AUTHOR AND EDITOR INFORMATION

Section 1 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

INTRODUCTION

Section 2 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Background

Scurvy is a condition that has been described for at least 500 years. In 1753, Sir James Lind demonstrated that the condition could be prevented in British sailors by adding citrus fruits to their diets. Forty years later, Sir Gilbert Blane convinced the admiralty of the British Navy to approve a preventive dietary regimen for sailors. Prior to that, all sea voyages had to be limited to 10 weeks or less to rehabilitate the crews. Scurvy produces characteristic perifollicular hemorrhages and gingival changes as well as hematologic, joint, and cardiac complications.

Pathophysiology

Scurvy is caused by a prolonged deficiency of vitamin C intake that results in defective collagen synthesis, tissue repair, and synthesis of lipids and proteins. It functions both as a reducing agent and as an antioxidant and is required for many physiologic functions, including metabolism of iron and folic acid, resistance to infection, and integrity of blood vessels.

The clinical manifestations of scurvy are primarily due to abnormal collagen synthesis resulting from a lack of vitamin C. Vitamin C is a cofactor required for the function of several hydroxylases. The absence of vitamin C reduces the function of prolyl hydroxylase, which is required to form hydroxyproline, an amino acid found in collagen but rarely found in other proteins. The presence of hydroxyproline in collagen stabilizes the collagen triple-helix structure by forming interstrand hydrogen bonds. Collagen lacking hydroxyproline is more fragile and contributes to the clinical manifestations of scurvy, including purpura due to vessel wall fragility. In addition, osteoid matrix formation is defective and bone resorption is increased in persons with vitamin C deficiency.

Frequency

United States

Scurvy is rare in the United States. Patients at risk include those with chronic malnutrition, such as persons with alcoholism, elderly people, and men who live alone (widower scurvy). Other reported cases include people with monotonous or peculiar diets, including patients undergoing dialysis and those with malabsorption, inflammatory bowel disease, cancer on chemotherapy, Whipple disease, or dyspepsia (those who avoid acidic foods). Scurvy may occur in infants fed evaporated or condensed milk formulas.

Infantile scurvy was fairly common in the United States during the 1950s, but changes in feeding practices in the next decade almost completely eliminated the disorder. If a mother has an adequate diet, breast milk contains sufficient vitamin C for a baby's needs. Commercially available formulas and many prepared fruit juices are fortified with vitamin C.

International

Scurvy is a problem when general malnutrition exists, as in some impoverished, underdeveloped third world countries. Scurvy also occurs in epidemic proportions in international refugee camps and in populations that subsist mainly on cereal grains.

Mortality/Morbidity

Until minimal daily requirements of vitamin C were supplied, scurvy plagued prolonged naval voyages and military campaigns as personnel succumbed to its devastating effects. Lethargy, fatigue, and hemorrhagic manifestations of impaired collagen synthesis affecting oral, ophthalmic, musculoskeletal, cardiac, and gastrointestinal structures and functions incapacitated or killed more people than enemy action in many cases. Manifestations of scurvy tend to dramatically improve, resolving within weeks, if adequate vitamin C is given in daily doses to recoup body stores.

Race

No racial predilection for scurvy has been sited in the literature

Sex

No sex predilection for scurvy has been sited in the literature.

CLINICAL

Section 3 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

History

• Symptoms of scurvy develop after 3 months of severe or total vitamin C deficiency.
• Patients may complain of weakness, fatigue, shortness of breath, and aching limbs. Left untreated scurvy progresses, with potentially fatal complications, including cerebral hemorrhage or hemopericardium.
• Infantile scurvy is uncommon before age 7 months, and clinical and radiographic manifestations rarely occur in infants younger than 3 months. Early clinical manifestations consist of pallor, irritability, and poor weight gain.
• In advanced infantile scurvy, the major clinical manifestation is extreme pain and tenderness of the arms and, particularly, the legs. The baby is miserable and tends to remain in a characteristic immobilized posture from subperiosteal pain, with semiflexion of the hips and the knees, as described by Thomas Barlow in 1884. The body is both wasted and edematous, and petechiae and ecchymoses are commonly present.

Physical

• Symptoms and signs of scurvy may be remembered by the 4 Hs: hemorrhage, hyperkeratosis, hypochondriasis, and hematologic abnormalities. Patients may be miserable, irritable, depressed, resentful, and full of aches and pains. The body is both wasted and edematous, and petechiae and ecchymoses are commonly present.
• The earliest signs are found on the skin, often on the shins, after 3 months of severe or total vitamin C deprivation. Perifollicular hyperkeratotic papules are surrounded by hemorrhagic halos. The central hairs are twisted like corkscrews, and they may become fragmented. The posterior parts of the legs develop purpura that may coalesce.
• Soft, spongy swelling of the gums and gingival interdental papillae is followed by gingival hemorrhage, which is accentuated by coexistent poor oral hygiene and periodontal disease. Disrupted tooth formation and loosening of teeth may result in permanent defects of dentition.
• Ocular features include those of Sjögren's syndrome, subconjunctival hemorrhage, and bleeding within the optic nerve sheath. Funduscopic changes include cotton wool spots and flame-shaped hemorrhages.
• Bleeding into the joints causes exquisitely painful hemarthroses. Subperiosteal hemorrhage may be palpable, especially along the distal portions of the femurs and the proximal parts of the tibias of infants. In advanced cases, clinically detectable beading may be present at the costochondral junctions of the ribs. This finding is known as the scorbutic rosary. Bleeding into the femoral sheaths may cause femoral neuropathies, and bleeding into the muscles of the arms and the legs may cause woody edema.
• Heart complications include cardiac enlargement, ECG changes (reversible ST-segment and T-wave changes), hemopericardium, and sudden death.
• Anemia develops in 75% of patients, resulting from blood loss into tissue, coexistent dietary deficiencies (folate deficiency), altered absorption and metabolism of iron and folate, gastrointestinal blood loss, and intravascular hemolysis. The anemia is most often characterized as normochromic and normocytic. Vitamin C enhances iron absorption by reducing dietary iron from the ferric form to the ferrous form. Thus, vitamin C deficiency may reduce the availability of intracellular iron. Vitamin C is also necessary to convert folic acid to its active metabolite, folinic acid.
• Other problems include increased redness and swelling in recently healed wounds and the failure of new wounds to heal.

Causes

• Scurvy is caused by a prolonged deficiency of vitamin C intake.
• Most animals can convert gluconate into ascorbate. Primates, including humans, and guinea pigs as well as a few other species cannot convert gluconate into ascorbate and, therefore, require exogenous ascorbic acid, otherwise known as vitamin C. Humans obtain 90% of their intake of vitamin C from fruits and vegetables, and cooking these sources decreases vitamin C content 20-40%. The US Food and Drug Administration recommends a daily dietary allowance of vitamin C of 75 mg for women and 90 mg for men.
• The total body pool of vitamin C is approximately 1500 mg. The absorbed vitamin is found ubiquitously in body tissues, with the highest concentrations in glandular tissue and the lowest concentrations in muscle and stored fat. Ascorbic acid is metabolized in the liver by oxidation and sulfation. The renal threshold for excretion by the kidney in urine is approximately 1.4 mg/100 mL plasma. Excess amounts of ascorbic acid are excreted unchanged or as metabolites. When body tissue or plasma concentrations of vitamin C are low, excretion of the vitamin is decreased. Scurvy occurs after vitamin C has been eliminated from the diet for at least 3 months and when the body pool falls below 350 mg.

DIFFERENTIALS

Section 4 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Other Problems to be Considered

Acute ulcerative gingivitis
Coagulation abnormalities
Collagen-vascular diseases
Deep vein thrombosis
Infection
Adverse medication effects
Platelet disorders
Septic arthritis
Systemic bleeding disorders
Trauma to legs and joints

WORKUP

Section 5 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Lab Studies

• The diagnosis is mainly made on the basis of the historical features and the physical findings. Plasma ascorbic acid level may help in establishing the diagnosis, but this level tends to reflect the recent dietary intake rather than the actual tissue levels of vitamin C. Signs of scurvy can occur with low-normal serum levels of vitamin C.
• The level of vitamin C in leukocytes more accurately correlates to tissue stores compared with serum levels because these cells are not affected acutely by circadian rhythm or dietary changes. Recently, a specific and reproducible reverse-phase, high-pressure liquid chromatographic method has been found reliably to measure vitamin C in lymphocytes. This test is currently not clinically available but it might be useful for screening. A more commonly used method is the ascorbic acid tolerance test, which quantitates urinary ascorbic acid over the 6 hours following an oral load of 1 g of ascorbic acid in water. The best confirmation of the diagnosis of scurvy is still its resolution following vitamin C administration.

Imaging Studies

• The earliest radiologic manifestation of infantile scurvy is generally seen at the distal ends of the radii where fuzziness of the lateral aspects of the cortices is present with slight rarefaction of the neighboring cancellous bone.
• As the disease progresses, radiographs demonstrate characteristic changes at the cartilage-shaft junctions of the long bones, especially at the distal ends of the femurs. Key imaging features show osteoporosis; increased density and widening of the zone of provisional calcification between the epiphysis and metaphysis (white line of Frankel); metaphyseal spurs or marginal fractures (Pelkan spur), a transverse band of radiolucency in the metaphysis (scurvy line or Trümmerfeld zone), which is subjacent to the zone of provisional calcification; ring of increased density surrounding the epiphysis (Wimberger ring); and periosteal elevation.

Histologic Findings

Noninflammatory perivascular extravasation of red cells and deposition of hemosiderin near hair follicles with intrafollicular keratotic plugs and coiled hair may be seen in a skin biopsy specimen.

TREATMENT

Section 6 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Medical Care

• Scurvy is treated with ascorbic acid.
• Patients who undergo dialysis should receive 500 mg of supplemental vitamin C daily.
• Patients benefit from a daily multivitamin because of potential coexisting nutritional deficiencies.

Consultations

• Consult a dermatologist for evaluation of skin findings.
• Consult a nutritionist for evaluation of diet, nutritional education, and assistance.
• Consult an internal medicine specialist for evaluation of systemic findings.

Diet

• As little as 6-10 mg of vitamin C a day is sufficient to maintain body stores of more than 350 mg. Higher intakes are recommended for smokers and pregnant women. Fresh fruits and vegetables are the best sources of vitamin C. A small orange contains approximately 50 mg of vitamin C. In the United States, many beverages are fortified with vitamin C.

MEDICATION

Section 7 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

The goal of treatment of scurvy is to saturate the body rapidly with ascorbic acid. At maximum doses, body stores become saturated in a few days. With proper treatment, bleeding stops within 24 hours, and perifollicular petechiae resolve in 2 weeks.

Drug Category: Vitamins

Vitamins are essential for normal DNA synthesis and cell function.

FOLLOW-UP

Section 8 of 9  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

Deterrence/Prevention:

• Scurvy can be prevented by consuming fresh fruit and vegetables. As little as 6-10 mg of vitamin C a day is sufficient to maintain body stores of more than 350 mg.

Complications:

• Lethargy, fatigue, and hemorrhagic manifestations of impaired collagen synthesis affecting oral, ophthalmic, musculoskeletal, cardiac, and gastrointestinal structures and functions may occur.
• Cerebral hemorrhage or hemopericardium are potential complications that may lead to death.

Patient Education:

• Patients and their families should be educated about proper nutrition.

REFERENCES

Section 9 of 9

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• References

• Abernethy DR, Arnold GJ, Azarnoff D. Mosby Drug Consult. St. Louis, Mo: Mosby; 2005.
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• Emadi-Konjin P, Verjee Z, Levin AV, Adeli K. Measurement of intracellular vitamin C levels in human lymphocytes by reverse phase high performance liquid chromatography (HPLC). Clin Biochem. May 2005;38(5):450-6. [Medline].
• Fain O. Musculoskeletal manifestations of scurvy. Joint Bone Spine. Mar 2005;72(2):124-8. [Medline].
• Formon J. Nutrition of Normal Infants. Chicago, Ill: Mosby-Year Book; 1993:22, 360-5.
• Garg K, Draganescu JM, Albornoz MA. Puzzles in practice. A rash imposition from a lifestyle omission. Vitamin C deficiency. Postgrad Med. Nov 1998;104(5):183-4. [Medline].
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• Hasan L, Vögeli P, Stoll P, Kramer SS, Stranzinger G, Neuenschwander S. Intragenic deletion in the gene encoding L-gulonolactone oxidase causes vitamin C deficiency in pigs. Mamm Genome. Apr 2004;15(4):323-33. [Medline].
• Hatuel H, Buffet M, Mateus C, Calmus Y, Carlotti A, Dupin N. Scurvy in liver transplant patients. J Am Acad Dermatol. Jul 2006;55(1):154-6. [Medline].
• Hirschmann JV, Raugi GJ. Adult scurvy. J Am Acad Dermatol. Dec 1999;41(6):895-906; quiz 907-10. [Medline].
• McKenna KE, Dawson JF. Scurvy occurring in a teenager. Clin Exp Dermatol. Jan 1993;18(1):75-7. [Medline].
• Olmedo JM, Yiannias JA, Windgassen EB, Gornet MK. Scurvy: a disease almost forgotten. Int J Dermatol. Aug 2006;45(8):909-13. [Medline].
• Onorato J, Lynfield Y. Scurvy. Cutis. May 1992;49(5):321-2. [Medline].
• Pimentel L. Scurvy: historical review and current diagnostic approach. Am J Emerg Med. Jul 2003;21(4):328-32. [Medline].
• Sarkany RPE, Breathnach SM, Seymour CA. Vitamin C deficiency. In: Burns T, Breathnach SM, Cox N, Griffiths CE, eds. Rook's Textbook of Dermatology. Vol. 57. 7^th ed. Oxford, England: Blackwell; 2004:94-95.
• Schnitzler CM, Schnaid E, MacPhail AP, Mesquita JM, Robson HJ. Ascorbic acid deficiency, iron overload and alcohol abuse underlie the severe osteoporosis in black African patients with hip fractures--a bone histomorphometric study. Calcif Tissue Int. Feb 2005;76(2):79-89. [Medline].
• Yalçin A, Ural AU, Beyan C, Tastan B, Demiriz M, Cetin T. Scurvy presenting with cutaneous and articular signs and decrease in red and white blood cells. Int J Dermatol. Dec 1996;35(12):879-81. [Medline].

Article Last Updated: Feb 1, 2007