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  1 / 1142 MEDLINE  
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[PMID]:28742296
[Au] Autor:Patel S; Kumar S; Laudenbach JM; Teruel A
[Ti] Título:Mucocutaneous Diseases: Oral Lichen Planus, Mucous Membrane Pemphigoid and Pemphigus Vulgaris.
[So] Source:J Calif Dent Assoc;44(9):561-70, 2016 Sep.
[Is] ISSN:1043-2256
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Mucocutaneous diseases affect the oral cavity and can present a diagnostic challenge. They can have systemic involvement, necessitating multidisciplinary management. Frequently, patients will see their general dentists initially for evaluation. A better understanding of mucocutaneous diseases can prevent delay in appropriate diagnosis and treatment. Oral lichen planus, mucous membrane pemphigoid and pemphigus vulgaris are three mucocutaneous diseases that affect the oral mucosa. This review describes the clinical features, epidemiology, etiology, pathogenesis and management for each condition.
[Mh] Termos MeSH primário: Líquen Plano Bucal
Penfigoide Mucomembranoso Benigno
Pênfigo
[Mh] Termos MeSH secundário: Seres Humanos
Líquen Plano Bucal/patologia
Líquen Plano Bucal/terapia
Penfigoide Mucomembranoso Benigno/patologia
Penfigoide Mucomembranoso Benigno/terapia
Pênfigo/patologia
Pênfigo/terapia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180102
[Lr] Data última revisão:
180102
[Sb] Subgrupo de revista:D
[Da] Data de entrada para processamento:170726
[St] Status:MEDLINE


  2 / 1142 MEDLINE  
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[PMID]:29052880
[Au] Autor:Yuan H; Pan M
[Ad] Endereço:Department of Dermatology, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, China.
[Ti] Título:Endoscopic characteristics of oesophagus involvement in mucous membrane pemphigoid.
[So] Source:Br J Dermatol;177(4):902-903, 2017 10.
[Is] ISSN:1365-2133
[Cp] País de publicação:England
[La] Idioma:eng
[Mh] Termos MeSH primário: Penfigoide Mucomembranoso Benigno
Penfigoide Bolhoso
[Mh] Termos MeSH secundário: Endoscopia
Esôfago
Seres Humanos
Membrana Mucosa
[Pt] Tipo de publicação:JOURNAL ARTICLE; COMMENT
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171107
[Lr] Data última revisão:
171107
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171021
[St] Status:MEDLINE
[do] DOI:10.1111/bjd.15804


  3 / 1142 MEDLINE  
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[PMID]:29029901
[Au] Autor:Cizenski JD; Michel P; Watson IT; Frieder J; Wilder EG; Wright JM; Menter MA
[Ad] Endereço:Division of Dermatology, Baylor University Medical Center, Dallas, Texas.
[Ti] Título:Spectrum of orocutaneous disease associations: Immune-mediated conditions.
[So] Source:J Am Acad Dermatol;77(5):795-806, 2017 Nov.
[Is] ISSN:1097-6787
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:There are a number of diseases that manifest both on the skin and the oral mucosa, and therefore the importance for dermatologists in clinical practice to be aware of these associations is paramount. In the following continuing medical education series, we outline orocutaneous disease associations with both immunologic and inflammatory etiologies.
[Mh] Termos MeSH primário: Doenças Autoimunes/imunologia
Doenças da Boca/imunologia
Mucosa Bucal/imunologia
Dermatopatias Vesiculobolhosas/epidemiologia
Dermatopatias Vesiculobolhosas/imunologia
[Mh] Termos MeSH secundário: Autoanticorpos/sangue
Doenças Autoimunes/epidemiologia
Doenças Autoimunes/fisiopatologia
Educação Médica Continuada
Feminino
Seres Humanos
Incidência
Masculino
Doenças da Boca/epidemiologia
Doenças da Boca/fisiopatologia
Mucosa Bucal/patologia
Penfigoide Mucomembranoso Benigno/epidemiologia
Penfigoide Mucomembranoso Benigno/imunologia
Penfigoide Mucomembranoso Benigno/fisiopatologia
Penfigoide Bolhoso/epidemiologia
Penfigoide Bolhoso/imunologia
Penfigoide Bolhoso/fisiopatologia
Pênfigo/epidemiologia
Pênfigo/imunologia
Pênfigo/fisiopatologia
Prognóstico
Medição de Risco
Dermatopatias Vesiculobolhosas/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Autoantibodies)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171102
[Lr] Data última revisão:
171102
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171015
[St] Status:MEDLINE


  4 / 1142 MEDLINE  
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[PMID]:28860044
[Au] Autor:Labowsky MT; Stinnett SS; Liss J; Daluvoy M; Hall RP; Shieh C
[Ad] Endereço:Duke University School of Medicine, Durham, North Carolina. Electronic address: marytlabowsky@gmail.com.
[Ti] Título:Clinical Implications of Direct Immunofluorescence Findings in Patients With Ocular Mucous Membrane Pemphigoid.
[So] Source:Am J Ophthalmol;183:48-55, 2017 Nov.
[Is] ISSN:1879-1891
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: To examine the clinical implications of positive or negative direct immunofluorescence biopsies (DIF) in patients with clinically typical ocular mucous membrane pemphigoid (MMP). DESIGN: Retrospective cohort study. METHODS: The study population was patients with clinically typical ocular MMP disease with documented DIF results who were followed for at least 1 year at the Duke University multidisciplinary ocular MMP clinic. Data were collected by chart review and included patient demographics, clinical examination findings, and history of autoimmune disease and/or malignancy, as well as topical, systemic, and surgical treatments received. Main outcome measures included MMP Disease Area Index, Foster stages, proportion legally blind, duration of follow-up, and use of systemic immunosuppression and ocular procedures in treatment. RESULTS: In multivariable analysis restricted to 55 patients, patients with negative and positive biopsies were similar in the outcome measures; however, positive-biopsy patients were more likely to be treated with systemic immunosuppression and were followed for longer at our clinic. Patients with isolated ocular disease were also more likely to have negative biopsies compared to those who also had extraocular disease. Patients who had conjunctival biopsies were more likely to have a negative direct immunofluorescence result than patients with biopsies from other sites. CONCLUSIONS: We encourage clinicians and patients to consider treatment with systemic immunosuppression even in the absence of diagnosis confirmation by DIF. Furthermore, this study supports current standard of care to pursue a nonocular biopsy of normal-appearing, perilesional skin or oral mucosa when possible.
[Mh] Termos MeSH primário: Autoanticorpos/análise
Túnica Conjuntiva/patologia
Doenças da Túnica Conjuntiva/diagnóstico
Técnica Direta de Fluorescência para Anticorpo/métodos
Penfigoide Mucomembranoso Benigno/diagnóstico
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Autoanticorpos/imunologia
Membrana Basal/patologia
Biópsia
Doenças da Túnica Conjuntiva/imunologia
Feminino
Seguimentos
Seres Humanos
Masculino
Meia-Idade
Penfigoide Mucomembranoso Benigno/imunologia
Reprodutibilidade dos Testes
Estudos Retrospectivos
Índice de Gravidade de Doença
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Autoantibodies)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171102
[Lr] Data última revisão:
171102
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170902
[St] Status:MEDLINE


  5 / 1142 MEDLINE  
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[PMID]:28676329
[Au] Autor:Shimanovich I; Nitz JM; Zillikens D
[Ad] Endereço:Department of Dermatology, University of Lübeck, Lübeck, Germany. Electronic address: iakov.shimanovich@uksh.de.
[Ti] Título:Multiple and repeated sampling increases the sensitivity of direct immunofluorescence testing for the diagnosis of mucous membrane pemphigoid.
[So] Source:J Am Acad Dermatol;77(4):700-705.e3, 2017 Oct.
[Is] ISSN:1097-6787
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Mucous membrane pemphigoid (MMP) is an autoimmune disease characterized by the predominant blistering of mucosal surfaces and the linear deposition of complement, IgG, or IgA along the basement membrane detected by direct immunofluorescence (DIF) test. OBJECTIVE: To assess the impact of multiple and repeated DIF sampling on establishing the diagnosis of MMP. METHODS: We reviewed the results of DIF studies in 136 nonlesional biopsies from 78 patients who were immunologically confirmed to have MMP. RESULTS: Thirty-six of 52 patients (69%) who underwent only 1 biopsy at the first workup were positive. In 13 cases, the initial single biopsy was negative, and later biopsies were positive. Twenty-two of 26 patients (85%) who underwent multiple biopsies at the initial workup showed ≥1 positive DIF test result. Simultaneously obtained biopsies yielded discordant positive and negative findings in 11 patients. Overall, 74 of 78 patients (95%) had ≥1 positive result by DIF test. In the remaining 4 cases, the diagnosis was confirmed by the detection of circulating autoantibodies against BP180. LIMITATIONS: This is a retrospective, single-center study. CONCLUSION: Our data demonstrate that multiple and repeated biopsies increase the sensitivity of the DIF test for MMP diagnosis. Negative DIF test findings in cases clinically suggestive of MMP should prompt repeat biopsies.
[Mh] Termos MeSH primário: Membrana Basal/patologia
Proteínas do Sistema Complemento/análise
Imunoglobulina A/análise
Imunoglobulina G/análise
Penfigoide Mucomembranoso Benigno/diagnóstico
Penfigoide Mucomembranoso Benigno/patologia
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Autoanticorpos/sangue
Autoantígenos/imunologia
Membrana Basal/química
Biópsia
Moléculas de Adesão Celular/imunologia
Feminino
Técnica Direta de Fluorescência para Anticorpo
Seres Humanos
Masculino
Meia-Idade
Colágenos não Fibrilares/imunologia
Penfigoide Mucomembranoso Benigno/sangue
Estudos Retrospectivos
Sensibilidade e Especificidade
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Autoantibodies); 0 (Autoantigens); 0 (Cell Adhesion Molecules); 0 (Immunoglobulin A); 0 (Immunoglobulin G); 0 (Non-Fibrillar Collagens); 0 (collagen type XVII); 0 (kalinin); 9007-36-7 (Complement System Proteins)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170927
[Lr] Data última revisão:
170927
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170706
[St] Status:MEDLINE


  6 / 1142 MEDLINE  
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[PMID]:28533859
[Au] Autor:Aounallah A; Jrad M; Ksiaa M; Mokni S; Saidi W; Boussofara L; Sriha B; Denguezli M; Ghariani N; Belajouza C; Nouira R
[Ad] Endereço:Université de Sousse, Tunisie.
[Ti] Título:[A particular type of cicatricial Pemphigoid with unique IgA deposit].
[Ti] Título:Forme particulière de Pemphigoide cicatricielle à dépôt unique d'IgA..
[So] Source:Pan Afr Med J;26:136, 2017.
[Is] ISSN:1937-8688
[Cp] País de publicação:Uganda
[La] Idioma:fre
[Ab] Resumo:Cicatricial Pemphigoid is a subepithelial bullous dermatosis which essentially involves the mucous membranes with cicatricial evolution We report the case of a 66-year old patient hospitalized with erosive gingivitis associated with dysphagia, dyspnea and blurred vision. Dermatologic examination showed erosive lesions involving the palate and the pharynx. Ophthalmologic examination showed symblepharons, ectropion and bilateral cataract. Gingival biopsy revealed a necrotic detachment of the buccal epithelium. Direct immunofluorescence showed linear IgA deposit at the dermo-epidermal junction. Indirect immunofluorescence test was negative. The diagnosis of cicatricial pemphigoid was confirmed. Esophagogastroduodenoscopy objectified double stenosis of the esophagus. Nasopharyngeal and bronchial endoscopy showed ulceration of the epiglottis, hypopharynx, pharynx and bronchial tree. The patient was treated with Solumedrol bolus corresponding to 0.5mg/kg/day prednisone associated with 100mg/day disulone. The patient showed a favorable early clinical outcome complicated because of the aggravation of dysphagia and esophageal stenosis after 2 months. Our case study is singular due to the occurrence of a cicatricial pemphigoid in a male patient with a serious clinical picture due to lesions extending to conjunctival, oral, nasal, esophageal and bronchial mucous membranes associated with direct immunofluorescence only showing IgA deposit.
[Mh] Termos MeSH primário: Dapsona/administração & dosagem
Imunoglobulina A/imunologia
Hemissuccinato de Metilprednisolona/administração & dosagem
Penfigoide Mucomembranoso Benigno/fisiopatologia
[Mh] Termos MeSH secundário: Idoso
Transtornos de Deglutição/etiologia
Endoscopia do Sistema Digestório
Estenose Esofágica/etiologia
Técnica Direta de Fluorescência para Anticorpo
Seres Humanos
Masculino
Penfigoide Mucomembranoso Benigno/diagnóstico
Penfigoide Mucomembranoso Benigno/imunologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Immunoglobulin A); 5GMR90S4KN (Methylprednisolone Hemisuccinate); 8W5C518302 (Dapsone)
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170613
[Lr] Data última revisão:
170613
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170524
[St] Status:MEDLINE
[do] DOI:10.11604/pamj.2017.26.136.9702


  7 / 1142 MEDLINE  
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[PMID]:28322362
[Au] Autor:Feller L; Ballyram R; Khammissa RA; Altini M; Lemmer J
[Ti] Título:Immunopathogenic Oral Diseases: An Overview Focusing on Pemphigus Vulgaris and Mucous Membrane Pemphigoid.
[So] Source:Oral Health Prev Dent;15(2):177-182, 2017.
[Is] ISSN:1602-1622
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Pemphigus vulgaris, mucosal pemphigoid (mucous membrane pemphigoid), lichen planus, discoid lupus erythematosus and erythema multiforme are a group of immune-mediated mucocutaneous disorders characterised clinically by the formation of blisters, erosions or ulcers. The oral mucosa is often affected, and sometimes the disease is limited to the mouth. The target antigens, autoreactive immune responses, microscopic features, treatment and prognosis vary from one disease to the other. Treatment aims to eliminate exogenous risk factors, suppress the pathogenic immuno-inflammatory reactions, promote healing and prevent infection. The aim of this article is to provide the general dental practitioner with a succinct overview of the diagnostic, clinical, aetiopathogenic features and characteristics of, as well as treatment guidelines for oral pemphigus vulgaris and oral mucosal pemphigoid. Early diagnosis and treatment could prevent severe consequences of the disease in their full-blown forms.
[Mh] Termos MeSH primário: Doenças da Boca/imunologia
Penfigoide Mucomembranoso Benigno/imunologia
Pênfigo/imunologia
[Mh] Termos MeSH secundário: Seres Humanos
Doenças da Boca/patologia
Penfigoide Mucomembranoso Benigno/patologia
Pênfigo/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171103
[Lr] Data última revisão:
171103
[Sb] Subgrupo de revista:D; IM
[Da] Data de entrada para processamento:170322
[St] Status:MEDLINE
[do] DOI:10.3290/j.ohpd.a37930


  8 / 1142 MEDLINE  
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[PMID]:28154932
[Au] Autor:You C; Lamba N; Lasave AF; Ma L; Diaz MH; Foster CS
[Ad] Endereço:Massachusetts Eye Research and Surgery Institution (MERSI), 1440 Main Street, Suite 201, Waltham, MA, 02451, USA.
[Ti] Título:Rituximab in the treatment of ocular cicatricial pemphigoid: a retrospective cohort study.
[So] Source:Graefes Arch Clin Exp Ophthalmol;255(6):1221-1228, 2017 Jun.
[Is] ISSN:1435-702X
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:PURPOSE: The purpose was to evaluate the effectiveness and safety of rituximab (RTX) for the treatment of patients with aggressive ocular cicatricial pemphigoid (OCP). METHODS: A review of patient records at a tertiary referral center with biopsy confirmed OCP who presented between 2006 and 2016. Sixty-one eyes of 32 patients with symptomatic OCP who received treatment with RTX monotherapy or RTX in combination with additional immunomodulatory treatment (IMT) were evaluated. Main outcomes included clinically evident remission of disease, the percentage of corticosteroid sparing patients, stage of OCP (Foster), best corrected visual acuity, and treatment complications. Remission was defined as absence of progressive scarring and active ocular inflammation for ≥ 2 months. Partial remission/responding was defined as disease control and clinical improvement for ≥ 2 months. RESULTS: Mean age at the initiation of RTX treatment was 59.1 years (range, 24-80 years) with a median follow-up time after RTX initiation of 32 months (range, 14 to 127 months). Twenty-six patients achieved clinical remission with an average sustained remission of 24.5 months (from 9 months to 84 months). RTX monotherapy was used in six patients, RTX in combination with intravenous immunoglobulin in 14 patients, and RTX with intravenous immunoglobulin and/or with other IMT agent in six patients. Seven eyes (11.5%) of six patients had favorable response to RTX and achieved response and partial remission, while inflammation remained active in the other seven eyes (11.5%) of four patients though there was no progressive scarring. At the last visit, three patients (9.4%) were on topical corticosteroid, three patients (9.4%) were treated with systemic corticosteroid treatments, and the other 26 patients (81.2%) achieved corticosteroid sparing therapy. Five eyes (8.2%) progressed one Foster stage. No other cicatrization progression or worsening of LogMAR visual acuity (p = 0.641) was observed during the follow-up period. Adverse events included leukopenia in three patients (9.4%), anemia in two patients (6.2%), liver enzyme elevation in three patients (9.4%) who were also on another concomitant IMT drug, and Epstein-Barr Virus infection and sinus infection in one patient each (3.1%). No other severe adverse events were noted during the follow-up period. CONCLUSIONS: These retrospective data suggest that RTX is efficacious and well tolerated when included for the treatment of OCP. Controlled studies are necessary to identify the role of this IMT agent in the therapeutic arsenal, especially its optimum dose and duration of administration.
[Mh] Termos MeSH primário: Túnica Conjuntiva/patologia
Penfigoide Mucomembranoso Benigno/tratamento farmacológico
Rituximab/administração & dosagem
Acuidade Visual
[Mh] Termos MeSH secundário: Adulto
Idoso
Idoso de 80 Anos ou mais
Biópsia
Relação Dose-Resposta a Droga
Feminino
Seguimentos
Seres Humanos
Fatores Imunológicos/administração & dosagem
Masculino
Meia-Idade
Penfigoide Mucomembranoso Benigno/diagnóstico
Estudos Retrospectivos
Fatores de Tempo
Resultado do Tratamento
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Immunologic Factors); 4F4X42SYQ6 (Rituximab)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:171005
[Lr] Data última revisão:
171005
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170204
[St] Status:MEDLINE
[do] DOI:10.1007/s00417-017-3603-3


  9 / 1142 MEDLINE  
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[PMID]:28106896
[Au] Autor:Dart JK
[Ad] Endereço:Ocular Biology and Therapeutics, UCL Institute of Ophthalmology, London, UK.
[Ti] Título:The 2016 Bowman Lecture Conjunctival curses: scarring conjunctivitis 30 years on.
[So] Source:Eye (Lond);31(2):301-332, 2017 Feb.
[Is] ISSN:1476-5454
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:This review is in two sections. The first section summarises 35 conditions, both common and infrequent, causing cicatrising conjunctivitis. Guidelines for making a diagnosis are given together with the use of diagnostic tests, including direct and indirect immunofluorescence, and their interpretation. The second section evaluates our knowledge of ocular mucous membrane pemphigoid, which is the commonest cause of cicatrizing conjunctivitis in most developed countries. The clinical characteristics, demographics, and clinical signs of the disease are described. This is followed by a review and re-evaluation of the pathogenesis of conjunctival inflammation in mucous membrane pemphigoid (MMP), resulting in a revised hypothesis of the autoimmune mechanisms causing inflammation in ocular MMP. The relationship between inflammation and scarring in MMP conjunctiva is described. Recent research, describing the role of aldehyde dehydrogenase (ALDH) and retinoic acid (RA) in both the initiation and perpetuation of profibrotic activity in MMP conjunctival fibroblasts is summarised and the potential for antifibrotic therapy, using ALDH inhibition, is discussed. The importance of the management of the ocular surface in MMP is briefly summarised. This is followed with the rationale for the use of systemic immunomodulatory therapy, currently the standard of care for patients with active ocular MMP. The evidence for the use of these drugs is summarised and guidelines given for their use. Finally, the areas for research and innovation in the next decade are reviewed including the need for better diagnostics, markers of disease activity, and the potential for biological and topical therapies for both inflammation and scarring.
[Mh] Termos MeSH primário: Conjuntivite
[Mh] Termos MeSH secundário: Autoanticorpos/análise
Doenças Autoimunes/diagnóstico
Doenças Autoimunes/etiologia
Doenças Autoimunes/imunologia
Doenças Autoimunes/terapia
Cicatriz/patologia
Conjuntivite/diagnóstico
Conjuntivite/imunologia
Conjuntivite/patologia
Conjuntivite/terapia
Fibroblastos/patologia
Técnica Indireta de Fluorescência para Anticorpo
Seres Humanos
Imunossupressores/uso terapêutico
Inflamação/imunologia
Isoenzimas/metabolismo
Penfigoide Mucomembranoso Benigno/diagnóstico
Penfigoide Mucomembranoso Benigno/imunologia
Penfigoide Mucomembranoso Benigno/terapia
Retinal Desidrogenase/metabolismo
Tretinoína/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Autoantibodies); 0 (Immunosuppressive Agents); 0 (Isoenzymes); 5688UTC01R (Tretinoin); EC 1.2.1.- (aldehyde dehydrogenase 1); EC 1.2.1.36 (Retinal Dehydrogenase)
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170718
[Lr] Data última revisão:
170718
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170121
[St] Status:MEDLINE
[do] DOI:10.1038/eye.2016.284


  10 / 1142 MEDLINE  
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[PMID]:28079687
[Au] Autor:Vahdani K; Thaller VT; Albanese G; Dean AF
[Ad] Endereço:*Oculoplastics Department, Royal Eye Infirmary, Derriford Hospital, Plymouth, United Kingdom; and †Histopathology Department, Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom.
[Ti] Título:Periocular Amyloidosis Manifesting as Pseudopemphigoid Treated With Mitomycin C.
[So] Source:Cornea;36(4):518-520, 2017 Apr.
[Is] ISSN:1536-4798
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: To describe an unusual cicatrizing manifestation of periocular amyloid and the utility of adjuvant antimetabolite therapy. METHODS: This is a case report of a 49-year old woman with progressive bilateral upper lid ptosis, who was found to have bilateral inferior forniceal masses with thickening of the conjunctiva. Conjunctival and eyelid biopsies showed evidence of amyloid deposition. Systemic evaluation did not reveal any evidence of systemic amyloidosis. Her blepharoptosis continued to deteriorate, and she underwent bilateral sequential upper lid ptosis correction surgery and debulking procedures. Subsequently, she developed severe and progressive cicatricial conjunctivitis with extensive symblepharon formation in all 4 fornices and restriction of ocular motility, simulating a clinical picture of ocular mucous membrane pemphigoid. RESULTS: She was treated surgically by division of symblepharon augmented with application of topical mitomycin C intraoperatively. This has been partially successful in freeing up differential movement between her lid and her eye and consequently improving eye protection and lubrication. CONCLUSIONS: Pseudopemphigoid represents a rare manifestation of periocular amyloid. Risks and benefits of surgical procedures in this context need to be carefully weighed to minimize the risk of secondary complications. Topical mitomycin C may be considered as a relatively safe adjunct in managing the cicatricial component of the disease.
[Mh] Termos MeSH primário: Alquilantes/uso terapêutico
Amiloidose/diagnóstico
Doenças da Túnica Conjuntiva/diagnóstico
Doenças Palpebrais/diagnóstico
Mitomicina/uso terapêutico
Penfigoide Mucomembranoso Benigno/diagnóstico
[Mh] Termos MeSH secundário: Amiloidose/tratamento farmacológico
Doenças da Túnica Conjuntiva/tratamento farmacológico
Doenças Palpebrais/tratamento farmacológico
Feminino
Seres Humanos
Meia-Idade
Penfigoide Mucomembranoso Benigno/tratamento farmacológico
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Alkylating Agents); 50SG953SK6 (Mitomycin)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170922
[Lr] Data última revisão:
170922
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170113
[St] Status:MEDLINE
[do] DOI:10.1097/ICO.0000000000001138



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