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KMID : 0363220160540050368
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Korean Journal of Dermatology
2016 Volume.54 No. 5 p.368 ~ p.371
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A Case of Gorham-Stout Disease with Progressive Cutaneous Involvement
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Kim Tae-In
Jeong Ki-Heon
Shin Min-Kyung
Kim Nack-In
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Abstract
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Gorham-Stout disease is a rare bone disorder that is characterized by angiomatous proliferation and results in destruction and resorption of the osseous matrix. It has variable presentations and a number of different sites may be affected, including the dermis, soft tissue, and viscera. The involved cutaneous and soft tissue lesions typically present with sponge-like, soft, brown or purple lesions. The clinical course is generally prolonged, with eventual stabilization of the affected bone; however, it is rarely fatal. There is no standard treatment defined for this disease, and variable therapies such as medical, surgical, and radiation therapy have been used. We report a case of 24-year-old man with Gorham disease who presented with progressive cutaneous lesions and severe lymphedema adjacent to the diseased bone.
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KEYWORD
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Gorham-Stout disease, Lymphangiomatosis
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