Results 21 to 30 of about 214 (125)
Abstract BACKGROUND & AIMS Primary sclerosing cholangitis (PSC) is a rare bile duct disease strongly associated with inflammatory bowel disease (IBD). Whole‐exome sequencing (WES) has contributed to understanding the molecular basis of very early‐onset IBD, but rare protein‐altering genetic variants have not been identified for early‐onset PSC.
Sjoukje‐Marije Haisma +19 more
wiley +1 more source
Case for diagnosis. Lichen myxedematosus [PDF]
Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal gammopathy and may show extracutaneous manifestations, affecting the heart, lung, kidney,
Priscila Regina Orso Rebellato +3 more
doaj +1 more source
Acral papular mucinosis: a new case of this rare entity [PDF]
Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus. It consists of small papules localized exclusively on the back of the hands, wrists and extensor aspects of distal forearms with no other clinical or ...
María Encarnación Gómez Sánchez +4 more
doaj +1 more source
Summary Scleromyxedema is a rare, cutaneous deposition disorder from the group of mucinoses, which can affect multiple organs and is virtually always associated with a monoclonal gammopathy. Cutaneous manifestations are usually generalized, 2 to 3 mm sized, dome‐shaped or flat‐topped, waxy, slightly red to skin‐colored papules and sclerodermoid ...
Jochen H. O. Hoffmann, Alexander H. Enk
wiley +1 more source
Escleromixedema associado a miocardiopatia Scleromyxedema with associated cardiomyopathy
Relato de um caso de escleromixedema com lesões cutâneas associadas a miopatia, disfunção esofageana e paraproteinemia. Durante a evolução, a paciente apresentou sintomas de insuficiência cardíaca congestiva (ICC) que foram relacionados à miocardiopatia,
Gladys Aires Martins +2 more
doaj +1 more source
A mucinose follicular (MuF), também chamada de alopecia mucinosa, é mucinose cutânea caracterizada pelo acúmulo de mucina no folículo pilossebáceo. As mucinoses têm duas formas de apresentação: uma idiopática ou primária, e outra sintomática ou associada
Antônio de Pádua Marques da Fonseca +4 more
doaj +1 more source
Pretibial mucinosis in an euthyroid patient [PDF]
A case of exuberant pretibial mucinosis in a patient with normal thyroid function is reported. A review of literature on possible etiologies other than thyroid disease for the accumulation of mucin in the pretibial area is presented.
Laura de Mattos Milman +4 more
doaj +2 more sources
Scleredema associated with Sjögren’s syndrome [PDF]
Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk.
João Alves +4 more
doaj +2 more sources
Caso para diagnóstico Case for diagnosis
É relatado um caso de nevo mucinoso em adolescente do sexo masculino que apresentava lesões papulosas acastanhadas assintomáticas, confluentes em placas de distribuição linear no quadril esquerdo, surgidas aos 14 anos de idade.
Ana Luiza Baeta Pereira Barbosa +2 more
doaj +1 more source
Escleromixedema: um caso tratado com prednisona oral [PDF]
O escleromixedema é uma mucinose cutânea idiopática caracterizada por erupção papulosa, induração da pele e paraproteinemia. Histologicamente, se observa proliferação de fibroblastos na derme superior associada a depósito de mucina.
Pedro Bezerra da Trindade Neto +3 more
doaj +3 more sources

