Progressive pseudorheumatoid arthropathy of childhood (Q88362): Difference between revisions
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A artropatia (displasia) progressiva pseudo-reumatoide da infância (PPD) se apresenta como displasia espondiloepifisária (SED) tardia com artropatia progressiva e é descrita como um subtipo autossômico recessivo específico de SED. Tipicamente, não há sintomas no período neonatal. Os sinais mais comuns no momento do diagnóstico são arqueamento das pernas, fraqueza muscular, edema simétrico das articulações interfalangianas proximais. Limitação da amplitude de movimento, deformidades e dor se desenvolvem gradualmente e se espalham para outras articulações. Muitos pacientes têm envolvimento da coluna vertebral manifestando-se como lordose lombar exagerada, cifose torácica e/ou escoliose. | |||
| description / en | description / en | ||
Progressive pseudorheumatoid arthropathy (dysplasia) of childhood (PPAC; PPD) presents as spondyloepiphyseal dysplasia (SED) tarda with progressive arthropathy and is described as a specific autosomal recessive subtype of SED. Typically, there are no symptoms in the newborn period. The most common signs at diagnosis are bowing of the legs, muscle weakness, symmetric swelling of the proximal interphalangeal joints. Motion range limitation, deformities and pain develop gradually, and spread to other joints. Many patients have spinal involvement manifesting as exaggerated lumbar lordosis, thoracic kyphosis, and/or scoliosis. | |||
Revision as of 14:33, 15 August 2026
Progressive pseudorheumatoid arthropathy (dysplasia) of childhood (PPAC; PPD) presents as spondyloepiphyseal dysplasia (SED) tarda with progressive arthropathy and is described as a specific autosomal recessive subtype of SED. Typically, there are no symptoms in the newborn period. The most common signs at diagnosis are bowing of the legs, muscle weakness, symmetric swelling of the proximal interphalangeal joints. Motion range limitation, deformities and pain develop gradually, and spread to other joints. Many patients have spinal involvement manifesting as exaggerated lumbar lordosis, thoracic kyphosis, and/or scoliosis.
| Language | Label | Description | Also known as |
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| default for all languages | ID_280808713 |
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| English | Progressive pseudorheumatoid arthropathy of childhood |
Progressive pseudorheumatoid arthropathy (dysplasia) of childhood (PPAC; PPD) presents as spondyloepiphyseal dysplasia (SED) tarda with progressive arthropathy and is described as a specific autosomal recessive subtype of SED. Typically, there are no symptoms in the newborn period. The most common signs at diagnosis are bowing of the legs, muscle weakness, symmetric swelling of the proximal interphalangeal joints. Motion range limitation, deformities and pain develop gradually, and spread to other joints. Many patients have spinal involvement manifesting as exaggerated lumbar lordosis, thoracic kyphosis, and/or scoliosis. |
