Granuloma annulare on the palms in an elderly patient

Abstract: Granuloma annulare is a relatively common, idiopathic, benign inflammatory dermatosis, with a varied clinical presentation that often makes diagnosis difficult. It mainly affects the extremities, such as the dorsa of the hands and feet, forearms and legs. Palmar and plantar regions are generally spared. It occurs mainly in young female patients. The presentation of the palmar variant in an elderly patient is a rarity.

Sexual risk behaviors in Brazilian adolescents and young women: a community-based study / Comportamento sexual de risco entre mulheres adolescentes e jovens brasileiras: um estudo de base comunitária

Introduction: Risky sexual behaviors among adolescents can result in high rates of sexually transmitted infections and unwanted pregnancies. Objective: To estimate the prevalence and to identify sociodemographic factors associated with early start of sexual activity and inconsistent condom use among adolescents and young women. Methods: This cross-sectional, community-based study enrolled 1,072 women from 2007 to 2009. The participants were between 15 and 24 years old and were residents of three different mid-sized cities in the state of Goiás, Central-Western region of Brazil. Sociodemographic and behavioral data were collected in a structured questionnaire, following ethics committee approval. Logistic regression analysis was performed, with odds ratio and adjusted odds ratio calculation, with confidence interval of 95% (95%CI) and statistical significance of 5% (p<0.05). Results: Of the 1,072 interviewees, 64.9% were sexually active, of which 46.4% reported sexual initiation at the age of 15 or younger, and 73.2% reported inconsistent condom use. The factors associated with the early start of sexual activity were to be under 20, to have less than eight years of education, and to report no religion with odds ratio of 3.13 (95%CI 2.22­4.40), 6.21 (95%CI 4.41­9.32) and 2.05 (95%CI 1.17­3.58) respectively. The factor associated with inconsistent condom use was being married or in a stable relationship, with odds ratio of 4.63 (95%CI 2.86­7.50). Conclusion: The high prevalence of risky sexual behaviors among Brazilian adolescents and young women is due to socioeconomic and cultural factors.

Introdução: Comportamento sexual de risco entre adolescentes resulta em altas taxas de infecções sexualmente transmissíveis e gravidez indesejada. Objetivo: Estimar a prevalência e identificar os fatores sociodemográficos associados ao início precoce da atividade sexual e ao uso inconsistente do preservativo masculino entre mulheres adolescentes e jovens. Métodos: Estudo transversal, de base comunitária, com 1.072 mulheres realizado entre 2007 e 2009. As participantes tinham entre 15 e 24 anos, residentes em três cidades de médio porte do estado de Goiás, Região Centro-Oeste do Brasil. Dadossociodemográficos e comportamentais foram coletados por meio de questionário estruturado. Análises de regressão logística com cálculo de odds ratio e odds ratio ajustado foram realizadas com intervalo de confiança de 95% (IC95%) e significância estatística de 5% (p<0,05). O estudo foi aprovado pelo Comitê de Ética. Resultados: Das 1.072 entrevistadas, 64,9% eram sexualmente ativas, entre as quais 46,4% reportaram iniciação sexual aos 15 anos ou menos, e 73,2% reportaram uso inconsistente do preservativo masculino. Os fatores associados com a iniciação sexual precoce foram idade menor que 20 anos, ter menos que oito anos de escolaridade e não possuir religião, com odds ratio de 3,13 (IC95% 2,22­4,40), 6,21 (IC95% 4,41 ­9,32) e 2,05 (IC95% 1,17­3,58), respectivamente. O fator associado ao uso inconsistente do preservativo foi o estado civil casada ou união estável, com odds ratio de 4,63 (IC95% 2,86­7,50). Conclusão: A prevalência de comportamento sexual de risco entre mulheres adolescentes e jovens brasileiras é elevada em consequência de fatores socioeconômicos e culturais.

DRESS: relato de caso com estudo genético / DRESS syndrome: case report with genetic testin

A síndrome de hipersensibilidade a drogas com eosinofilia e sintomas sistêmicos (DRESS) é uma rara reação adversa a drogas com potencial de morte e sequelas em longo prazo. Os anticonvulsivantes aromáticos estão entre os medicamentos mais relacionados. Relatamos um caso de DRESS em associação com o alelo HLA-A*31:01, destacando aspectos clínico-laboratoriais, abordagem diagnóstica e acompanhamento ambulatorial de sequelas tardias. Homem com 69 anos, natural do Japão, internado com suspeita clínica de DRESS. Havia iniciado carbamazepina 4 semanas antes do rash cutâneo para tratamento de epilepsia. Apresentou biópsia cutânea compatível com farmacodermia. O paciente foi tratado com prednisolona por 4 meses. A tipagem HLA-A-B-DRB1 por PCR-RSSO (ONE LAMBDA) e SSP alelo específico revelou HLA relacionado a reações de hipersensibilidade à carbamazepina. O teste de contato realizado com carbamazepina a 10% no primeiro ano após a reação foi positivo. A restrição futura da classe de anticonvulsivantes aromáticos foi recomendada. Oito meses após a aparente resolução clínica da DRESS, o paciente desenvolveu aumento dos anticorpos antitireoideanos e doença de Hashimoto. Treze meses após a o início da reação, foi observado aumento nos títulos de FAN, sem manifestações clínicas. Este relato de caso descreve aspectos clínico-laboratoriais típicos de DRESS relativos ao diagnóstico clínico-laboratorial e histopatológico, bem como evolução clínica em curto e longo prazos. A abordagem farmacogenética e o teste de contato foram importantes para a confirmação da imputabilidade da carbamazepina na etiologia da DRESS.

Drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) is a rare, potentially fatal adverse reaction to drugs that may have long-term sequelae. Aromatic anticonvulsants are among the drugs most commonly associated with DRESS. We report a case of DRESS associated with allele HLA-A*31:01, with emphasis on clinical and laboratory findings, the diagnostic approach adopted, and outpatient follow-up of late sequelae. A 69-year old Japanese male patient was admitted with a clinical suspicion of DRESS. He had started carbamazepine treatment for epilepsy 4 weeks before the rash. He presented skin biopsy compatible with pharmacodermia. The patient was treated with prednisolone for 4 months. HLA-A-B-DRB1 typing using the PCRRSSO technique (ONE LAMBDA) and specific SSP allele revealed HLA related to hypersensitivity reactions to carbamazepine. The skin test performed with carbamazepine 10% on the first day after the reaction resulted positive. Future restriction of aromatic anticonvulsants was recommended. Eight months after the apparent clinical resolution of DRESS, the patient showed increased levels of antithyroid antibodies and Hashimoto disease. Thirteen months after the onset of the reaction, increased FAN results were observed, with no clinical manifestations. This case report describes clinical and laboratory aspects of DRESS related to clinical, laboratory, and histopathological diagnosis, as well as clinical evolution in the short and long terms. The pharmacogenetic approach and the skin test were important to confirm the imputability of carbamazepine in the etiology of DRESS.

Clinical exuberance of classic Kaposi's sarcoma and response to radiotherapy

Abstract: Kaposi's sarcoma (KS) is a multicentric vascular neoplasm, with cutaneous and extracutaneous involvement. Different clinical and epidemiological variants have been identified. The classic form is manifested mainly in elderly men with indolent and long-term evolution, with lesions localized primarily in the lower extremities. We present two cases of classic Kaposi's sarcoma (CKS) in two female patients with extensive, exuberant skin involvement and rapid evolution, with good response to radiotherapy.

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

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.

Pigmented Bowen's disease

Pigmented Bowen's disease is rare, though more prevalent in men. It presents as a well-delineated plaque in areas unexposed to sun. There are reports of association with seborrheic keratosis, solar lentigo or exuberant pigmentation of genital and intertriginous regions. A specific dermoscopy finding is the presence of brown or gray dots in regular arrangement and coiled or dotted vessels. Thus, we aim to raise awareness of the diagnosis of pigmented Bowen's disease in pigmented lesions.

Primary systemic amyloidosis, acquired cutis laxa and cutaneous mucinosis in a patient with multiple myeloma / Amiloidose sistemica primaria, cutis laxa adquirida e mucinose cutanea em paciente com mieloma multiplo

A 57-year-old woman presented with periorbital ecchymoses, laxity in skin folds, polyneuropathy and bilateral carpal tunnel syndrome. A skin biopsy of the axillary lesion demonstrated fragmentation of elastic fibers, but with a negative von Kossa stain, consistent with cutis laxa. The diagnosis of primary systemic amyloidosis was made by the presence of amyloid material in the eyelid using histopathological techniques, besides this, the patient was also diagnosed with purpura, polyneuropathy, bilateral carpal tunnel syndrome and monoclonal gammopathy. She was diagnosed as suffering from multiple myeloma based on the finding of 40% plasma cells in the bone marrow, component M in the urine and anemia. The patient developed blisters with a clear content, confirmed as mucinosis by the histopathological exam. The final diagnoses were: primary systemic amyloidosis, acquired cutis laxa and mucinosis, all related to multiple myeloma.

Mulher de 57 anos, com equimose periorbitária, frouxidão cutânea nas dobras, polineuropatia e síndrome do túnel do carpo bilateral.O exame histopatológico da lesão axilar revelou fragmentação de fibras elásticas, porém a coloração de von Kossa foi negativa;o diagnóstico foi de cútis laxa. Amiloidose sistêmica primária foi confirmada pela presença de material amilóide no exame histopatológico da pálpebra, além de púrpura, polineuropatia, síndrome do túnel do carpo bilateral e gamopatia monoclonal. Foi diagnosticada como portadora de mieloma múltiplo por apresentar 40% de plasmócitos na medula óssea, componente M urinário e anemia. A paciente evoluiu com bolhas de conteúdo citrino, cujo exame histopatológico mostrou mucinose. Os diagnósticos finais foram: amiloidose sistêmica primária, cútis laxa adquirida e mucinose, todos vinculados ao mieloma múltiplo.

Diffuse systemic sclerosis with bullous lesions without systemic manifestations / Esclerose sistemica difusa bolhosa sem acometimentos sistemicos

Here, we describe an atypical case of systemic sclerosis in its diffuse cutaneous form with acute and rapid progression of the cutaneous condition, without any systemic manifestations and the infrequent formation of bullae, showing the importance of diagnosis and early treatment in such cases. This case also shows that special measures should be taken for bullous cutaneous lesions and ulcerations resulting from serious sclerosis, which are entry points and increase morbidity and risk of death. Other prognostic factors include age, ESR and renal and pulmonary involvement. Capillaroscopies can be useful predictors of greater severity of systemic scleroderma, revealing a greater link with systemic, rather than cutaneous, involvement.

Descrevemos um caso atípico de esclerose sistêmica em sua forma cutânea difusa com instalação aguda e rápida progressão do quadro cutâneo sem qualquer acometimento sistêmico e a infrequente formação de bolhas, demostrando a importância do diagnóstico e tratamento precoce frente a casos semelhantes e mostrando, com a experiência deste caso, que cuidados especiais devem ser tomados com as lesões cutâneas bolhosas e as ulcerações decorrentes da grave esclerose que são portas de entrada e aumentam a morbidade e risco de morte. Outros fatores prognósticos descritos são idade, VHS e envolvimento pulmonar e renal. A capilaroscopia pode ser preditor de maior gravidade da esclerodermia sistêmica, guardando maior relação com o envolvimento sistêmico do que cutâneo.

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 / 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 / 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.

Sarcoidosis simulating syringomas / Sarcoidose simulando siringomas

Sarcoidosis is a granulomatous disease of unknown etiology. The skin is commonly affected. Cutaneous manifestations can mimic other diseases and autoimmune disorders. The dermatologist plays a critical role in elucidating the clinical diagnosis and assisting other specialists in the investigation of a systemic disease. We report a patient with typical cutaneous manifestation of sarcoidosis with pulmonary involvement.

A sarcoidose é uma doença granulomatosa de etiologia desconhecida. A pele é comumente afetada. As manifestações cutâneas podem mimetizar outras afecções e desordens auto-imunes. Assim, o dermatologista assume papel fundamental para elucidar o diagnóstico clínico e auxiliar outros especialistas na investigação de uma doença sistêmica. Relatamos um caso de paciente com manifestação cutânea típica de sarcoidose associada com acometimento pulmonar.

Case for diagnosis / Caso para diagnóstico

Vascular leiomyoma are uncommon benign smooth muscle tumors which generally present as a single painful nodule in the lower limbs. We report a case of vascular leiomyoma on the second finger of the left hand, an unusual location for this tumor.

Angioleiomiomas são tumores benignos raros derivados da musculatura lisa vascular, que geralmente se apresentam como nódulo doloroso solitário nos membros inferiores. Relata-se um caso de angioleiomioma no segundo quirodátilo esquerdo, localização incomum deste tumor.

Caso para diagnóstico / Case for diagnosis

Relatamos um caso de histiocitose cefálica benigna em uma criança do sexo masculino, de um ano e três meses de idade que desenvolveu múltiplas pápulas na região malar bilateralmente, sem outros comemorativos associados. A histopatologia caracterizou-se pelo padrão derme papilar, com imuno-histoquímica negativa para S100 e CD1a, e positiva para CD68, ficando assim estabelecido o diagnóstico desta histiocitose não- Langerhans, baseado nos aspectos clínicos, histopatológicos e imuno-histoquímicos característicos.

The present paper reports a case of benign cephalic histiocytosis in a 15-month baby boy, who developed multiple papules bilaterally in the malar region with no other associated manifestations. Histopathology revealed a papillary dermal pattern, while immunohistochemistry was negative for S100 and CD1a and positive for CD68. Therefore, diagnosis was established as non-Langerhans cell histiocytosis, based on the clinical, histopathological and immunohistochemical features present.

Doença de Dowling-Degos: apresentação clínica e histopatológica clássica / Dowling-Degos disease: classic clinical and histopathological presentation

A doença de Dowling-Degos é uma genodermatose rara que consiste numa desordem pigmentar reticulada. Caracteriza-se pela presença de máculas hiperpigmentadas nas regiões flexurais com distribuição em rede; lesões tipo comedão no dorso e na região cervical; e cicatrizes cribriformes na face, particularmente periorais. Apresentamos um caso de um paciente de 51 anos, masculino, com lesões tipo macrocomedões, cicatrizes cribriformes, cistos e máculas hipercrômicas no dorso, tórax anterior, axilas, pescoço, região genital e face. Relatava ter dois filhos, três irmãos e o pai com quadro semelhante. As biópsias de pele foram características da doença de Dowling-Degos, mostrando dilatação folicular, epiderme digitiforme, com áreas de aspecto de "chifre de veado" e focos de hiperpigmentação da camada basal.

Dowling-Degos disease (DDD) is a rare genetic disease of the skin (reticulate pigmented anomaly), clinically characterized by flexural brown pigmented reticulate macules, comedo-like papules on the back, neck and pitted perioral or facial scars. We present the case of a 51 year-old man with macrocomedo-like lesions, pitted scars, cysts, hyperpigmented macules in his back, chest, axillae, neck, groin and face. The patient reported having two children, three brothers and a father with a similar condition. The histopathology of the skin biopsies was very characteristic of Dowling-Degos disease, showing dilated follicular, fingerlike projections called rete ridges (dermal pegs), with thinning of the suprapapillary plates, resulting in an "antler-like" pattern and increased pigmentation of the basal layer.

Síndrome de Sweet associada a linfoma de Hodgkin: relato de caso / Sweet's Syndrome associated with Hodgkin's disease: case report

A síndrome de Sweet é enfermidade cutânea rara e de etiologia pouco esclarecida. Cerca de 20 por cento dos casos são associados a neoplasias hematológicas, sendo raros os casos relacionados à doença de Hodgkin. Relata-se caso de paciente masculino de 57 anos que desenvolveu a síndrome concomitantemente à neoplasia. As doenças foram controladas com o tratamento específico.

Sweet's syndrome is a rare cutaneous disease of unknown etiology. About 20 percent of the cases are associated with hematological neoplasms, and cases related with Hodgkin's disease are rare. We present the case of a 57-year old male patient who developed the syndrome concomitantly with the neoplasm. The diseases were controlled with specific treatment.

Tinea granulomatosa de Majocchi / Majocchi's granuloma

Relata-se o caso de um homem de 45 anos com dermatofitose superficial de longa data, tratado, inadvertidamente, com corticoide e antibiótico, com progressão subsequente para a forma profunda, conhecida como granuloma de Majocchi. O tratamento com terbinafina VO foi curativo.

We report the case of a man of 45 with superficial dermatophytosis longtime inadvertently treated with antibiotics and corticosteroids with subsequent progression to the deep form, known as granuloma Majocchi. Treatment with orally terbinafine was successful.