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The Red Scalp Sign
252
Letters to the Editor
Mayo Clin Proc, February 2003, Vol 78
Letters to the Editor chemia, confirmed with coronary angiography. The patient underwent successful 2-vessel coronary artery bypass grafting. Postoperatively, the patient’s scalp erythema resolved (hematocrit range, 24%-45%), and with subsequent hydroxyurea therapy and intermittent phlebotomy, has remained absent; his hemoglobin level has stayed within the normal range. Subtle physical findings may be helpful, when used in conjunction with historical and laboratory data, in leading to a specific diagnosis. The patient, his spouse, and friends noted the presence of scalp redness for several months. However, despite the persistence of this finding and epistaxis, a blood cell count was not obtained until the patient presented with syncope. The fact that the scalp erythema disappeared as the hematocrit decreased implicates erythrocytosis as the etiology of the red scalp. We propose that the red scalp sign be considered a clue to underlying erythrocytosis and, in particular, to polycythemia vera and that clinicians check the hematocrit level of such patients.
The Red Scalp Sign To the Editor: Polycythemia vera is a chronic myeloproliferative disorder characterized predominantly by erythrocytosis and occasional thrombotic events. Peculiar manifestations suggestive of polycythemia vera include aquagenic pruritus, erythromelalgia, and acute gouty arthritis.1,2 Patients with polycythemia vera commonly exhibit a ruddy facial complexion and conjunctival injection. However, we recently diagnosed polycythemia vera in a patient with considerable scalp erythema, a sign not noted in major internal medical texts3,4 or review articles.5 We named this previously unreported finding the red scalp sign because it may be a useful clue to the presence of polycythemia vera. A previously healthy 75-year-old man presented to the emergency department after a syncopal episode that occurred while he was square-dancing. He denied chest pain, dyspnea, palpitations, diaphoresis, dizziness, or vertigo. Review of his medical history revealed only episodic mild epistaxis over the previous several months. Pulse oximetry was normal. Physical examination findings were unremarkable except for a ruddy facial complexion, conjunctival injection, and diffuse erythema of the scalp (Figure 1). The patient’s wife noted that the scalp redness was present for months; it was originally attributed to sunburn that happened when the patient mowed the grass the previous summer. Admission laboratory studies revealed the following: hemoglobin, 21.9 g/dL; hematocrit, 67%; leukocyte count, 11 × 109/L; and platelet count, 482 × 109/ L. Routine serum chemistries were normal. Electrocardiography revealed T-wave inversion over the precordium, but cardiac enzymes were normal. A chromium 51 tagged red cell scan was consistent with severe polycythemia. The patient underwent phlebotomy, and 5 U of blood was removed over the next 3 days with no further symptoms. The erythropoietin level was 5.4 mU/mL (reference range, 4.1-19.5 mU/mL). A treadmill thallium stress test revealed left ventricular is-
Mark A. Marinella, MD Wright State University School of Medicine Dayton, Ohio 1. 2. 3.
4.
5.
Marinella MA. Recognizing Clinical Patterns: Clues to a Timely Diagnosis. Philadelphia, Pa: Hanley & Belfus Inc; 2002:167-168. Marinella MA. Pocket Brain of 50 Unusual Symptoms. Malden, Mass: Blackwell Science Publishers; 2002. Spivak JL. Polycythemia vera and other myeloproliferative diseases. In: Braunwald E, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL, eds. Harrison’s Principles of Internal Medicine. 15th ed. New York, NY: McGraw-Hill; 2002:701-706. Tefferi A, Silverstein MN. Myeloproliferative diseases. In: Goldman L, Bennett JC, eds. Cecil Textbook of Medicine. 21st ed. Philadelphia, Pa: WB Saunders Co; 2000:935-941. Tefferi A. Diagnosing polycythemia vera: a paradigm shift. Mayo Clin Proc. 1999;74:159-162.
Invasive Infections Due to Apophysomyces elegans To the Editor: We read with great interest the review by Blair et al1 of reported cases of invasive infections due to the fungus Apophysomyces elegans. These investigators observed that this fungus exhibits unique features compared with other members of the Mucoraceae family. In contrast with other members of the order Mucorales, such as species of the genera Rhizopus, Rhizomucor, and Absidia, patients infected by A elegans are often immunocompetent. Among the 25 cases reviewed by Blair et al,116 patients (64%) had no known underlying disease, suggesting that A elegans is more virulent to humans than to other Zygomycetes. Recently, we established a nonneutropenic murine model in which we compared the virulence of A elegans with that of Absidia corymbifera and Rhizopus microsporus.2 Although we were able to cause acute and lethal infections in mice by intravenous injection of fungal spores, the inoculum that corresponded to the 90% lethal dose was considerably different
Figure 1. The red scalp sign in a patient with polycythemia vera. Restoration of a normal hematocrit resulted in resolution of the erythema. Mayo Clin Proc. 2003;78:252-253
252
© 2003 Mayo Foundation for Medical Education and Research
For personal use. Mass reproduce only with permission from Mayo Clinic Proceedings.
Letters to the Editor
Mayo Clin Proc, February 2003, Vol 78
Letters to the Editor chemia, confirmed with coronary angiography. The patient underwent successful 2-vessel coronary artery bypass grafting. Postoperatively, the patient’s scalp erythema resolved (hematocrit range, 24%-45%), and with subsequent hydroxyurea therapy and intermittent phlebotomy, has remained absent; his hemoglobin level has stayed within the normal range. Subtle physical findings may be helpful, when used in conjunction with historical and laboratory data, in leading to a specific diagnosis. The patient, his spouse, and friends noted the presence of scalp redness for several months. However, despite the persistence of this finding and epistaxis, a blood cell count was not obtained until the patient presented with syncope. The fact that the scalp erythema disappeared as the hematocrit decreased implicates erythrocytosis as the etiology of the red scalp. We propose that the red scalp sign be considered a clue to underlying erythrocytosis and, in particular, to polycythemia vera and that clinicians check the hematocrit level of such patients.
The Red Scalp Sign To the Editor: Polycythemia vera is a chronic myeloproliferative disorder characterized predominantly by erythrocytosis and occasional thrombotic events. Peculiar manifestations suggestive of polycythemia vera include aquagenic pruritus, erythromelalgia, and acute gouty arthritis.1,2 Patients with polycythemia vera commonly exhibit a ruddy facial complexion and conjunctival injection. However, we recently diagnosed polycythemia vera in a patient with considerable scalp erythema, a sign not noted in major internal medical texts3,4 or review articles.5 We named this previously unreported finding the red scalp sign because it may be a useful clue to the presence of polycythemia vera. A previously healthy 75-year-old man presented to the emergency department after a syncopal episode that occurred while he was square-dancing. He denied chest pain, dyspnea, palpitations, diaphoresis, dizziness, or vertigo. Review of his medical history revealed only episodic mild epistaxis over the previous several months. Pulse oximetry was normal. Physical examination findings were unremarkable except for a ruddy facial complexion, conjunctival injection, and diffuse erythema of the scalp (Figure 1). The patient’s wife noted that the scalp redness was present for months; it was originally attributed to sunburn that happened when the patient mowed the grass the previous summer. Admission laboratory studies revealed the following: hemoglobin, 21.9 g/dL; hematocrit, 67%; leukocyte count, 11 × 109/L; and platelet count, 482 × 109/ L. Routine serum chemistries were normal. Electrocardiography revealed T-wave inversion over the precordium, but cardiac enzymes were normal. A chromium 51 tagged red cell scan was consistent with severe polycythemia. The patient underwent phlebotomy, and 5 U of blood was removed over the next 3 days with no further symptoms. The erythropoietin level was 5.4 mU/mL (reference range, 4.1-19.5 mU/mL). A treadmill thallium stress test revealed left ventricular is-
Mark A. Marinella, MD Wright State University School of Medicine Dayton, Ohio 1. 2. 3.
4.
5.
Marinella MA. Recognizing Clinical Patterns: Clues to a Timely Diagnosis. Philadelphia, Pa: Hanley & Belfus Inc; 2002:167-168. Marinella MA. Pocket Brain of 50 Unusual Symptoms. Malden, Mass: Blackwell Science Publishers; 2002. Spivak JL. Polycythemia vera and other myeloproliferative diseases. In: Braunwald E, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL, eds. Harrison’s Principles of Internal Medicine. 15th ed. New York, NY: McGraw-Hill; 2002:701-706. Tefferi A, Silverstein MN. Myeloproliferative diseases. In: Goldman L, Bennett JC, eds. Cecil Textbook of Medicine. 21st ed. Philadelphia, Pa: WB Saunders Co; 2000:935-941. Tefferi A. Diagnosing polycythemia vera: a paradigm shift. Mayo Clin Proc. 1999;74:159-162.
Invasive Infections Due to Apophysomyces elegans To the Editor: We read with great interest the review by Blair et al1 of reported cases of invasive infections due to the fungus Apophysomyces elegans. These investigators observed that this fungus exhibits unique features compared with other members of the Mucoraceae family. In contrast with other members of the order Mucorales, such as species of the genera Rhizopus, Rhizomucor, and Absidia, patients infected by A elegans are often immunocompetent. Among the 25 cases reviewed by Blair et al,116 patients (64%) had no known underlying disease, suggesting that A elegans is more virulent to humans than to other Zygomycetes. Recently, we established a nonneutropenic murine model in which we compared the virulence of A elegans with that of Absidia corymbifera and Rhizopus microsporus.2 Although we were able to cause acute and lethal infections in mice by intravenous injection of fungal spores, the inoculum that corresponded to the 90% lethal dose was considerably different
Figure 1. The red scalp sign in a patient with polycythemia vera. Restoration of a normal hematocrit resulted in resolution of the erythema. Mayo Clin Proc. 2003;78:252-253
252
© 2003 Mayo Foundation for Medical Education and Research
For personal use. Mass reproduce only with permission from Mayo Clinic Proceedings.