A Rare Case Report of Extensive Polyostotic Gorham's Disappearing Bone Disease Involving the Upper Extremity

Elke R Ahlmann,Yanling Ma,Vonny Tunru-Dinh

doi:10.1155/2011/486756

Abstract

Gorham's disease is a rare disorder involving the proliferation of endothelial channels resulting in resorption and disappearance of bone. An unusual case of polyostotic Gorham's disease affecting the scapula, humerus, radius, and ulna in a 39-year-old woman is described. The patient had extensive disease spreading across both the glenohumeral and humeroulnar joints. This is the first report of Gorham's disease spreading across multiple joints in the upper extremity.

Highlights

Massive spontaneous osteolysis of bone was first described by Gorham et al [1] in 1954 and later Gorham and Stout [2] in 1955 in which the first 24 cases of this disease were presented
Multiple names have been given to this condition including vanishing bone disease, phantom bone, hemangiomatosis, lymphangiomatosis, and GorhamStout syndrome, but it is best known as Gorham’s disease [3]
It is a very rare disorder characterized by abnormal proliferation of thin-walled endothelial-lined channels of vascular or lymphatic origin and an increased number of osteoclasts resulting in progressive resorption of bone [2]

Summary

Introduction

Massive spontaneous osteolysis of bone was first described by Gorham et al [1] in 1954 and later Gorham and Stout [2] in 1955 in which the first 24 cases of this disease were presented. Multiple names have been given to this condition including vanishing bone disease, phantom bone, hemangiomatosis, lymphangiomatosis, and GorhamStout syndrome, but it is best known as Gorham’s disease [3]. It is a very rare disorder characterized by abnormal proliferation of thin-walled endothelial-lined channels of vascular or lymphatic origin and an increased number of osteoclasts resulting in progressive resorption of bone [2]. This paper presents an unusual case of polyostotic Gorham’s disease affecting the scapula, humerus, radius, and ulna and, to our knowledge, is the first report of this disease spreading across multiple joints in an adult

Case Presentation

Findings

Discussion