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[PMID]:28328617
[Au] Autor:Marrero Alemán G; El Habr C; Islas Norris D; Montenegro Dámaso T; Borrego L; Sangueza OP
[Ad] Endereço:*Department of Dermatology, Complejo Hospitalario Universitario Insular Materno-Infantil, Universidad de Las Palmas de Gran Canaria, Las Palmas de Gran Canaria, Spain;†Department of Pathology and Dermatology, Wake Forest Baptist Health, Winston-Salem, NC; and‡Department of Pathology, Complejo Hospitalario Universitario Insular Materno-Infantil, Las Palmas de Gran Canaria, Spain.
[Ti] Título:Cutaneous Mastocytosis With Atypical Mast Cells in a 7-Year-Old Girl.
[So] Source:Am J Dermatopathol;39(4):310-312, 2017 Apr.
[Is] ISSN:1533-0311
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Cutaneous mastocytosis is defined by the presence of mast cells within the skin in the absence of other criteria for the diagnosis of systemic mastocytosis. Mast cells are characterized by an abundant granular cytoplasm and a round to oval or spindle-shaped nuclei. The presence of mast cells with bilobed and multilobed nuclei in cutaneous mastocytosis is a rare phenomenon and has been rarely reported in the literature. To our knowledge, there are only 4 reported cases of cutaneous mastocytosis with atypical mast cells. We hereby report a case of urticaria pigmentosa in a 7-year-old female patient. The patient presented with asymptomatic skin lesions of several years duration over the neck and left scapular area. Histopathological examination revealed the presence of middermal perivascular infiltrates mainly composed of mast cells, few lymphocytes, and eosinophils. Most mast cells showed pleomorphic nuclei with bilobed and multilobed morphology that revealed a positive expression for CD117, tryptase, CD68, and Giemsa stains. Based on these findings the diagnosis of urticaria pigmentosa with atypical mast cells was made. Additional tests to rule out systemic involvement were performed. All values, including a tryptase level, were within normal limits. No changes were noted after 1-year follow-up.
[Mh] Termos MeSH primário: Mastócitos/patologia
Urticaria Pigmentosa/patologia
[Mh] Termos MeSH secundário: Criança
Feminino
Seres Humanos
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170420
[Lr] Data última revisão:
170420
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170323
[St] Status:MEDLINE
[do] DOI:10.1097/DAD.0000000000000768


  2 / 1038 MEDLINE  
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[PMID]:27927825
[Au] Autor:Cookson H; Grattan C
[Ad] Endereço:St John's Institute of Dermatology, Guys and St Thomas's Hospitals, London, UK hannahscookson@gmail.com.
[Ti] Título:An update on mast cell disorders.
[So] Source:Clin Med (Lond);16(6):580-583, 2016 Dec.
[Is] ISSN:1473-4893
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Disorders of mast cell activation can be classified as primary (mastocytosis), secondary (reactive) or idiopathic. This article discusses how to recognise and approach the diagnosis of patients suspected to have symptoms of abnormal mast cell activation. Given the highly varied and often complex symptomatology of such patients, we advocate applying a logical step-wise approach to investigating these patients to ensure the correct diagnosis is made. Treatments of mast cell activation disorders are discussed, dividing them into those that ameliorate the effects of mast cell mediators and those that act to stabilise the mast cell.
[Mh] Termos MeSH primário: Mastocitose
[Mh] Termos MeSH secundário: Adulto
Biomarcadores/sangue
Seres Humanos
Mastócitos/fisiologia
Pele/patologia
Triptases/sangue
Urticaria Pigmentosa
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers); EC 3.4.21.59 (Tryptases)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161209
[St] Status:MEDLINE


  3 / 1038 MEDLINE  
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[PMID]:27333655
[Au] Autor:Tsujita J; Doi K; Nakahara M; Nakahara T; Kaku Y; Nishio K; Kan N; Sato Y; Nagata S; Nakao A; Yoshida M; Uchi H; Furue M
[Ti] Título:Overexpression of p16(INK4a) in Mastocytosis (Urticarial Pigmentosa).
[So] Source:Fukuoka Igaku Zasshi;107(1):12-7, 2016 Jan.
[Is] ISSN:0016-254X
[Cp] País de publicação:Japan
[La] Idioma:eng
[Ab] Resumo:The expression of p16(INK4a) has been reported to induce cell-cycle arrest and cellular senescence. The p16(INK4a) expression has never been examined in human mast cells and mastocytosis. We immunohistologically examined the expression of p16(INK4a) and tryptase in 5 normal human skin and 4 mastocytosis. In normal mast cells, only 5.9 ± 3.4 (mean ± standard deviation) % of tryptase-positive mast cells coexpressed p16(INK4a). However, significantly higher percentage (86.0 ± 14.1%) of tryptase-positive tumor cells was immunoreactive to p16(INK4a) in all of 4 mastocytosis. The p16(INK4a) overexpression may induce the senescence of neoplastic mast cells to undergo spontaneous regression of mastocytosis.
[Mh] Termos MeSH primário: Inibidor p16 de Quinase Dependente de Ciclina/genética
Expressão Gênica
Urticaria Pigmentosa/genética
[Mh] Termos MeSH secundário: Pontos de Checagem do Ciclo Celular/genética
Transformação Celular Neoplásica/genética
Transformação Celular Neoplásica/metabolismo
Senescência Celular/genética
Inibidor p16 de Quinase Dependente de Ciclina/metabolismo
Inibidor p16 de Quinase Dependente de Ciclina/fisiologia
Seres Humanos
Mastócitos/patologia
Mastocitose Cutânea/genética
Mastocitose Cutânea/patologia
Regressão Neoplásica Espontânea/genética
Regressão Neoplásica Espontânea/patologia
Pele/metabolismo
Pele/patologia
Triptases/genética
Triptases/metabolismo
Urticaria Pigmentosa/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Cyclin-Dependent Kinase Inhibitor p16); EC 3.4.21.59 (Tryptases)
[Em] Mês de entrada:1607
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160624
[St] Status:MEDLINE


  4 / 1038 MEDLINE  
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Texto completo SciELO Chile
[PMID]:27299829
[Au] Autor:Florenzano P; Mezzano V; Le-Bert M; González G
[Ti] Título:[Bone involvement in systemic mastocytosis: Report of two cases].
[Ti] Título:Compromiso óseo en mastocitosis sistémica: una asociación frecuente, pero muchas veces inadvertida..
[So] Source:Rev Med Chil;144(3):401-5, 2016 Mar.
[Is] ISSN:0717-6163
[Cp] País de publicação:Chile
[La] Idioma:spa
[Ab] Resumo:Systemic mastocytosis (SM) is characterized by pathologic expansion and activation of mast cells. The main clinical manifestations of SM include skin involvement, gastrointestinal symptoms and anaphylaxis due to the release of its mediators. Thirty percent of pat ients with SM have a low bone mass and 20% fractures. At the same time, SM affects 10% of male patients with idiopathic osteoporosis. Measuring serum tryptase is essential for the screening of MS. We report two cases of SM with bone involvement. A 25-year- old woman with prior diagnosis of SM, based on skin involvement, flushing, high serum tryptase and compatible bone marrow (BM) biopsy and genetic study. Low bone mass was diagnosed and treatment was started with calcium and vitamin D plus oral bisphosphona tes with adequate response. A 47 years old man who presented with multiple osteoporotic vertebral fractures and low bone mass. Treatment with vitamin D and alendronate was started, but the patient developed new vertebral fractures. The study was extended w ith measurement of serum tryptase that was elevated. Diagnosis of SM was confirmed with BM biopsy and the patient was referred to hematology for specific care. These cases emphasize the importance of bone assessment in SM, as well as the need to rule out S M in patients with osteoporosis and no evident cause.
[Mh] Termos MeSH primário: Mastocitose Sistêmica/complicações
Osteoporose/etiologia
[Mh] Termos MeSH secundário: Adulto
Biópsia
Densitometria
Feminino
Fraturas Ósseas/etiologia
Seres Humanos
Masculino
Mastocitose Sistêmica/patologia
Meia-Idade
Osteoporose/patologia
Fatores de Risco
Triptases/sangue
Urticaria Pigmentosa/etiologia
Urticaria Pigmentosa/patologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
EC 3.4.21.59 (Tryptases)
[Em] Mês de entrada:1701
[Cu] Atualização por classe:170123
[Lr] Data última revisão:
170123
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160615
[St] Status:MEDLINE


  5 / 1038 MEDLINE  
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[PMID]:26934274
[Au] Autor:Aloysi AS; Ahle GM; Geduldig E; Kellner CH; Bryson EO
[Ad] Endereço:From the Departments of *Psychiatry and †Anesthesiology Icahn School of Medicine at Mount Sinai, New York, NY.
[Ti] Título:General Anesthesia in a Patient With Urticaria Pigmentosa Referred for Electroconvulsive Therapy.
[So] Source:J ECT;32(3):204-6, 2016 Sep.
[Is] ISSN:1533-4112
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Urticaria pigmentosa is a rare disorder characterized by an abnormal systemic proliferation of mast cells. In this condition, various triggers can induce either cutaneous histamine release, resulting in rash, or generalized histamine release, resulting in symptomatic hypotension, syncope, or in its severest form, an anaphylactoid reaction resistant to most resuscitative measures. Many anesthetic agents and adjuncts are known potential triggers, and patients who require surgery or procedures under anesthesia must be managed carefully. In this review, we describe the safe use of general anesthesia for electroconvulsive therapy in a patient with urticaria pigmentosa and discuss the association between psychiatric disorders and mastocytoses.
[Mh] Termos MeSH primário: Anestesia Geral/métodos
Eletroconvulsoterapia/métodos
Urticaria Pigmentosa/complicações
[Mh] Termos MeSH secundário: Transtorno Depressivo/complicações
Transtorno Depressivo/psicologia
Feminino
Seres Humanos
Urticaria Pigmentosa/psicologia
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170302
[Lr] Data última revisão:
170302
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160303
[St] Status:MEDLINE
[do] DOI:10.1097/YCT.0000000000000305


  6 / 1038 MEDLINE  
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[PMID]:26899198
[Au] Autor:Severino M; Chandesris MO; Barete S; Tournier E; Sans B; Laurent C; Apoil PA; Lamant L; Mailhol C; Laroche M; Fraitag S; Hanssens K; Dubreuil P; Hermine O; Paul C; Bulai Livideanu C
[Ad] Endereço:Paul Sabatier University, Toulouse, France; Department of Dermatology, Mastocytosis Competence Center of Midi-Pyrénées, Toulouse University Hospital, Toulouse, France; Toulouse University Hospital, Toulouse, France.
[Ti] Título:Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement.
[So] Source:J Am Acad Dermatol;74(5):885-91.e1, 2016 May.
[Is] ISSN:1097-6787
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Telangiectasia macularis eruptiva perstans (TMEP) has not been fully characterized. OBJECTIVE: We sought to estimate the frequency and clinical characteristics of TMEP in a cohort of adult patients with cutaneous mastocytosis, and to assess the presence of systemic involvement. METHODS: We included all consecutive patients evaluated for cutaneous mastocytosis in 2 centers: the Mastocytosis Competence Center of the Midi-Pyrénées from May 2006 to December 2013, and the French Reference Center for Mastocytosis from January 2008 to September 2013. Skin phenotype, histopathology, presence of KIT mutation in the skin, and assessment of systemic involvement according to World Health Organization (WHO) criteria were prospectively investigated. RESULTS: Of 243 patients with cutaneous mastocytosis, 34 (14%) were given a diagnosis of TMEP. The diagnosis of systemic mastocytosis was established in 16 patients (47%) with TMEP. Three patients (9%) had aggressive systemic mastocytosis (C-findings according to WHO). In all, 32 patients (94%) exhibited at least 1 mast cell activation-related symptom. LIMITATIONS: Patient recruitment was undertaken at 2 referral centers with expertise in the diagnosis and treatment of mastocytosis so that the clinical findings and incidence of systemic involvement may be overestimated in comparison with the overall population of patients with TMEP. CONCLUSION: TMEP accounts for about 14% of patients with cutaneous mastocytosis. The disease manifests as mast cell activation symptoms in almost all patients and can be associated with systemic involvement in about 50% of cases.
[Mh] Termos MeSH primário: Mastocitose Sistêmica/patologia
Telangiectasia/patologia
Urticaria Pigmentosa/patologia
[Mh] Termos MeSH secundário: Adulto
Fatores Etários
Biópsia por Agulha
Estudos de Coortes
Progressão da Doença
Feminino
França
Testes Hematológicos
Seres Humanos
Imuno-Histoquímica
Masculino
Mastocitose Sistêmica/epidemiologia
Mastocitose Sistêmica/fisiopatologia
Meia-Idade
Prognóstico
Encaminhamento e Consulta
Estudos Retrospectivos
Medição de Risco
Índice de Gravidade de Doença
Fatores Sexuais
Telangiectasia/fisiopatologia
Urticaria Pigmentosa/epidemiologia
Urticaria Pigmentosa/fisiopatologia
[Pt] Tipo de publicação:JOURNAL ARTICLE; MULTICENTER STUDY
[Em] Mês de entrada:1609
[Cu] Atualização por classe:160418
[Lr] Data última revisão:
160418
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160223
[St] Status:MEDLINE


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[PMID]:26786799
[Au] Autor:Mulder KM; Vermeer MH
[Ad] Endereço:Leids Universitair Medisch Centrum, afd. Dermatologie, Leiden.
[Ti] Título:[A toddler with an itchy, brown rash].
[Ti] Título:Een peuter met een jeukende, bruine huiduitslag..
[So] Source:Ned Tijdschr Geneeskd;160:A9630, 2016.
[Is] ISSN:1876-8784
[Cp] País de publicação:Netherlands
[La] Idioma:dut
[Ab] Resumo:An otherwise healthy 15-month-old boy presented with a pruritic rash that had gradually developed since birth, leaving brown pigmentation. Darier's sign was positive and the diagnosis of juvenile urticaria pigmentosa was made. Juvenile urticaria pigmentosa usually shows spontaneous remission before puberty. Progression to systemic mastocytosis is extremely rare.
[Mh] Termos MeSH primário: Urticaria Pigmentosa/diagnóstico
[Mh] Termos MeSH secundário: Exantema
Seres Humanos
Lactente
Masculino
Exame Físico
Remissão Espontânea
Maturidade Sexual
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1610
[Cu] Atualização por classe:161230
[Lr] Data última revisão:
161230
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160121
[St] Status:MEDLINE


  8 / 1038 MEDLINE  
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Texto completo SciELO Chile
[PMID]:26541705
[Au] Autor:Zegpi-Trueba MS; Hasbún-Acuña P; Berroeta-Mauriziano D
[Ad] Endereço:Profesor asociado, Departamento de Dermatología, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
[Ti] Título:[Cutaneous mastocytosis: A case report].
[Ti] Título:Mastocitosis cutánea: reporte de un caso..
[So] Source:Rev Chil Pediatr;87(3):204-7, 2016 May-Jun.
[Is] ISSN:0717-6228
[Cp] País de publicação:Chile
[La] Idioma:spa
[Ab] Resumo:INTRODUCTION: Mastocytosis represents a group of diseases characterised by an excesive accumulation of mastocytes in one or multiple tissues. It can affect only the skin, or have a systemic involvement. It has a low prevalence, and the prognosis is benign in children. OBJECTIVE: To report a case of urticaria pigmentosa as a subtype of cutaneous mastocytosis, and present a literature review focused on clinical findings, diagnosis and initial basic management. CLINICAL CASE: A child of six months of age presenting with multiple blemishes and light brown papules located on the trunk, arms and legs. The symptoms were compatible with urticaria pigmentosa, and was confirmed by biopsy. Tests to rule out systemic involvement were requested. The patient was treated with general measures, education, and antihistamines, with favourable results. CONCLUSIONS: Cutaneous mastocytosis is a rare disease with a good prognosis. In childhood general measures and education are usually enough to obtain favourable results. Histamine H1 antagonists are the first line drug treatment.
[Mh] Termos MeSH primário: Mastocitose Cutânea/diagnóstico
Urticaria Pigmentosa/diagnóstico
[Mh] Termos MeSH secundário: Biópsia
Feminino
Antagonistas dos Receptores Histamínicos H1/uso terapêutico
Seres Humanos
Lactente
Mastocitose Cutânea/patologia
Mastocitose Cutânea/terapia
Prognóstico
Urticaria Pigmentosa/patologia
Urticaria Pigmentosa/terapia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Histamine H1 Antagonists)
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170404
[Lr] Data última revisão:
170404
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:151107
[St] Status:MEDLINE


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[PMID]:26006310
[Au] Autor:Ortega-Marlasca MM
[Ad] Endereço:Medicina Familiar y Comunitaria; Ciencias de la Salud, Departamento de Medicina, Facultad de Medicina, Universidad de Cádiz, Cádiz, España. Electronic address: marlasca@makyyo.es.
[Ti] Título:[Allergic dermatopathology or a rare disease?].
[Ti] Título:¿Dermatopatología alérgica o una enfermedad rara?.
[So] Source:Semergen;42(3):192-5, 2016 Apr.
[Is] ISSN:1578-8865
[Cp] País de publicação:Spain
[La] Idioma:spa
[Mh] Termos MeSH primário: Dermatopatias/diagnóstico
Urticaria Pigmentosa/diagnóstico
[Mh] Termos MeSH secundário: Adulto
Diagnóstico Diferencial
Feminino
Seres Humanos
Dermatopatias/patologia
Urticaria Pigmentosa/patologia
[Pt] Tipo de publicação:CASE REPORTS; LETTER
[Em] Mês de entrada:1701
[Cu] Atualização por classe:170130
[Lr] Data última revisão:
170130
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150527
[St] Status:MEDLINE


  10 / 1038 MEDLINE  
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[PMID]:26576050
[Au] Autor:Tew S; Taicher BM
[Ad] Endereço:From the Department of Anesthesiology, Duke University Medical Center, Durham, North Carolina.
[Ti] Título:Avoiding Cardiovascular Collapse: Pediatric Cutaneous Mastocytosis and Anesthetic Challenges.
[So] Source:A A Case Rep;5(10):179-81, 2015 Nov 15.
[Is] ISSN:2325-7237
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Mastocytosis includes a spectrum of diseases characterized by abnormal mast cell infiltration in various organs, which can lead to mast cell mediator release and immediate hypersensitivity. We review anesthetic challenges presented by a 6-year-old girl with a history of mast cell mediator release because of the urticaria pigmentosa variant of cutaneous mastocytosis, factor VII deficiency, increasing episodes of urinary tract infections, and pyelonephritis. She underwent spine magnetic resonance imaging, subsequent lumbar laminectomy for fatty filum release, and a cystourethroscopy. Perioperative management included factor VII desensitization, avoidance of triggers, minimizing histamine-releasing medications, mast cell stabilization, and preparation for potential immediate hypersensitivity.
[Mh] Termos MeSH primário: Anestesia Geral/métodos
Fenômenos Fisiológicos Cardiovasculares
Urticaria Pigmentosa/complicações
[Mh] Termos MeSH secundário: Criança
Fator VII/uso terapêutico
Deficiência do Fator VII/complicações
Feminino
Seres Humanos
Laminectomia/métodos
Vértebras Lombares
Mastócitos
Éteres Metílicos
Infecções Urinárias/complicações
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Methyl Ethers); 38LVP0K73A (sevoflurane); 9001-25-6 (Factor VII)
[Em] Mês de entrada:1607
[Cu] Atualização por classe:151120
[Lr] Data última revisão:
151120
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:151118
[St] Status:MEDLINE
[do] DOI:10.1213/XAA.0000000000000227



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