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

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Abstract

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.

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Authors and Affiliations

  1. Division de Cirugia Maxilofacial, Hospital Universitario de Puebla, Benemerita Universidad de Puebla, Puebla, Puebla, Mexico

    Maria José Franco-Barrera

  2. Programa Internacional de Medicina, Universidad Autónoma de Guadalajara, Guadalajara, Jalisco, Mexico

    Maria Guadalupe Zavala-Cerna, Georgina Aguilar-Portillo, Diana Brisa Sánchez-Gomez & Olivia Torres-Bugarin

  3. Facultad de Medicina, Universidad Autonoma de Guadalajara, Guadalajara, Jalisco, Mexico

    Miguel Angel Franco-Barrera

  4. Division de Cirugia Maxilofacial, Unidad de Alta Especialidad de Pediatria, Centro Medico Nacional de Occidente, Instituto Mexicano del Seguro Social, Guadalajara, Jalisco, Mexico

    Carlos Manuel Roa-Encarnacion

Authors
  1. Maria José Franco-Barrera
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  2. Maria Guadalupe Zavala-Cerna
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  3. Georgina Aguilar-Portillo
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  4. Diana Brisa Sánchez-Gomez
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  5. Olivia Torres-Bugarin
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  6. Miguel Angel Franco-Barrera
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  7. Carlos Manuel Roa-Encarnacion
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Corresponding author

Correspondence to Maria Guadalupe Zavala-Cerna.

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The authors declare that they have no conflict of interest.

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This article does not contain any studies with human participants or animals by any of the authors.

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Franco-Barrera, M.J., Zavala-Cerna, M.G., Aguilar-Portillo, G. et al. Gorham-Stout Disease: a Clinical Case Report and Immunological Mechanisms in Bone Erosion. Clinic Rev Allerg Immunol 52, 125–132 (2017). https://doi.org/10.1007/s12016-016-8594-z

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  • DOI: https://doi.org/10.1007/s12016-016-8594-z

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