A Case of Gorham-Stout Disease with Progressive Cutaneous Involvement.
- Author:
Taein KIM
1
;
Ki Heon JEONG
;
Min Kyung SHIN
;
Nack In KIM
Author Information
1. Department of Dermatology, School of Medicine, Kyung Hee University, Seoul, Korea. mdfamily@hanmail.net
- Publication Type:Case Report
- Keywords:
Gorham-Stout disease;
Lymphangiomatosis
- MeSH:
Dermis;
Humans;
Lymphedema;
Osteolysis, Essential*;
Viscera;
Young Adult
- From:Korean Journal of Dermatology
2016;54(5):368-371
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
