Necrobiotic xanthogranuloma without paraproteinemia

Necrobiotic xanthogranuloma is a chronic granulomatous and xantomathous disease, characterized by indurated, nontender, yellowish and erythematous nodules and plaques especially located on the periorbital region. It is commonly associated with paraproteinemia and an increased risk for hematological and lymphoproliferative malignancies. Its pathogenesis remains unclear. Alkylating agents, such as chlorambucil and melphalan may be used to treat the disease with variable success. We report a case of this rare disease in a patient with a solitary tumor and without paraproteinemia.O xantogranuloma necrobiótico é doença crônica granulomatosa e xantomatosa, caracterizada por pápulas e placas infiltradas, eritematosas e amareladas, preferencialmente localizadas na região periorbital. É comum associar-se com paraproteinemia e risco aumentado para malignidades hematológicas e linfoproliferativas. Sua patogênese permanece desconhecida. Agentes alquilantes, como clorambucil e melfalan, podem ser utilizados no tratamento com sucesso variável. Relata-se um exemplo dessa rara doença em paciente com lesão única e sem paraproteinemia.Universidade Federal de São Paulo (UNIFESP) Departamento de Dermatologia Escola Paulista de MedicinaUNIFESP, Depto. de Dermatologia Escola Paulista de MedicinaSciEL

Polypoid melanoma and superficial spreading melanoma different subtypes in the same lesion

Melanoma is a malignant melanocytic neoplasm with high mortality rate, and steadily and universally increasing incidence rates. Polypoid melanoma is considered an exophytic variant of the nodular subtype. The incidence of polypoid melanoma is extremely variable, most likely because of the different criteria used for its characterization. We presented a rare case of polypoid melanoma and superficial spreading melanoma in the same lesion.Universidade Federal de São Paulo (UNIFESP)UNIFESPSciEL

Nevo de Spitz e nevo de Reed

Spitz and Reed nevi, also called spindle and/or epithelioid cell nevi, are a special group of melanocytic lesions due to their peculiar clinical, dermoscopic and histopathological features. The study of these nevi is of great interest because both their nomenclature and classification are still a matter of discussion. Most importantly, the difficulty to differentiate them from melanoma can lead to inadequate therapies. In this context, dermoscopy, a link between clinical and anatomopathological examinations, appears as a helpful diagnostic tool whose accuracy can reach 93%. Borderline lesions are still a great challenge and object of research, including molecular studies. The present study explores the relevant characteristics of these nevi, with emphasis on dermoscopic findings, aiming at understanding their natural history, as well as discussing treatment and patient follow-up.Os nevos de Spitz e de Reed, ou também chamados nevos de células fusiformes e/ou epitelióides, formam um grupo especial de lesões melanocíticas, por suas características peculiares, tanto clínicas como dermatoscópicas e histopatológicas. Torna-se grande o interesse no estudo desses nevos, pois tanto a nomenclatura como a classificação ainda são motivos de debate e, sobretudo, a dificuldade de diferenciação com o melanoma pode gerar condutas terapêuticas inadequadas. Neste contexto, surge a dermatoscopia, elo entre o exame clínico e o anatomopatológico, como método diagnóstico auxiliar importante cuja acurácia pode chegar a 93%. A grande dificuldade ainda são as lesões borderline, motivo de pesquisas, incluindo estudos moleculares. O presente estudo aborda os aspectos relevantes das características destes nevos, com ênfase nos achados dermatoscópicos, buscando o entendimento da sua história natural, além da discussão quanto ao tratamento e seguimento dos pacientes.ABC School of MedicineUniversidade Federal de São Paulo (UNIFESP) Department of Pathology and DermatologyUNIFESP, Department of Pathology and DermatologySciEL

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

Giant atypical lipoma

Liposarcomas correspond to the most common histological subtype of soft tissue sarcomas. They can be subdivided into: well differentiated or atypical lipoma, undifferentiated, myxoid, round, and pleomorphic cells. Atypical lipomas are the most prevalent and usually appear as asymptomatic softened tumors. They are locally aggressive but rarely lead to distant metastases. The diagnosis of this tumor is based on the imaging and histopathologic findings. Treatment consists of excision surgery with complete tumor removal. It has a good prognosis due to the low percentage of distant metastases. We report a rare case of giant atypical lipoma as well as the adopted therapy and evolution.Univ Fed Sao Paulo UNIFESP, Dept Dermatol, Sao Paulo, SP, BrazilUniv Fed Sao Paulo UNIFESP, Dept Pathol, Sao Paulo, SP, BrazilUniv Fed Sao Paulo UNIFESP, Dept Dermatol, Sao Paulo, SP, BrazilUniv Fed Sao Paulo UNIFESP, Dept Pathol, Sao Paulo, SP, BrazilWeb of Scienc

Dysplastic nevus associated with seborrheic keratosis

Seborrheic keratosis is a common skin lesion which may coincidentally be associated melanocytic nevi. The authors describe a case of dysplastic nevus associated with seborrheic keratosis and discuss the clinical, dermoscopic, and histological findings of this association. They also discuss the association between seborrheic keratosis and other benign and malignant tumours.Federal University of São Paulo Department of DermatologyFederal University of São Paulo Department of PathologyFederal University of São Paulo Departments of Dermatology and PathologyFederal University of São Paulo Departments of DermatologyUNIFESP, Department of DermatologyUNIFESP, Department of PathologyUNIFESP, Departments of Dermatology and PathologyUNIFESP, Departments of DermatologySciEL

Coexistence of chronic cutaneous lupus erythematosus and frontal fibrosing alopecia

Lupus erythemathosus is a chronic, relapsing disease with acute, subacute, and chronic lesions. Effluvium telogen occurs in the setting of systemic activity of the disease, and cicatricial alopecia results from discoid lesionsin on the scalp. Other types of alopecia, like alopecia areata, may rarely be found in lupus. Frontal fibrosing alopecia is characterized by frontotemporal hairline recession and eybrow loss. Histophatologically, it cannot be differentiated from lichen planopilaris. It is controversial whether frontal fibrosing alopecia is a subtype of lichen planopilaris.. A pacient with chronic lichenoid lupus erythematosus is described with clinical, histophatological and dermoscopic features of frontal fibrosing alopecia. We have not been able to find in the literature cases of frontal fibrosing alopecia as a clinical manifestation of lupus.Univ Fed Sao Paulo, Unifesp, Connect Tissue Dis Outpatient Clin, Dept Dermatol, Sao Paulo, SP, BrazilUniv Fed Sao Paulo, Unifesp, Dept Dermatol, Sao Paulo, SP, BrazilUniv Fed Sao Paulo, Unifesp, Dept Anat Pathol, Sao Paulo, SP, BrazilUniv Fed Sao Paulo, Unifesp, Connect Tissue Dis Outpatient Clin, Dept Dermatol, Sao Paulo, SP, BrazilUniv Fed Sao Paulo, Unifesp, Dept Dermatol, Sao Paulo, SP, BrazilUniv Fed Sao Paulo, Unifesp, Dept Anat Pathol, Sao Paulo, SP, BrazilWeb of Scienc

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

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

Acne arising on a facial Becker nevus following the lines of Blaschko

Univ Fed São Paulo UNIFESP, Escola Paulista Med, Dept Dermatol, São Paulo, SP, BrazilUniv Fed São Paulo UNIFESP, Escola Paulista Med, Dept Pathol, São Paulo, SP, BrazilUniv Fed São Paulo UNIFESP, Escola Paulista Med, Dept Dermatol, São Paulo, SP, BrazilUniv Fed São Paulo UNIFESP, Escola Paulista Med, Dept Pathol, São Paulo, SP, BrazilWeb of Scienc