A difficult diagnosis 

There is an useful list of factors to help distinguish Gorham disease from other destructive bone disorders; they include a biopsy sample indicating the presence of lymphatic tissue (D2-40 positivity on inmunohistochemistry); absence of cellular atypia; little or no osteoblastic response or dystrophic calcification; local progressive osseous resorption; a nonexpansile, nonulcerative lesion; an osteolytic radiographic pattern; and no hereditary, metabolic, neoplastic, immunological, or infectious cause. The diagnosis is usually made on the basis of the characteristic history of osteolysis and failure of bone healing in conjunction with the histological findings of marrow fibrosis and increased vascularity. This bone disorder is largely attributed to focal lymphatic endothelial proliferation, whereby the bone is progressively destroyed and resorbed. The expected course of events after a fracture in a bone affected by Gorhams Disease is failure of healing followed by progressive reabsorption of bone around the fracture site. Progression of the disease has been observed to cease without treatment in many cases, but regeneration of the destroyed bone is not expected. The self-limited course correlates well with two phases of evolution of the histopathological lesions with neoplastic-like proliferation of lymphatic endothelial cells corresponding to the rapid and massive bone destruction, and a later differentiation of the cells in mature vascular structures, but still with accelerated bone resorption which is partly compensated by appositional activity.

The criteria for what is and is not Gorham's Disease are not well worked out. The most easily separable group is that of multicentric osteolysis of which the great majority primarily affect the hands and feet, so-called acro-osteolysis. A subset has been described which is clearly inherited and two syndromes are recognised. Yet another group of multicentric osteolysis patients has an associated nephropathy.