Necrose gordurosa subcutânea do recém-nascido: manifestações clínicas de dois casos

Subcutaneous fat necrosis of the newborn is an unusual form of panniculitis, with few cases described in medical literature. The disease affects newborns at term or post-term, with normal general health. We describe two cases of newborns affected by the disease. One of them already had lesions since birth. Also, we discuss the use of puncture for diagnostic assistance.A Necrose Gordurosa Subcutânea do Recém-Nascido é uma paniculite incomum com poucos casos descritos na literatura médica. A doença acomete recém-nascidos a termo ou pós-termo, com saúde geral normal. Relatamos dois casos de recém-nascidos acometidos pela doença. Um deles já apresentava lesões ao nascer. Também discutimos o uso da punção para auxílio diagnóstico.Federal University of São Paulo Paulista Medical SchoolFederal University of São Paulo Paulista Medical School dermatology departmentFederal University of São Paulo Paulista Medical School pathology departmentFederal University of São Paulo Paulista Medical School department of dermatologyUNIFESP, Paulista Medical SchoolUNIFESP, EPM, dermatology departmentUNIFESP, EPM, pathology departmentUNIFESP, EPM, department of dermatologySciEL

Disseminated cryptococcosis with skin lesions: report of a case series

Cryptococcosis is a common fungal infection in immunocompromised patients, caused by genus Cryptococcus, presenting with meningitis, pneumonia, and skin lesions. Cutaneous presentation can be varied, but specifically in solid organ transplant recipients (iatrogenically immunocompromised), cryptococcosis should always be considered in the differential diagnosis of cellulitis-like lesions, since the delay in diagnosis leads to worse prognosis and fatal outcome. We report four cases of cryptococcosis with cutaneous manifestation not only for its rarity, but also to emphasize the important role of the dermatologist in the diagnosis of this disease.Univ Fed Sao Paulo EPM UNIFESP, Escola Paulista Med, Dept Pathol, Sao Paulo, SP, BrazilUniv Fed Sao Paulo EPM UNIFESP, Escola Paulista Med, Dept Pathol, Sao Paulo, SP, BrazilWeb of Scienc

DRESS: características clinicopatológicas em 10 casos de Hospital Universitário em São Paulo

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms is a severe form of drug-induced reaction with multiorgan involvement. OBJECTIVES: The aim of this study is to evaluate the epidemiological, clinical and pathological features and prognosis of this drug reaction among patients seen at a dermatology service. METHOD: Retrospective review of medical records of ten patients diagnosed with drug reaction with eosinophilia and systemic symptoms at the Federal University of São Paulo, from august 2008 to may 2011. RESULTS: Phenytoin was the leading cause of drug reaction with eosinophilia and systemic symptoms in our cases, followed by allopurinol. Abnormal liver function tests were observed in 7 patients and renal function impairment in 2 patients. In all cases, patients were hospitalized and the culprit drug was withdrawn. The main treatment was systemic corticosteroid. Drug reaction with eosinophilia and systemic symptoms resulted in death in 2 cases. The causes of death were septic shock and hepatic failure. CONCLUSION: Our mortality rate of 20%, supports that drug reaction with eosinophilia and systemic symptoms is a severe form of drug-induced reaction and must be recognized by all dermatologists.FUNDAMENTOS: A reação a droga com eosinofilia e sintomas sistêmicos é uma reação medicamentosa severa com envolvimento de múltiplos órgãos. OBJETIVO: Avaliar as características epidemiológicas, clínicas, histológicas e o prognóstico dessa reação medicamentosa entre os pacientes atendidos pelo serviço da dermatologia. MÉTODOS: Levantamento retrospectivo dos prontuários de dez pacientes diagnosticados com reação a droga com eosinofilia e sintomas sistêmicos pelo serviço de dermatologia da Universidade Federal de São Paulo (UNIFESP), entre agosto de 2008 e maio de 2011. RESULTADOS: A fenitoína foi a principal causa de reação a droga com eosinofilia e sintomas sistêmicos entre os pacientes, seguida pelo alopurinol. A alteração das enzimas hepáticas foi observada em sete pacientes e diminuição da função renal em dois casos. Todos os pacientes estavam hospitalizados e o medicamento implicado foi suspenso. O principal tratamento foi corticóide sistêmico. Dois pacientes faleceram devido à síndrome reação a droga com eosinofilia e sintomas sistêmicos. A causa da morte foi choque séptico e falência hepática. CONCLUSÃO: A mortalidade de 20% entre os pacientes do estudo confirma que essa reação induzida por droga é grave e deve ser reconhecida por todos os dermatologistas.Universidade Federal de São Paulo (UNIFESP) Dermatology DepartmentUniversidade Federal de São Paulo (UNIFESP) Dermatology and Pathology DepartmentUNIFESP, Dermatology DepartmentUNIFESP, Dermatology and Pathology DepartmentSciEL

Transgrediens pachydermodactyly: report of a case

Pachydermodactyly is a rare form of digital fibromatosis involving the proximal portions of the fingers that usually affects young males. We present a 25-year-old male patient with a twoyear history of asymptomatic nodules in the fingers. Three months before the visit, he had observed similar lesions on the feet and right knee. Histopathological analysis showed thickened dermis with proliferation of fibroblasts and collagenous fibers, with deposition of mucinous material. This represents a rare case of pachydermodactyly of transgrediens form.A paquidermodactilia é forma rara de fibromatose digital envolvendo as porções proximais dos dedos, que afeta homens jovens. Apresenta-se caso de paciente de 25 anos com quadro de nódulos assintomáticos nas mãos há dois anos, e há três meses com lesões semelhantes nos pés e no joelho direito. O exame histopatológico revelou espessamento da derme com proliferação de fibroblastos e de fibras colágenas e aumento de mucina. O caso descrito corresponde à paquidermodactilia do tipo transgressiva.Universidade Federal de São Paulo (UNIFESP)Universidade Federal de São Paulo (UNIFESP) Departamento de PatologiaUniversidade Federal de São Paulo (UNIFESP) Departamento de DermatologiaUNIFESP, Depto. de PatologiaUNIFESP, Depto. de DermatologiaSciEL

Fabry disease: clinical and genotypic aspects of three cases in first degree relatives

Fabry disease is an X-linked, lysosomal storage disease caused by the inherited deficiency of the enzyme α-galactosidase A. The diagnosis is usually late, with renal, cardiovascular and/or cerebral complications that reduce life expectancy. Angiokeratomas are asymptomatic lesions present as the initial manifestation and usually less appreciated. Their detection is important for early diagnosis and institution of treatment with enzyme replacement therapy, which prevents late complications reducing morbidity and mortality. We report a case of a male teenager with acroparestesias and angiokeratomas. Family medical research discovered that his mother and brother had similar signs and symptoms and that the three patients had the same mutation in the gene encoding the enzyme, confirming the diagnosis.Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Dermatology DepartmentUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Dermatology and Pathology departmentsUNIFESP, EPM, Dermatology DepartmentUNIFESP, EPM, Dermatology and Pathology departmentsSciEL

Fatal outcome in classic Kaposi's sarcoma

First described in 1872, Kaposi's sarcoma is defined as a rare multifocal tumor that originates in the endothelial cells and presents with cutaneous and extracutaneous manifestations. The classic form is most common in elderly men and progression is slow. This tumor responds well to chemotherapy and radiotherapy. This report describes a classic case of Kaposi's sarcoma in a woman with skin and visceral manifestations in whom the disease rapidly progressed to a fatal outcome.Descrito em 1872, o sarcoma de Kaposi é neoplasia multicêntrica rara originária de células endoteliais com manifestação cutânea e extracutânea. A forma clássica é muito mais frequente em homens idosos, com evolução prolongada e boa resposta a quimioterapia e radioterapia. Apresentaremos um caso de sarcoma de Kaposi clássico com comprometimento cutâneo e visceral em paciente do sexo feminino com rápida evolução fatal.Universidade Federal de São Paulo (UNIFESP)UNIFESPSciEL

Melanoma developed during pregnancy - A case report

We describe a case of plantar interdigital cutaneous melanoma in a 22-year-old woman who reported changes in a pigmented lesion during pregnancy. Diagnosis was late and evolution unfavourable. The purpose of this report is to draw the attention of dermatologists to the need for careful regular examination of melanocytic lesions in pregnant women, not ignoring possible changes as always physiological.Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM) Department of DermatologyUniversidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM) Departments of Dermatology and PathologyUNIFESP, EPM, Department of DermatologyUNIFESP, EPM, Departments of Dermatology and PathologySciEL