Gorham-Stout Disease: a Clinical Case Report and Immunological Mechanisms in Bone Erosion

Gorham-Stout disease (GSD) is a rare condition of osteolysis with excessive lymphangiogenesis within bone tissue. The etiology of this condition remains unknown but seems to affect mainly children and young adults of both genders all over the world. Unfortunately, there is no standardized method for...

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Bibliographic Details
Published in:Clinical reviews in allergy & immunology 2017-02, Vol.52 (1), p.125-132
Main Authors: Franco-Barrera, Maria José, Zavala-Cerna, Maria Guadalupe, Aguilar-Portillo, Georgina, Sánchez-Gomez, Diana Brisa, Torres-Bugarin, Olivia, Franco-Barrera, Miguel Angel, Roa-Encarnacion, Carlos Manuel
Format: Article
Language:English
Subjects:
Allergology
Child
Dendritic cells
Female
Health aspects
Histochemistry
Humans
Immunology
Internal Medicine
Mandible - abnormalities
Mastication
Medicine
Medicine & Public Health
Osteolysis, Essential - diagnosis
Osteolysis, Essential - pathology
T cells
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Summary:Gorham-Stout disease (GSD) is a rare condition of osteolysis with excessive lymphangiogenesis within bone tissue. The etiology of this condition remains unknown but seems to affect mainly children and young adults of both genders all over the world. Unfortunately, there is no standardized method for diagnosis; however, histopathology remains as the gold standard. This condition is often misdiagnosed due to its varying clinical presentations from case-to-case. Here, we report the case of an 8-year-old girl who presented with chronic mandibular pain during mastication and received multiple antibiotic treatment due to infectious origin suspicion. After integrating information from clinical manifestations, radiographic, laboratory, and histopathology information, she was diagnosed with GSD. Additionally, due to the lack of literature with respect to insights into biological mechanisms and standardized treatment for this condition, we underwent a literature revision to provide information related to activation of cells from the immune system, such as macrophages, T-cells, and dendritic cells, and their contribution to the lymphangiogenesis, angiogenesis, and osteoclastogenic process in GSD. It is important to consider these mechanisms in patients with GSD, especially since new studies performed in earlier stages are required to confirm their use as novel diagnostic tools and find new possibilities for treatment.
ISSN:1080-0549
1559-0267
DOI:10.1007/s12016-016-8594-z