Osteopoikilosis

Osteopoikilosis of the skeleton of the hand.

Osteopoikilie Huefte CT

Osteopoikilie Fuss - 48jm - Roe 2Eb - 001

Osteopoicilosis (osteopoicilia) is a very rare AD hereditary disease.

It is manifested by irregular thickenings of the bone → we find round or oval islands hyperostosis inside the cancellous Bones and the metaphysis and epiphysis.

Clinical picture

• Usually without significant clinical manifestations;
• the disease is mostly revealed as an accidental X-ray finding;
• in 10% affected subcutaneous and skin nodules (dermatofibrosis lenticularis disseminata - Bloch-Sulzberg syndrome).

X-ray image

• Irregularly demarcated sclerosis → characteristic mottled appearance of the skeleton.

Treatment

• None (illness without subjective difficulties);
• in adults, bone metastases must be distinguished.

References

DUNGL, Pavel, et al. Ortopedie. 1. edition. Praha : Grada Publishing, 2005. ISBN 80-247-0550-8.