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A Case of Secondary Erythromelalgia Associated with Polycythemia Vera

Sang Jai Jang, Hyun Soo Park, Yong Suk Lee, Duk Kyu Chun

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Abstract

Erythromelalgia(EM) is a rare clinical syndrome characterized by the triad of redness, increased temperature, and pain of the extremities. The patient was a 69-year-old woman presented erythematous tender patches on both palms and soles. Laboratory and clinical investigations revealed an underlying myeloproliferative disorder. Histologic examination of the erythema on her foot showed some vessels with thickened endothelial walls and partially obstructed lumens by organized thrombi. Hydroxyurea used for the treatment of polycythemia vera eliminated the skin lesions and symptoms of erythromelalgia. We report a case of secondary erythromelalgia associated with polycythemia vera.

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What this paper is about

Erythromelalgia(EM) is a rare clinical syndrome characterized by the triad of redness, increased temperature, and pain of the extremities. The patient was a 69-year-old woman presented erythematous tender patches on both palms and soles. Laboratory and clinical investigations revealed an underlying myeloproliferative disorder. Histologic examination of the erythema on her foot showed some vessels with thickened endothelial walls and partially obstructed lumens by organized thrombi. Hydroxyurea used for the treatment of polycythemia vera eliminated the skin lesions and symptoms of erythromelalgia. We report a case of secondary erythromelalgia associated with polycythemia vera.

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Available abstract

Erythromelalgia(EM) is a rare clinical syndrome characterized by the triad of redness, increased temperature, and pain of the extremities. The patient was a 69-year-old woman presented erythematous tender patches on both palms and soles. Laboratory and clinical investigations revealed an underlying myeloproliferative disorder. Histologic examination of the erythema on her foot showed some vessels with thickened endothelial walls and partially obstructed lumens by organized thrombi. Hydroxyurea used for the treatment of polycythemia vera eliminated the skin lesions and symptoms of erythromelalgia. We report a case of secondary erythromelalgia associated with polycythemia vera.

Key concepts: Erythromelalgia, Polycythemia vera, Medicine, Erythema, Dermatology, Myeloproliferative Disorders, Polycythemia rubra vera, Pathology

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