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1.
Am J Dermatopathol ; 2019 Nov 12.
Artigo em Inglês | MEDLINE | ID: mdl-31725481

RESUMO

Dermatofibroma (DF) represents one of the most common mesenchymal proliferations of the skin. Their recurrence rate, even when incompletely excised, is very low, whereas the atypical, aneurysmal, and cellular variants have recurrence rates of up to 20% each. Extraordinary rare malignant lesions with metastases to lymph nodes and/or lung have been described. We report a 64-year-old woman with a long history (years) of a skin lesion on her right arm that became painful during the last months. Histologically, it consisted of a conventional cellular DF in which perineural invasion was present. Subsequently, the lesion showed a clinically aggressive course with recurrences, sarcomatous transformation, and pulmonary metastases. Given that no predictive morphological features have been identified to separate classical benign DF from rare metastasizing forms, perineural invasion in an otherwise conventional DF could be a histopathologic clue for an adverse prognosis and should provoke a closer clinical follow-up.

2.
Dermatol Online J ; 25(8)2019 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-31553863

RESUMO

Cutaneous collagenous vasculopathy is a rare clinicopathological entity, first described in 2000. Cutaneous collagenous vasculopathy has been considered a form of microangiopathy of superficial dermal vessels and produce lesions that appear as telangiectasia. We present a patient with histopathologic features of cutaneous collagenous vasculopathy and scattered erythematous papules on the trunk with a striking dermatoscopic finding. We propose the term of 'cutaneous papular collagenous vasculopathy' as a new clinical manifestation of this disease.


Assuntos
Dermatopatias Vasculares/patologia , Idoso , Dermoscopia , Humanos , Masculino , Tronco
3.
Am J Dermatopathol ; 40(10): 754-757, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29570132

RESUMO

Graft-versus-host disease (GVHD) is one of the most common and serious complications of hematopoietic stem-cell transplantation that mainly affects the skin, gastrointestinal tract, and liver. Hepatic GVHD is associated with high morbidity and mortality, and its diagnosis can be especially challenging because of nonspecific clinical signs and symptoms. It must be suspected in patients with elevated liver enzymes and cholestasis, especially in those with a history of preceding skin rash and diarrhea. We describe 3 patients with cutaneous and hepatic GVHD that presented with severe hypercholesterolemia and hypertriglyceridemia, and no xanthomatous macular lesions, in which cutaneous biopsies revealed the presence of xanthomatous dermal histiocytes. We propose that the presence of these xanthomatous cells in skin biopsies from patients with cutaneous GVHD could be a dermatopathological clue for the diagnosis of hepatic GVHD.


Assuntos
Doença Enxerto-Hospedeiro/patologia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Histiócitos/patologia , Hepatopatias/patologia , Fígado/patologia , Dermatopatias/patologia , Pele/patologia , Xantomatose/patologia , Adulto , Biópsia , Criança , Doença Enxerto-Hospedeiro/etiologia , Humanos , Hipercolesterolemia/diagnóstico , Hipercolesterolemia/etiologia , Hipertrigliceridemia/diagnóstico , Hipertrigliceridemia/etiologia , Imuno-Histoquímica , Hepatopatias/etiologia , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Dermatopatias/etiologia
4.
Pediatr Dermatol ; 35(3): e191-e192, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29575181

RESUMO

Papular epidermal nevus with "skyline" basal cell layer is a variant of keratinocytic nevus that usually occurs sporadically but may affect different family members. We report on the fourth family with papular epidermal nevus with "skyline" basal cell layer affecting a 3-month-old girl and her father.


Assuntos
Nevo/patologia , Neoplasias Cutâneas/patologia , Pele/patologia , Feminino , Humanos , Lactente , Masculino
7.
Am J Dermatopathol ; 39(9): 696-699, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28825606

RESUMO

Allogeneic hematopoietic stem-cell transplantation and solid-organ transplantation are associated with an increased risk of secondary neoplasms. Indeterminate cell histiocytosis (ICH) is a rare disease composed of so-called indeterminate cells, an alleged cutaneous dendritic cell subset displaying histological and some ultrastructural and immunophenotypic features of Langerhans cells but lacking Birbeck granules. We report a case of cutaneous ICH occurring after allogeneic hematopoietic stem-cell transplantation for a myelodysplastic syndrome in a 56-year-old man. Microsatellite analysis demonstrated that the neoplastic cells were derived from the donor's hematopoietic system. This case broadens the spectrum of complications after stem-cell transplantation and demonstrates that cutaneous ICH in the setting of myelodysplastic syndromes may have a nonrelated origin to dysplastic myeloid cells.


Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Histiocitose/etiologia , Dermatopatias/etiologia , Humanos , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/cirurgia
8.
Pediatr Dermatol ; 34(4): e223-e224, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28488284

RESUMO

Striated muscle hamartoma (SMH) is a rare, congenital or acquired, benign tumor that predominantly affects children. Therapeutic management has classically been surgical intervention. We present a pediatric case of a facial plaque-type SMH with spontaneous regression that highlights the importance of clinical observation for a conservative approach.


Assuntos
Hamartoma/patologia , Músculo Estriado/patologia , Doenças Musculares/patologia , Criança , Face , Feminino , Humanos , Recém-Nascido , Remissão Espontânea
10.
Pediatr Dermatol ; 34(4): e216-e218, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28543313

RESUMO

Fibroblastic connective tissue nevus (FCTN) is a rare, benign, dermal mesenchymal hamartoma that affects children. We report a 15-year-old boy with a congenital FCTN and describe the clinical, dermatoscopic, and histopathologic features.


Assuntos
Nevo/diagnóstico , Pele/patologia , Adolescente , Antígenos CD34/metabolismo , Dermoscopia , Diagnóstico Diferencial , Humanos , Masculino , Nevo/patologia , Nevo/cirurgia
12.
Am J Dermatopathol ; 39(4): 291-295, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-28323782

RESUMO

Therapies targeting the BRAF oncogene have improved the overall and disease-free survival of patients with advanced melanomas. An unresolved issue in clinical practice is the existence (or not) of BRAF-mutated and BRAF-nonmutated tumors in individual patients (intrapatient BRAF mutation heterogeneity), which may serve as a mechanism of resistance to BRAF inhibitors or lead to diagnostic problems. Different research groups have reported differing results after analyzing the BRAF mutation statuses of multiple melanoma tumors. Herein, we present a brief revision of the literature on this controversial topic and propose a theory to justify the divergence of the results found in the literature.


Assuntos
Análise Mutacional de DNA/métodos , Melanoma/genética , Mutação , Proteínas Proto-Oncogênicas B-raf/genética , Neoplasias Cutâneas/genética , Humanos
13.
JAMA Dermatol ; 153(7): 651-659, 2017 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-28296991

RESUMO

Importance: Histiocytoid Sweet syndrome is a rare histopathologic variant of Sweet syndrome. The nature of the histiocytoid infiltrate has generated considerable controversy in the literature. Objective: The main goal of this study was to conduct a comprehensive overview of the immunohistochemical phenotype of the infiltrate in histiocytoid Sweet syndrome. We also analyze whether this variant of Sweet syndrome is more frequently associated with hematologic malignancies than classic Sweet syndrome. Design: This is a retrospective case series study of the clinicopathologic, immunohistochemical, and molecular features of 33 patients with a clinicopathologic diagnosis of histiocytoid Sweet syndrome was conducted in the dermatology departments of 5 university hospitals and a private laboratory of dermatopathology. Main Outcome and Measures: The clinical, histopathological, immunohistochemical, and follow-up features of 33 patients with histiocytoid Sweet syndrome were analyzed. In some cases, cytogenetic studies of the dermal infiltrate were also performed. We compare our findings with those of the literature. Results: The dermal infiltrate from the 33 study patients (20 female; median age, 49 years; age range, 5-93 years; and 13 male; median age, 42 years; age range, 4-76 years) was mainly composed of myeloperoxidase-positive immature myelomonocytic cells with histiocytoid morphology. No cytogenetic anomalies were found in the infiltrate except in 1 case in which neoplastic cells of chronic myelogenous leukemia were intermingled with the cells of histiocytoid Sweet syndrome. Authentic histiocytes were also found in most cases, with a mature immunoprofile, but they appeared to be a minor component of the infiltrate. Histiocytoid Sweet syndrome was not more frequently related with hematologic malignancies than classic neutrophilic Sweet syndrome. Conclusions and Relevance: The dermal infiltrate of cutaneous lesions of histiocytoid Sweet syndrome is composed mostly of immature cells of myeloid lineage. This infiltrate should not be interpreted as leukemia cutis.


Assuntos
Histiócitos/patologia , Leucemia Mielogênica Crônica BCR-ABL Positiva/patologia , Síndrome de Sweet/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Seguimentos , Hospitais Universitários , Humanos , Imuno-Histoquímica , Leucemia Mielogênica Crônica BCR-ABL Positiva/diagnóstico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Síndrome de Sweet/complicações , Adulto Jovem
15.
J Cutan Pathol ; 44(2): 144-151, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27859466

RESUMO

BACKGROUND: Cutaneous squamous cell carcinoma (CSCC) is the second most frequent cancer in humans and can be both locally invasive and metastatic at distant sites. While research efforts have been made to predict poor outcome of CSCC, there is a lack of knowledge regarding molecular markers. Podoplanin has been associated with poor outcome in several types of cancer including CSCC, but this is controversial and only a few studies have evaluated the prognostic implications of podoplanin in the development of this tumor. METHODS: We evaluated podoplanin expression in a series of 94 CSCCs, and searched for associations between podoplanin expression and histopathological characteristics and with events of poor clinical evolution of the disease. RESULTS: Podoplanin expression was observed in 48.9% of the cases and the expression was considered moderate to intense in 19 of the cases. Moderate/intense podoplanin was associated with infiltrative growth pattern, desmoplasia, lymphovascular invasion, higher risk of nodal progression (NP) and short disease-free survival, specifically with a short latency to NP. CONCLUSIONS: This article provides evidence supporting the implication of podoplanin expression as a marker of bad prognosis of CSCC.


Assuntos
Biomarcadores Tumorais/análise , Carcinoma de Células Escamosas/patologia , Glicoproteínas de Membrana/biossíntese , Neoplasias Cutâneas/patologia , Carcinoma de Células Escamosas/mortalidade , Intervalo Livre de Doença , Feminino , Humanos , Imuno-Histoquímica , Masculino , Glicoproteínas de Membrana/análise , Prognóstico , Neoplasias Cutâneas/mortalidade , Análise Serial de Tecidos
17.
Am J Dermatopathol ; 39(9): 637-662, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27759698

RESUMO

We review the most characteristic clinical and histopathologic findings of the cutaneous manifestations of the occlusive nonvasculitic vasculopathic disorders. Clinically, most of these conditions are characterized by retiform purpura. Histopathologic findings consist of occlusion of the vessel lumina with no vasculitis. Different disorders may produce nonvasculitic occlusive vasculopathy in cutaneous blood and lymphatic vessels, including embolization due to cholesterol and oxalate emboli, cutaneous intravascular metastasis from visceral malignancies, atrial myxomas, intravascular angiosarcoma, intralymphatic histiocytosis, intravascular lymphomas, endocarditis, crystal globulin vasculopathy, hypereosinophilic syndrome, and foreign material. Other times, the occlusive disorder is due to platelet pugging, including heparin necrosis, thrombocytosis secondary to myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, and thrombotic thrombocytopenic purpura. Occlusive vasculopathy may also appear in cold-related gelling agglutination, like that occurring in cryofibrinogenemia, cryoglobulinemia, cold agglutinin syndrome, and crystalglobulinemia. Microorganisms may also occlude the vessels lumina and this is especially frequent in ecthyma gangrenosum, opportunistic fungi as aspergillosis or fusariosis, Lucio phenomenon of lepromatous leprosy and disseminated strongyloidiasis. Systemic coagulopathies due to defects of C and S proteins, coumarin/warfarin-induced skin necrosis, disseminated intravascular coagulation, and antiphospholipid antibody/lupus anticoagulant syndrome may also result in occlusive nonvasculitic vasculopathy. Finally, vascular coagulopathies such as Sneddon syndrome, livedoid vasculopathy, and atrophic papulosis may also cause occlusion of the vessels of the dermis and/or subcutis. Histopathologic study of occlusive vasculopathic lesions is the first step to achieve an accurate diagnosis, and they should be correlated with clinical history, physical examination, and laboratory findings to reach a final diagnosis.


Assuntos
Dermatopatias Vasculares/patologia , Humanos
18.
Clin Case Rep ; 4(12): 1096-1100, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27980740

RESUMO

Primary cutaneous plasmacytoma should be in the differential diagnosis in case of solitary or multiple erythematous-violaceous nodules or papules. The diagnosis relies on clinical, histological, and immunochemical findings, without underlying evidence of multiple myeloma. Treatment should be individualized, and agents such as bortezomib or lenalidomide have shown to be effective.

20.
J Hematol Oncol ; 9(1): 113, 2016 10 20.
Artigo em Inglês | MEDLINE | ID: mdl-27765055

RESUMO

BACKGROUND: Graft-versus-host disease (GvHD) remains the major obstacle to successful allogeneic hematopoietic stem cell transplantation, despite of the immunosuppressive regimens administered to control T cell alloreactivity. PI3K/AKT/mTOR pathway is crucial in T cell activation and function and, therefore, represents an attractive therapeutic target to prevent GvHD development. Recently, numerous PI3K inhibitors have been developed for cancer therapy. However, few studies have explored their immunosuppressive effect. METHODS: The effects of a selective PI3K inhibitor (BKM120) and a dual PI3K/mTOR inhibitor (BEZ235) on human T cell proliferation, expression of activation-related molecules, and phosphorylation of PI3K/AKT/mTOR pathway proteins were analyzed. Besides, the ability of BEZ235 to prevent GvHD development in mice was evaluated. RESULTS: Simultaneous inhibition of PI3K and mTOR was efficient at lower concentrations than PI3K specific targeting. Importantly, BEZ235 prevented naïve T cell activation and induced tolerance of alloreactive T cells, while maintaining an adequate response against cytomegalovirus, more efficiently than BKM120. Finally, BEZ235 treatment significantly improved the survival and decreased the GvHD development in mice. CONCLUSIONS: These results support the use of PI3K inhibitors to control T cell responses and show the potential utility of the dual PI3K/mTOR inhibitor BEZ235 in GvHD prophylaxis.


Assuntos
Doença Enxerto-Hospedeiro/prevenção & controle , Ativação Linfocitária/efeitos dos fármacos , Fosfatidilinositol 3-Quinases/metabolismo , Inibidores de Proteínas Quinases/farmacologia , Proteínas Proto-Oncogênicas c-akt/metabolismo , Serina-Treonina Quinases TOR/metabolismo , Aminopiridinas/farmacologia , Aminopiridinas/uso terapêutico , Animais , Proliferação de Células/efeitos dos fármacos , Imidazóis/farmacologia , Imidazóis/uso terapêutico , Camundongos , Morfolinas/farmacologia , Morfolinas/uso terapêutico , Fosforilação/efeitos dos fármacos , Inibidores de Proteínas Quinases/uso terapêutico , Quinolinas/farmacologia , Quinolinas/uso terapêutico , Transdução de Sinais/efeitos dos fármacos , Linfócitos T/imunologia , Serina-Treonina Quinases TOR/antagonistas & inibidores
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