Osteopathia striata

Osteopathia striata, also known as Voorhoeve disease, is a rare, benign sclerosing bone dysplasia, involving the epiphysis and metaphysis of tubular bones.

Epidemiology

Oseopathia striata can be found in any age group.

Clinical presentation

It is typically asymptomatic, although there can be associated joint discomfort.

Pathology

Associations

There is an association with focal dermal hypoplasia (Goltz syndrome).

Variants

• osteopathia striata with cranial sclerosis 

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) .

Differential diagnosis

The differential diagnosis includes:

• normal variation in the prominence of periarticular vertical trabeculation
• adult osteopetrosis
• enchondromatosis
• osteopoikilosis

Siehe auch:

• Osteopoikilose
• celery stalk metaphysis
• Ollier-Syndrom
• Melorheostose
• Goltz-Gorlin-Syndrom
• adult osteopetrosis

und weiter:

• diffuse bony sclerosis (mnemonic)
• vegetable and plant inspired signs
• conditions involving skin and bone
• osteoid lesions
• diffuse skelettale Sklerosierung
• sklerosierende Knochendysplasien