Table 1.
Type | Typical age at manifestation | Location of the manifestation | Nephropathy | Prognosis |
---|---|---|---|---|
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 |