with immune reconstitution inflammatory syndrome. CONCLUSIONS: In this study, CTB associated with HIV infection presented as localised forms or in association with pulmonary TB. In patients with HIV who have subacute and chronic skin lesions, CTB should be considered in differential diagnosis, which may represent a good opportunity for early diagnosis of active TB.

Cutaneous tuberculosis and HIV infection at a referral centre in Rio de Janeiro, Brazil

BACKGROUND Cutaneous tuberculosis (CTB) is a rare extrapulmonary form of tuberculosis (TB). Despite the increase in the number of cases of TB and HIV, few cases of CTB have been reported. OBJECTIVE To describe CTB cases among patients with HIV infection from a cohort with tuberculosis. METHODS We describe a series of 15 CTB and HIV cases, based on secondary data from 2000 to 2016. Diagnosis was based on isolation of Mycobacterium tuberculosis in culture or clinical response to anti-tuberculous treatment associated with positive smear or histopathologic findings from affected skin or an adjacent lymph node. FINDINGS Scrofuloderma was present in 12 (80%) patients and solitary gumma in three (20%) patients. One case of scrofuloderma was associated with papulonecrotic tuberculid. Seven (46.6%) patients had pulmonary TB. Diagnosis was based on culture in nine patients (60%). The median CD4 cell count was 262 cells/µL. All patients were cured at the end of treatment (median time 6 months). Three patients presented with immune reconstitution inflammatory syndrome. CONCLUSIONS In this study, CTB associated with HIV infection presented as localised forms or in association with pulmonary TB. In patients with HIV who have subacute and chronic skin lesions, CTB should be considered in differential diagnosis, which may represent a good opportunity for early diagnosis of active TB.

Evolução clínica e terapêutica dos pacientes com tuberculose cutânea atendidos no Instituto Nacional de Infectologia Evandro Chagas, Fiocruz ­ RJ, entre 2000 e 2016 / Clinical and therapeutic evolution of cutaneous tuberculosis patients treated at the Evandro Chagas National Infectious Disease Institute, Fiocruz - RJ, between 2000 and 2016

Introdução: A tuberculose cutânea (TBC) é uma forma extrapulmonar rara, representando 1-2% dos casos de tuberculose (TB). Objetivo: Avaliar os aspectos clínicos, terapêuticos e evolutivos da TBC em pacientes atendidos em um centro de referência no Rio de Janeiro. Métodos: Realizou-se um estudo longitudinal descritivo de pacientes com TBC da coorte de pacientes do Laboratório de Pesquisa Clínica em Micobacterioses a partir de dados secundários obtidos nos prontuários eletrônicos, de janeiro de 2000 a dezembro de 2016. Foram incluídos pacientes com formas clínicas compatíveis com TBC, idade igual ou maior a 16 anos e: a) diagnóstico estabelecido por cultura positiva, de aspirado de abscesso e/ou de biópsia de lesão cutânea ou de foco contíguo à pele ou; b) resposta terapêutica associada a baciloscopia positiva em exame microscópico direto ou a exame histopatológico compatível com TBC dos mesmos espécimes. Resultados: Foram incluídos 75 (4,5%) pacientes do total de 1.643 casos da coorte de TB no período, com idade mediana de 42 anos, predomínio de mulheres (58,7%) e brancos (58,7%). Condições imunossupressoras estiveram presentes, como infecção por HIV (20%), alcoolismo (6,7%), desnutrição (2,7%) e corticoterapia sistêmica (1,3%). No diagnóstico de TBC, a cultura foi positiva em 42,7% dos casos e, nos demais, o critério foi baseado nos achados histopatológicos (49,3%), no esfregaço corado positivo para bacilos álcool-ácido resistentes (5,3%) ou ambos (2,7%)

A duração mediana dos sintomas até o início do tratamento foi de oito meses. Escrofuloderma (50,7%) foi a forma clínica mais encontrada, seguida de eritema indurado de Bazin (18,7%), TB gomosa (13,3%), lúpus vulgar (8%), TB verrucosa (4%), TB orificial (2,7%) e associação de formas de TBC (2,7%). Outros locais de acometimento foram identificados como pulmonar (22,7%), mamário (6,6%) e sistema osteoarticular (4%). Na análise histopatológica de 39 casos não relacionados à hipersensibilidade (52%), foi encontrado infiltrado granulomatoso (92,3%) e necrose (38,5%) e, nos 14 casos de eritema indurado de Bazin (18,7%), infiltrado granulomatoso (71,4%), necrose (64,3%), paniculite (57,1%) e vasculite (14,3%). Reações adversas ao tratamento ocorreram em 70,7% dos casos, entretanto, apenas 2 (2,6%) necessitaram de mudança no tratamento antituberculose. Todos os pacientes que concluíram o tratamento (97,3%) evoluíram para cura das lesões. A recorrência ocorreu em 50% dos casos de eritema indurado de Bazin. Conclusão: A TBC é um evento raro dentro das TB extrapulmonares e acometeu predominantemente pacientes de gênero feminino e cor branca. Uma vez que é difícil o isolamento de M. tuberculosis na TBC, a histopatologia torna-se uma importante ferramenta diagnóstica. O tratamento no geral foi bem tolerado e, mesmo os casos com reações adversas maiores, evoluíram com cura. Recidivas ocorreram com frequência nos casos de eritema indurado de Bazin. (AU)

Syndrome in question.

Muir-Torre syndrome is a rare genodermatosis characterized by the occurrence of at least one sebaceous tumor associated with visceral neoplasia, but with no predisposing factors. The sebaceous neoplasm may appear before, during or after the diagnosis of colorectal cancer. As it is regarded as a subtype of nonpolyposis hereditary colorectal cancer, it is important to evaluate the patient's first-degree relatives. The clinical course of the neoplasm is usually more indolent and the syndrome has a good prognosis. We report the case of a patient who, after a ten-year diagnosis of colorectal cancer, presented with multiple sebaceous neoplasms.

Syndrome in question

AbstractMuir-Torre syndrome is a rare genodermatosis characterized by the occurrence of at least one sebaceous tumor associated with visceral neoplasia, but with no predisposing factors. The sebaceous neoplasm may appear before, during or after the diagnosis of colorectal cancer. As it is regarded as a subtype of nonpolyposis hereditary colorectal cancer, it is important to evaluate the patient's first-degree relatives. The clinical course of the neoplasm is usually more indolent and the syndrome has a good prognosis. We report the case of a patient who, after a ten-year diagnosis of colorectal cancer, presented with multiple sebaceous neoplasms.

Granulomatous mycosis fungoides--a diagnostic challenge.

Granulomatous mycosis fungoides is a rare subtype of T-cell cutaneous lymphoma. Due to its clinical heterogenicity the diagnosis is delayed and based on histopathological and immuno-histochemical findings, sometimes requiring gene rearrangement studies for confirmation. We report the case of a patient who was submitted to several biopsies before diagnostic conclusion.

Granulomatous mycosis fungoides - A diagnostic challenge

AbstractGranulomatous mycosis fungoides is a rare subtype of T-cell cutaneous lymphoma. Due to its clinical heterogenicity the diagnosis is delayed and based on histopathological and immuno-histochemical findings, sometimes requiring gene rearrangement studies for confirmation. We report the case of a patient who was submitted to several biopsies before diagnostic conclusion.

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.

Dermatoscopic findings in telangiectasia macularis eruptiva perstans.

Telangiectasia macularis eruptiva perstans is a rare form of cutaneous mastocytosis, characterized by the presence of erythematous or yellowish-brown macules with telangiectasias, preferably located on the trunk and upper limbs. We have described a case of telangiectasia macularis eruptiva perstans focusing on the dermoscopic characteristics of this disease.

Dermatoscopic findings in telangiectasia macularis eruptiva perstans / Achados dermatoscopicos na telangiectasia macular eruptiva perstans

Telangiectasia macularis eruptiva perstans is a rare form of cutaneous mastocytosis, characterized by the presence of erythematous or yellowish-brown macules with telangiectasias, preferably located on the trunk and upper limbs. We have described a case of telangiectasia macularis eruptiva perstans focusing on the dermoscopic characteristics of this disease.

A telangiectasia macular eruptiva perstans é uma forma rara de mastocitose cutânea, caracterizada pela presença de máculas eritematosas ou castanho-amareladas com telangiectasias, localizadas preferencialmente no tronco e membros superiores. Descrevemos um caso de telangiectasia macular eruptiva perstans enfocando nas características dermatoscópicas dessa doença.

Case for diagnosis.

Lichen sclerosus is a chronic inflammatory mucocutaneous disorder of unknown etiology that most commonly affects the female genitalia. Cutaneous involvement with nonhaemorrhagic bullous is very unusual. We describe a case of bullous lichen sclerosus.

Case for diagnosis / Caso para diagnóstico

Lichen sclerosus is a chronic inflammatory mucocutaneous disorder of unknown etiology that most commonly affects the female genitalia. Cutaneous involvement with nonhaemorrhagic bullous is very unusual. We describe a case of bullous lichen sclerosus.

Líquen escleroso é uma doença mucocutânea inflamatória, crônica, de etiologia desconhecida, que afeta mais comumente a genitália feminina. O envolvimento cutâneo com bolhas não hemorrágicas é muito raro. Descrevemos um caso de líquen escleroso bolhoso.

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.

Alterações cutâneas no Kwashiorkor: relato de caso de um homem adulto após cirurgia abdominal / Cutaneous manifestations of kwashiorkor: a case report of an adult man after abdominal surgery

Kwashiorkor é um tipo de desnutrição proteico-energética em que há deficiência dietética de proteína, embora a ingestão de calorias se mantenha adequada. As manifestações cutâneas incluem pele xerótica, com aspecto de esmalte descascado, típica coloração avermelhada a branco-acinzentada dos cabelos, o sinal da bandeira e edema mais evidente, nos membros inferiores e na face, dando aspecto de lua cheia. O presente artigo relata o caso de um paciente adulto, do sexo masculino, previamente submetido à duodenopancreatectomia para tratamento de pancreatite crônica associada ao pseudotumor em cabeça de pâncreas que evoluiu com alterações cutâneas de kwashiorkor após tuberculose pulmonar.

Kwashiorkor is a type of protein-energy malnutrition where diet protein deficit is found, in spite of appropriate caloric intake. Cutaneous manifestations include xerosis, with abnormally dry skin that has a flaking enamel paint aspect, a typical red to gray-white hair color, the "flag sign" and more evident edema in lower limbs and face, giving it a full moon appearance. This article reports a case of a male adult patient who had undergone Whipple surgery for treatment of chronic pancreatitis associated with pseudotumor of the pancreatic head that progressed to cutaneous manifestations of kwashiorkor after pulmonary tuberculosis.

Dermatomyofibroma: a case report of a rare disease.

Dermato myofibroma is included in the group of benign cutaneous mesenchymal neoplastic lesions of fibroblastic and myofibroblastic lineage. It's a rare disease and there are approximately only one hundred cases described worldwide in the medical literature up to now. The present study reports the case of a young woman with typical clinical cutaneous lesion and histopathological diagnosis of dermato myofibroma. Special stains were carried out which showed preserved collagen fibers and immunohistochemistry was positive for vimentin and negative for actin and S100. As it is a rare disease, the histopathological findings are of great importance but clinical suspicion is possible in typical cases such as this one.

Dermatomiofibroma: relato de caso de doença rara / Dermatomyofibroma: a case report of a rare disease

O Dermatomiofibroma está incluído no grupo de lesões neoplásicas mesenquimais benignas de linhagem fibroblástica e miofibroblástica da pele. É uma doença rara, havendo aproximadamente 100 casos descritos na literatura mundial até o momento. Este artigo relata o caso de uma mulher jovem com apresentação clínica típica e diagnóstico histopatológico de dermatomiofibroma. Foram realizadas colorações especiais que mostraram preservação das fibras colágenas e a imunohistoquímica revelou positividade para vimentina e negatividade para actina e S100. Por se tratar de doença rara, os achados histopatológicos são de grande importância, mas a supeição clínica é possível em casos típicos como este.

Dermato myofibroma is included in the group of benign cutaneous mesenchymal neoplastic lesions of fibroblastic and myofibroblastic lineage. It's a rare disease and there are approximately only one hundred cases described worldwide in the medical literature up to now. The present study reports the case of a young woman with typical clinical cutaneous lesion and histopathological diagnosis of dermato myofibroma. Special stains were carried out which showed preserved collagen fibers and immunohistochemistry was positive for vimentin and negative for actin and S100. As it is a rare disease, the histopathological findings are of great importance but clinical suspicion is possible in typical cases such as this one.

Cutaneous manifestations of kwashiorkor: a case report of an adult man after abdominal surgery.

Kwashiorkor is a type of protein-energy malnutrition where diet protein deficit is found, in spite of appropriate caloric intake. Cutaneous manifestations include xerosis, with abnormally dry skin that has a flaking enamel paint aspect, a typical red to gray-white hair color, the "flag sign" and more evident edema in lower limbs and face, giving it a full moon appearance. This article reports a case of a male adult patient who had undergone Whipple surgery for treatment of chronic pancreatitis associated with pseudotumor of the pancreatic head that progressed to cutaneous manifestations of kwashiorkor after pulmonary tuberculosis.