Vol. 100 No.

December 2005

ORAL MEDICINE Identication and treatment of scurvy: A case report

Editor: Martin S. Greenberg

Timothy J. Halligan, DMD,a Nathan G. Russell, DDS,b William J. Dunn, DDS,c Steven J. Caldroney, DDS,d and Timothy B. Skelton, MD,e Ocean Springs, Mississippi
KEESLER MEDICAL CENTER

Scurvy is a nondiscriminatory disease process resulting from a nutritional deciency of ascorbic acid (vitamin C). The severe vitamin deciency produces a breakdown in the cellular structure of the body. This case report describes a middle-age woman with a history of edema, bruising of the lower extremities, anemia, and severe periodontal disease. Her presentation and medical history are classic for the signs of scurvy. Scurvy is now only uncommonly seen in developed countries, but there are still vulnerable populations whose nutritional status can lead to scurvy. The aim of this report is to help the clinician identify and treat scurvy, a disease that was once feared for its high mortality but is now easily treatable, even in cases that have progressed to multiple organ dysfunction and failure. (Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;100:688-92)

Scurvy is a nondiscriminatory disease process resulting from a nutritional deciency of ascorbic acid (vitamin C). The disease gets its name from the Latin word scurbutus, which means to rupture or lacerate. The severe vitamin deciency produces a breakdown in the cellular structure of the body, evident in the putrescence of the esh and bones of sufferers, along with night blindness and personality disorders associated with pellagra.1 In the 18th century there was no consensus on what caused scurvy and the symptoms of other known diseases were so varied that it was often mistaken for asthma, leprosy, syphilis, dysentery, and madness.2 Scurvy is an ancient disease, colorfully described as early as 1500 BC in the Ebers papyrus and recorded in the logs of numerous sea captains throughout the 19th century.3 Sailors were among the most common and seriously affected victims. Vasco de Gama lost two

The authors wish to thank Scott Corey, MS, for the digital imaging of the gures used in this manuscript. a GPR Director, Keesler Medical Center, Biloxi, MS. b GPR Resident, Keesler Medical Center, Biloxi, MS. c Director of Research, Keesler Medical Center, Biloxi, MS. d Resident, Keesler Medical Center, Biloxi, MS. e Department of Internal Medicine, Keesler Medical Center, Biloxi, MS. Received for publication Feb 22, 2005; returned for revision Mar 31, 2005; accepted for publication Apr 6, 2005. 1079-2104/$ - see front matter 2005 Mosby, Inc. All rights reserved. doi:10.1016/j.tripleo.2005.04.023

thirds of his crew to the disease on his journey to India in 1499,4 while Magellan lost more than 80% of his crew crossing the Pacic Ocean in 1520.1 The symptoms were described by the ships chaplain: skin black as ink, ulcers, difcult respiration, rictus of the limbs, teeth falling out and, perhaps most revolting of all, a strange plethora of gum tissue sprouting out of the mouth, which immediately rotted and lent the victims breath an abominable odour.2 Sir James Lind, a ships surgeon in the British Navy, became the rst researcher to use scientic methods to identify a cure for scurvy.3 Lind concluded that citrus fruits were the only effective treatment for the disease. In 1845, the great potato famine of Ireland changed the common assumption of scurvy being limited to arduous sea travel. Potatoes, a main staple food for the Irish, have strong antiscorbutic effects.5 However, during the famine, many Irish lacked this main staple, and subsequently developed scurvy. Today, the incidence of scurvy is extremely rare in industrialized countries. In March 2002, an outbreak of hemorrhagic fever was reported in western Afghanistan.6 These hemorrhagic symptoms and mortality were later discovered to be a result of scurvy. Because scurvy is uncommon worldwide, most aid workers and physicians did not consider this disease process in the differential diagnosis. Groups at risk for scurvy include economically disadvantaged populations with poor nutritional health, including elderly persons living alone and alcoholics with poor nutrition.3,7

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This case report describes a middle-age female with a history of edema, bruising in the lower extremities, anemia, and severe periodontal disease. Her presentation and medical history are classic for the signs of scurvy. Scurvy has dropped off the radar scope for many clinicians, but there are still vulnerable populations whose nutritional status can lead to scurvy. The aim of this report is to help the practitioner identify and treat scurvy, a disease that was once feared for its high mortality but is now easily treatable. CASE REPORT
A 39-year-old female (Fig 1, A) was referred to the Hospital Dentistry Clinic from the Internal Medicine Department for clinical evaluation of severe halitosis, gingival bleeding, and tooth mobility. The patient initially presented to the Emergency Department with a chief complaint of swelling and bruising of the right lower extremity. Laboratory data revealed profound hypokalemia and anemia (K1 2.5 mmol/L, hemoglobin [HGB] 7.6 g/L, hematocrit [HCT] 24.5%). Following hospital admission and further evaluation, the patients HGB and HCT dropped to 5.9 g/L and 18.4%, respectively, the following day. The patients medical history revealed signicant ndings. The patient had been previously admitted for swelling and bruising of the right lower extremity, which was present for more than 6 weeks (Fig 1, B). The patient had a history of mental retardation and was under the primary care of her mother. Informed consent for photographs was obtained from both the patient and her mother. Records revealed no past surgical history, no known drug or food allergies, and the patient was not currently taking medication. The patient had no history of tobacco or alcohol use. The patients diet remained essentially unchanged for years, consisting solely of processed macaroni and cheese, peanut butter, and sweetened carbonated beverages. Neither the patient nor her mother could recall the last time fruit or vegetables were included in her diet. The patients serum vitamin C level was less than 0.12 mg/dL, consistent with profound ascorbic acid deciency. Normal physiological requirements for vitamin C are 60 mg/day.8 On examination the patient appeared to be under no apparent distress. The patients clinical signs and symptoms included cutaneous abnormalities with purpura in all extremities along with petechiae. She also exhibited delayed wound healing in an ulcer on the right arm (Fig 1, C), which had been present for several weeks, and lower extremity edema. In addition, the patient presented with corkscrew hair and mild hair exfoliation. Oral ndings were consistent with generalized severe chronic periodontitis.9 The patients panoramic radiograph revealed severe bone loss and a hopeless prognosis for retaining any teeth (Fig 2, A). The patient presented with signicant gingival hyperplastic lesions (Fig 2, B and C). Areas of gingival tissues exhibited necrosis. Spontaneous gingival hemorrhage was evident. The patients remaining dentition displayed Miller class III mobility secondary to advanced periodontal disease. In addition to oral ndings, the

Fig 1. A, Full facial photograph of the patient as she presented to the hospital dentistry department. B, Bruising of the right lower extremity. C, Delayed wound healing.

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Fig 2. A, The patients panoramic radiograph revealing severe bone loss. B, Gingival hyperplastic lesions from the facial aspect. C, Palatal view.

OOOOE Volume 100, Number 6 patient reported chronic generalized myalgia and muscle fatigue. The patient also revealed a recent history of chronic anemia. Clinical symptoms and severe nutritional deciency of vitamin C were consistent with the diagnosis of scurvy. Given the patients current medical condition, a decision was made to treat the patient in the operating room for total odontectomy and gingivoplasty. Complete odontectomy was accomplished and gingivoplasty performed to remove hyperplastic tissue. The patient was discharged with prenatal vitamins and 800-mg ibuprofen tablets for pain. The treatment for the patients medical condition required only prenatal vitamins, which included 120 mg ascorbic acid, along with strong suggestions to improve nutritional status. Two weeks after the surgery, gingival tissues were healing well (Fig 3, A). The patients mother reported an improvement in her daughters affect and the cutaneous abnormalities were almost completely resolved. The ulceration on the patients right arm had improved. At the 4-week follow-up visit a marked resolution had occurred. The gingival tissues were completely healed as well as complete resolution of the purpura and anemia (Fig 3, B and C).

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DISCUSSION When vitamin C is absent from the diet, collagen synthesis is disrupted resulting in malformation and absence of mature collagen. In early disease states, approximately 3 to 6 months after the dietary intake falls below 10 mg/day, nonspecic symptoms, such as fatigue, weakness, irritability, weight loss, vague myalgias, and arthralgias, predominate, making the diagnosis of scurvy difcult. More classic manifestations, such as petechiae, purpura, hemarthroses, dystrophic hair lesions, delayed wound healing, and bone fragility, occur some time later. The most distinguishing feature of scurvy is tissue bleeding with nonpalpable purpura. The onset and progression of the disease was studied prospectively in a prison population by Hodges et al10 in 1971. Symptoms rst appeared 29 to 90 days after the elimination of vitamin C from the diet. Petechial hemorrhage was the rst symptom to appear, while spontaneous ecchymoses was observed in 80% of the population. Periodontal changes occurred in all of the subjects, as did hyperkeratosis and loss of hair. Most subjects developed arthralgias, and a majority developed joint effusions as a late manifestation. All of the subjects also developed xerostomia or keratoconjunctivitis, symptoms that are often seen with Sjogrens syndrome. Untreated, scurvy is inevitably fatal. The clinical presentation of scurvy is applicable to a multitude of differential diagnoses ranging from hematologic abnormalities, trauma, medication side effects, infections, autoimmune disorders, and other vitamin deciencies. An accurate history and current radiographs usually help eliminate trauma as an etiology.

Fig 3. A, Wound healing at 2 weeks. B, Wound healing at 4 weeks. C, Resolution of purpura.

Complete blood cell count, renal and liver function tests, and coagulation studies will eliminate thrombocytopenias and other coagulopathies from the differential diagnosis. Flares of autoimmune disorders such as systemic lupus erythematosus, rheumatoid arthritis, and Sjogrens syndrome can be ruled out by normal levels of inammatory markers like erythrocyte sedimentation rate or C-reactive protein. A plasma or leukocyte vitamin C level will conrm the clinical diagnosis of scurvy. Generally, a vitamin C level of less than 0.1 mg/dL is conrmatory; however, patients with tuberculosis or rheumatic fever can also demonstrate very low plasma levels of vitamin C.3,11 If it is suspected that total

692 Halligan et al body vitamin C stores are depleted, little appears in the urine after an oral test dose of vitamin C is administered. A positive capillary fragility test is an almost constant nding, and anemia is common usually hypochromic, but it may also be normocytic or even microcytic if it is associated with chronic blood loss. Bleeding, coagulation, and prothrombin times are normal, in spite of the almost universal nding of petechiae and purpura. The treatment of scurvy is remarkably simple: vitamin C replacement is the only effective therapy. The recommended daily allowance (RDA) of vitamin C is 60 mg.12 The requirement may increase during pregnancy and lactation, or in cases of acute infection, trauma, or in smokers. Doses of vitamin C higher than the RDA have been suggested for the treatment of scurvy. A dose of 200 mg/day demonstrated signicant improvement in symptoms within several days.13 This amount of vitamin C can also be obtained by consuming 5 servings of fruits and vegetables daily.14 Stephen and Utecht7 and Oefnger15 reported symptom resolution within 3 to 5 days with a dosage of 1 gm/day for the rst 3 to 5 days followed by 300 to 500 mg/day for another week. Spontaneous bleeding stops within 1 day and oral lesions heal in 2 to 3 days.3,11 Ecchymoses heal within 12 days.11 The prognosis for scurvy is excellent when acute symptoms are treated with vitamin C, followed by appropriate dietary changes. Kieffer et al16 demonstrated that even in cases that progressed to multiple organ dysfunction and failure patients dramatically improved with vitamin C infusion therapy. Once a dreaded and fatal disease, scurvy is now easily treatable, even in cases that present in advanced stages of the disease.
REFERENCES
1. Smith MS. The diagnosis and treatment of scurvy: an historical perspective. J Roy Nav Med Serv 1986;72:104-6. 2. Lamb J. Captain Cook and the scourge of scurvy. British Broadcasting Corporation. 8 Jan 2001. BBCi History. 3 Sep 2004.

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Available at: http://www.bbc.co.uk/history/discovery/exploration/ captaincook_scurvy_print.html. Pimentel L. Scurvy: historical review and current diagnostic approach. Am J Emerg Med 2003;21:328-32. Martini E. How did Vasco da Gama sail for 16 weeks without developing scurvy? Lancet 2003;361:1480 [letter]. Forbes GB. Potatoes, a reliable source of vitamin C. Nutr Today 1993;28:33 [letter]. Cheung E, Mutahar R, Assefa F, Ververs MT, Nasiri SM, Borrel A, et al. An epidemic of scurvy in Afghanistan: assessment and response. Food Nutr Bull 2003;24:247-55. Stephen R, Utecht T. Scurvy identied in the emergency department: a case report. J Emerg Med 2001;21:235-7. Johnston CS, Solomon RE, Corte C. Vitamin C depletion is associated with alterations in blood histamine and plasma-free carnitine in adults. J Am Coll Nutr 1996;15:586-601. 1999 International Workshop for a Classication of Periodontal Diseases and Conditions. Papers. Oak Brook, Illinois, October 30-November 2, 1999. Ann Periodontol 1999;4:i, 1-112. Hodges RE, Hood J, Canham JE, Sauberlich HE, Baker EM. Clinical manifestations of ascorbic acid deciency in man. Am J Clin Nutr 1971;24:432-43. Goebel L, Driscoll H. Scurvy. Available at: http://www. emedicine.com/med/topic2086.htm. Accessed September 3, 2004. Subcommittee on the 10th Edition of the RDAs, Food and Nutrition Board, Commission on Life Sciences, National Research Council. Recommended dietary allowances. 10th ed. Washington, DC: Academy Press; 1987. Russell RM. Vitamin and trace mineral deciency and excess. In: Braunwald E, Fauci AS, Kasper DL, editors. Harrisons principles of internal medicine. 15th ed. New York: McGraw-Hill; 2001. p. 461-70. Food and Nutrition Board. Recommended dietary allowances. 9th ed. Washington, DC: National Academy Press; 1980. p. 72-82. Oefnger KC. Scurvy: more than historical relevance. Am Fam Phys 1993;48:609-13. Kieffer P, Thannberger P, Wilhelm JM, Kieffer C, Schneider F. Multiple organ dysfunction dramatically improving with the infusion of vitamin C: more support for the persistence of scurvy in our welfare society. Intensive Care Med 2001;27:448.

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Reprint requests: William Jackson Dunn, DDS Keesler Medical Center Hospital Dentistry 3701 Point Clear Drive Ocean Springs, Miss 39564 william.dunn@keesler.af.mil