Annular Elastolytic Giant Cell Granuloma Associated with Polycythemia Vera

• Autor(a) principal: Albino, Cátia
• Data de Publicação: 2016
• Outros Autores: Machado e Costa, Carlos, Neves, Manuel, Soares-de-Almeida, Luis, Braz Nogueira, J.
• Tipo de documento: Artigo
• Idioma: por
• Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
• Texto Completo: https://doi.org/10.29021/spdv.74.1.519
• topic: Granuloma; Giant Cell; Polycythemia Vera; Granuloma de Células Gigantes; Policitemia Vera

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.