Table 1 Classification of “idiopathic osteolysis” according to Hardegger et al

I

Hereditary multicentric osteolysis with dominant inheritance

Juvenile

Carpotarsal osteolysis, sometimes affecting the radius and ulna

No

Good, self-limiting in adolescence

II

Hereditary multicentric osteolysis with recessive transmission

Juvenile

Consistent with type I, in addition to generalized osteoporosis

No

Good, self-limiting in adolescence

III

Nonhereditary multicentric osteolysis with nephropathy

Juvenile

Mainly carpometacarpal, tarsal involvement is rare, malignant hypertension

Yes, proteinuria in progressive renal pathology

Unfavorable

IV

Gorham-Stout syndrome

Independent of age

Typical: shoulder, pelvis, facial skull bones

No

Usually good. When there is spinal involvement or chylothorax, mortality rises more than 50%

V

Winchester syndrome (hereditary, autosomal recessive)

Juvenile

Carpotarsal osteolysis and contractures, short stature, osteoporosis, corneal deterioration

No

Progressive