Table 2.

Classification of “Idiopathic osteolysis” according to Hardegger et al. [5]. Patients with Gorham-Stout syndrome do not show renal involvement or systemic symptoms.

Type		Typical age of manifestation	Location of manifestation	Nephropathy	Prognosis
1	Hereditary multicentric osteolysis with dominant inheritance	Juvenile	Carpotarsal osteolysis, less often with affection of the radius and ulna	No	Good, disease arrest in adolescence
2	Hereditary multicentric osteolysis with recessive transmission	Juvenile	According to type 1, in addition severe generalized osteoporosis	No	Good, disease arrest in adolescence
3	Non hereditary multicentric osteolysis with nephropathy	Juvenile	Mainly carpometacarpal, rare tarsal affection, malignant hypertension	Yes, proteinuria in progressive renal pathology	Often unfavourable
4	Gorham-Stout syndrome	Age-independent	Typical: shoulder girdle, pelvis, facial skull	No	Mostly good. When in spine or chylothorax: lethality up to 50%
5	Winchester syndrome (hereditary, autosomal recessive)	Juvenile	Carpotarsal osteolysis and contractures, short stature, osteoporosis, corneal deterioration	No	Progressive