AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

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

INTRODUCTION

Section 2 of 10  

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

Background

Scurvy was first described in 1541 by a Dutch physician named Echthius working in Cologne, Germany. Mistakenly, he thought it was an infectious disease. In 1540, a French explorer named Jacques Cartier learned of a remedy for scurvy from the Native Americans of lower Canada, which was prepared by extracting the needles of pine trees with hot water. The first English reference to the disease occurred in the Oxford English Dictionary in 1565.

Two physicians who played an enormous role in decreasing the mortality from the disease were John Woodall and James Lind. In 1617, Woodall wrote The Surgeon's Mate, which described scurvy and listed lemon juice as the cure. Woodall persuaded the East India Company to provide lemon juice for its sailors.

In 1747, Lind, an officer in the British Royal Navy, conducted a study on 12 patients with scurvy. He divided the patients into 6 groups of 2 and gave each group a different remedy. Only the group given oranges and lemons recovered. It took Lind 41 years to convince the British Royal Navy to implement his recommendation. The British used lime juice instead of lemon or orange juice to prevent the disease, and the sailors became known as limeys.

Pathophysiology

Humans, other primates, and guinea pigs are unable to synthesize L-ascorbic acid (vitamin C); therefore, they require it in their diet. The enzyme, L-gluconolactone oxidase, which usually would catalyze the conversion of L-gluconogammalactone to L-ascorbic acid, is defective due to a mutation or inborn error in carbohydrate metabolism.

Vitamin C is required as a redox agent, reducing metal ions in many enzymes and removing free radicals. In this capacity, it protects DNA, protein, and vessel walls from damage caused by free radicals. Vitamin C is necessary for the triple-helix formation of collagen. Deficiency of vitamin C leads to impaired collagen synthesis, causing capillary fragility, poor wound healing, and bony abnormalities in affected adults and children.

Although the clinical manifestations are unclear, vitamin C is a cofactor in the metabolism of tyrosine and cholesterol and the synthesis of carnitine, norepinephrine, peptide hormones, corticosteroids, and aldosterone.

It also enhances the absorption of iron from the small intestine. This may contribute to the anemia seen with vitamin C deficiency.

Frequency

United States

Data from the Third National Health and Nutrition Examination Survey (NHANES III) assessed the prevalence of vitamin C deficiency in the United States among a sample of 15,769 children and adults aged 12-74 years. They found that 14% of males and 10% of females were vitamin C deficient.

A recent study from the May Clinic in Rochester Minnesota revealed 12 cases of scurvy in a retrospective chart review between 1976 and 2002.  Eight of the patients were women and the average age was 48 years.

International

A study of nonhospitalized patients in Paris found that 5% of women and 12% of men were deficient. In those older than 65 years, this proportion increased to 15% of women and 20% of men.

Race

According to NHANES III, non-Hispanic black males had a slightly increased risk of vitamin C deficiency (OR = 1.2; 95% CI = 1.1,1.5) compared to white males. Mexican American males and females had a lower risk of vitamin C deficiency compared to white males and females probably because the traditional Mexican diet is rich in chilies, tomatoes, and squashes, which are high in vitamin C.

Sex

Some studies show vitamin C deficiency to be more common among men, whereas others show equal distribution among men and women.

Age

• The incidence of scurvy peaks in children aged 6-12 months who are fed a diet deficient in citrus fruits or vegetables.
• Incidence also peaks in elderly populations, who sometimes have "tea-and-toast" diets deficient in vitamin C.

CLINICAL

Section 3 of 10  

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

History

• Early symptoms are malaise and lethargy.
• After 1-3 months, patients develop shortness of breath and bone pain. Myalgias may occur because of reduced carnitine production.
• Other symptoms include skin changes with roughness, easy bruising and petechiae, gum disease, loosening of teeth, poor wound healing, and emotional changes.
• Dry mouth and dry eyes similar to Sjögren syndrome may occur.
• In the late stages, jaundice, generalized edema, oliguria, neuropathy, fever, and convulsions can be seen.

Physical

• Vital signs: Hypotension may be observed late in the disease. This may be due to an inability of the resistance vessels to constrict in response to adrenergic stimuli.
• Skin: Perifollicular hyperkeratotic papules, perifollicular hemorrhages, purpura, and ecchymoses are seen most commonly on the legs and buttocks where hydrostatic pressure is the greatest.  Poor wound healing and breakdown of old scars may be seen.
• Nails: Splinter hemorrhages may occur.
• Head and neck: Gum swelling, friability, bleeding, and infection with loose teeth; mucosal petechiae; scleral icterus (late, probably secondary to hemolysis); and pale conjunctiva are seen. Conjunctival hemorrhage, flame-shaped hemorrhages, and cotton-wool spots may be seen. Bleeding into the periorbital area, eyelids, and retrobulbar space also can be seen.  Alopecia may occur secondary to reduced disulfide bonding.
• Chest and cardiovascular: Scorbutic rosary (ie, sternum sinks inward) may occur in children. High-output heart failure due to anemia can be observed. Bleeding into the myocardium and pericardial space has been reported.
• Extremities: Fractures, dislocations, and tenderness of bones are common in children. Bleeding into muscles and joints may be seen. Edema may occur late in the disease.
• Gastrointestinal: Loss of weight secondary to anorexia is common. Upper endoscopy may show submucosal hemorrhage.

Causes

• Scurvy is caused by a dietary deficiency of vitamin C. The body's pool of vitamin C can be depleted in 1-3 months.
• Risk factors include the following:
• • Babies who are fed only cow's milk during the first year of life are at risk.
  • Alcoholism and conforming to food fads are risk factors.
  • Elderly individuals who eat a tea-and-toast diet are at risk. Retired people who live alone and those who eat primarily at fast food restaurants face increased risk of deficiency.
  • Economically disadvantaged persons tend to not purchase foods high in vitamin C (eg, green vegetables, citrus fruits), which results in them being at high risk.
  • More recently, vitamin C deficiency has been noted in refugees who are dependent on external suppliers for their food and have limited access to fresh fruits and vegetables.
  • Cigarette smokers require increased intake of vitamin C because of lower vitamin C absorption and increased catabolism.
  • Pregnant and lactating women and those with thyrotoxicosis require increased intake of vitamin C because of increased utilization.
  • People with anorexia nervosa or anorexia from other diseases such as AIDS or cancer are at increased risk of vitamin C deficiency.
  • People with type 1 diabetes have increased vitamin C requirements, as do those on hemodialysis and peritoneal dialysis.
  • Because vitamin C is absorbed in the small intestine, people with disease of the small intestine such as Crohn, Whipple, and celiac disease are at risk.
  • Iron overload disorders may lead to renal vitamin C wasting.

DIFFERENTIALS

Section 4 of 10  

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

Other Problems to be Considered

Clotting factor deficiencies
Platelet dysfunction
Senile purpura
Medication side effects
Hematologic malignancies
Necrotizing gingivitis

WORKUP

Section 5 of 10  

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

Lab Studies

• Obtaining a plasma or leukocyte vitamin C level can confirm clinical diagnosis.
• • Scurvy occurs at levels generally less than 0.1 mg/dL.
  • Symptoms occur at levels below 2.5 mg/L, which is considered deficiency.
  • Levels of 2.5-5 mg/L indicate depletion.
  • Levels can be low in patients who have tuberculosis, rheumatic fever, or other chronic illnesses; those who smoke cigarettes; and patients on oral contraceptive drugs.
• Capillary fragility can be checked by inflating a blood pressure cuff and looking for petechiae on the forearm.

Imaging Studies

• Radiography may show any of the following:
• • Subperiosteal elevation
  • Fractures and dislocation
  • Alveolar bone reabsorption
  • Ground-glass appearance of cortex

TREATMENT

Section 6 of 10  

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

Medical Care

Patients should take ascorbic acid at 100 mg 3-5 times a day until total of 4 g is reached, and then they should decrease intake to 100 mg daily.

Alternately, ascorbic acid may be taken at 1 g/d for the first 3-5 days followed by 300-500 mg/d for a week. Then the recommended daily allowance is resumed.

• Divided doses are given because intestinal absorption is limited to 100 mg at one time.
• Parenteral doses are necessary in those with gastrointestinal malabsorption.

Diet

• Foods high in vitamin C include the following.
• • Citrus fruits, especially grapefruits and lemons
  • Vegetables, including broccoli, green peppers, tomatoes, potatoes, and cabbage
• The recommended daily allowance for vitamin C varies. The current recommendation for adults is 120 mg daily, although a dose of 60 mg daily is all that is required to prevent scurvy. Some experts think the level should be as high as 200 mg daily to match the level present in 5 servings of fruits and vegetables daily, a diet shown to decrease cancer risk.
• Megadoses of vitamin C have not been shown in clinical trials to reduce viral illnesses such as colds. Large doses of vitamin C, ie, more than 1 g/d, may increase the risk of certain illnesses such as kidney stones, particularly oxalate stones.

MEDICATION

Section 7 of 10  

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

The only effective therapy is vitamin C replacement.

Drug Category: Vitamins

Provide critical cofactor necessary for collagen fibril synthesis.

FOLLOW-UP

Section 8 of 10  

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

Complications

• Scurvy is fatal if untreated.

Prognosis

• Patients respond quickly to oral therapy.
• • Spontaneous bleeding stops within one day.
  • Muscle and bone pain abate quickly.
  • Bleeding and sore gums heal in 2-3 days.
  • Ecchymoses heal within 12 days.
• In advanced scurvy, serum bilirubin normalizes in less than a week. Anemia is corrected in less than a month.

MISCELLANEOUS

Section 9 of 10  

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

Medical/Legal Pitfalls

• Failure to recognize the condition
• Failure to properly treat the condition with administration of vitamin C

REFERENCES

Section 10 of 10

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

• Carr AC, Frei B. Toward a new recommended dietary allowance for vitamin C based on antioxidant and health effects in humans. Am J Clin Nutr. Jun 1999;69(6):1086-107. [Medline].
• Fain O. Musculoskeletal manifestations of scurvy. Joint Bone Spine. 2005;72:124-128. [Medline].
• Fauci AS, Braunwald E, Isselbacher KJ, et al, eds. Harrison's Principles of Internal Medicine. 14th ed. New York, NY: McGraw-Hill; 1998:. 484-5.
• Hampl JS, Taylor CA, Johnston CS. Vitamin C deficiency and depletion in the United States: the Third National Health and Nutrition Examination Survey, 1988 to 1994. Am J Public Health. May 2004;94(5):870-5. [Medline].
• Hercberg S, Preziosi P, Galan P, et al. Vitamin status of a healthy French population: dietary intakes and biochemical markers. Int J Vitam Nutr Res. 1994;64(3):220-32. [Medline].
• Johnston CS, Thompson LL. Vitamin C status of an outpatient population. J Am Coll Nutr. Aug 1998;17(4):366-70. [Medline].
• Jukes TH. The prevention and conquest of scurvy, beri-beri, and pellagra. Prev Med. Nov 1989;18(6):877-83. [Medline].
• Levine M, Rumsey SC, Daruwala R, et al. Criteria and recommendations for vitamin C intake. JAMA. Apr 21 1999;281(15):1415-23. [Medline].
• Olmedo JM, Yiannias JA, Windgassen EB, Gornet MK. Scurvy: a disease almost forgotten. Int J Dermatol. Aug/2006;45:909-13. [Medline].
• Pimentel L. Scurvy: Historical Review and Current Diagnostic Approach. Am J Emerg Med. 2003;21:328-332.
• Schuman RW, Rahmin M, Dannenberg AJ. Scurvy and the gastrointestinal tract. Gastrointest Endosc. Feb 1997;45(2):195-6. [Medline].
• Smith MS. The diagnosis and treatment of scurvy: an historical perspective. J R Nav Med Serv. Summer 1986;72(2):104-6. [Medline].
• Toole MJ. Micronutrient deficiencies in refugees. Lancet. May 16 1992;339(8803):1214-6. [Medline].

Article Last Updated: Sep 11, 2007