Annular Elastolytic Giant Cell Granuloma Associated with Polycythemia Vera

  • Cátia Albino Interna do Internato Complementar de Medicina Interna/Resident, Internal Medicine, Serviço de Medicina 1, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
  • Carlos Machado e Costa Assistente Hospitalar Graduado de Medicina Interna/Consultant Chief, Internal Medicine, Serviço de Medicina 1, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
  • Manuel Neves Assistente Hospitalar de Hematologia/Consultant, Hematology, Serviço de Hematologia, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
  • Luis Soares-de-Almeida Professor Auxiliar de Dermatologia/Professor of Dermatology, Faculdade de Medicina da Universidade de Lisboa, Lisboa, Portugal
  • J. Braz Nogueira Diretor do Serviço de Medicina 1/Head of Internal Medicine Department, Centro Hospitalar Lisboa Norte, Hospital de Santa Maria; Professor Catedrático/Professor, Faculdade de Medicina da Universidade de Lisboa, Lisboa, Portugal
Keywords: Granuloma, Giant Cell, Polycythemia Vera

Abstract

Introduction: The authors describe a case of annular elastolytic giant cell granuloma (AEGCG) associated with polycythaemia vera. Both diseases resolved with treatment with hydroxyurea.

Case report: A 63-year-old man refers fatigue, nausea, right upper abdominal pain and marked weight loss for 4 months. During last month he presents multiple grouped erythematous skin papules and plaques in his back. He had a moderate increase of his spleen. The patient was diagnosed with polycythaemia vera (JAK 2 mutation positive in bone marrow). Skin biopsy revealed AEGCG. The patient was treated with hydroxyurea; there was normalization of hematologic changes and regression of skin lesions.

Discussion: AECG is, in most cases, an isolated cutaneous disease but it can be found in association with other systemic diseases. Our patient is the first described with and association of AEGCG and polycythaemia vera. The regression of both diseases with hydroxyurea suggests a possible relationship.

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Published
2016-04-29
How to Cite
Albino, C., Machado e Costa, C., Neves, M., Soares-de-Almeida, L., & Braz Nogueira, J. (2016). Annular Elastolytic Giant Cell Granuloma Associated with Polycythemia Vera. Journal of the Portuguese Society of Dermatology and Venereology, 74(1), 73-77. https://doi.org/10.29021/spdv.74.1.519
Section
Case Reports