Osteopathia striata, also known as Voorhoeve disease, is a rare, benign sclerosing bone dysplasia, involving the epiphysis and metaphysis of tubular bones.
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Epidemiology
Osteopathia striata can be found in any age group.
Associations
There is an association with focal dermal hypoplasia (Goltz syndrome).
Clinical presentation
It is typically asymptomatic, although there can be associated joint discomfort.
Pathology
Variants
Radiographic features
Osteopathia striata is typically bilateral, although occasionally it can be unilateral, typically in tubular bones.
Radiographically prominent vertical striations predominate in the metaphyses and epiphyses of the long bones (celery stalk metaphysis).
Not infrequently there are shared features of melorheostosis and osteopoikilosis, in a so-called overlap syndrome termed mixed sclerosing bone dysplasia. These conditions may share an underlying etiology (loss of function mutations in the LEMD3 gene) 5.
Differential diagnosis
The differential diagnosis includes:
normal variation in the prominence of periarticular vertical trabeculation
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