Vitamin C deficiency (Scurvy)

OVERVIEW: What every practitioner needs to know

What are the typical findings for this disease?

• In children, the most common presenting signs of vitamin C deficiency are musculoskeletal pain, petechiae/bruising and gingival bleeding (Figure 1).

• Other signs include alopecia, muscle atrophy, poor wound healing, and characteristic corkscrew hairs.

Figure 1.

Background

Scurvy is an uncommon but still encountered condition that has been reported in children with developmental or behavioral disorders (i.e., autism) who consume restricted diets deficient in vitamin C or those suffering from severe neglect. Vitamin C is a cofactor in collagen synthesis. Impairment in collagen biosynthesis is responsible for the presenting signs involving skin, blood vessels, bones and gums.

What other disease/condition shares some of these symptoms?

Scurvy is not usually considered upon first presentation because of its infrequent presentation. Bleeding and anemia may first lead to a suspicion of a bleeding disorder (e.g., immune thrombocytopenic purpura or coagulopathy), although the platelet count and coagulation profile is typically normal. Bleeding and bone pain may lead to consideration of malignancies such as leukemia or neuroblastoma. Musculoskeletal pain may also lead to a question of chronic arthritis, but the severity of the pain and functional limitation seen in children with scurvy is generally more severe than seen in those conditions.

What caused this disease to develop at this time?

• Scurvy is caused by a lack of dietary vitamin C found in fresh fruits and vegetables and juices with either natural or fortified levels of vitamin C. Experimental conditions have suggested that scurvy develops after months of severe vitamin C deficiency.

• Severe deficiency of vitamin C is required for the development of scurvy. While the recommended minimum intake of vitamin C varies from 50 mg/day in infants to 110 mg/day in adolescents, as little as 20 mg/day of vitamin C may be sufficient to avoid scurvy.

What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?

The most specific and readily available test is a serum vitamin C (ascorbic acid) level.

Other specific tests include vitamin C levels measured in buffy coat leukocytes or in urine after vitamin C loading, although these tests are not as readily available or practical.

Although non-specific, iron-deficiency anemia is a characteristic finding, partly secondary to reduced iron absorption in the setting of vitamin C deficiency, even in the setting of adequate iron intake. Note that serum ferritin levels may be normal/high due to inflammation.

High inflammatory markers (erythrocyte sedimentation rate [ESR], C-reactive protein [CRP]) may also be found, and clinicians should be aware of the potential for other nutrient/micronutrient deficiencies.

Would imaging studies be helpful? If so, which ones?

In children with musculoskeletal pain, plain X-rays may show osteopenia and muscle atrophy.

Other more specific radiologic findings on plain X-ray include preservation of the zones of calcification at the distal metaphyses with an adjacent lucency, referred to as the scurvy zone, through which fractures may occur, Pelkan spurs, which are the result of healing fractures at the periphery of the zone of calcification, and an increased density outlining the epiphyses. Periosteal bone may be seen along the shafts of the long bones when subperiosteal bleeding occurs.

MRI findings are more sensitive at detecting subperiosteal bleeding, but this investigation is not required for the a diagnosis in most cases.

If you are able to confirm that the patient has scurvy, what treatment should be initiated?

Treatment of scurvy is simple, with vitamin C supplementation generally in the range of 100-300 mg per day. Treatment is well tolerated, with diarrhea being the most commonly reported side effect if excessive vitamin C is taken, but this is unusual.

Other micronutrients (i.e., iron and vitamin D) may also be necessary.

What are the adverse effects associated with each treatment option?

What are the possible outcomes of scurvy?

Recovery is usually prompt with constitutional symptoms, with bleeding and gum disease improving within days. Depending on the duration of symptoms prior to treatment and whether muscle atrophy is present, bone disease and musculoskeletal function may take weeks for recovery.

What causes this disease and how frequent is it?

How do these pathogens/genes/exposures cause the disease?

What complications might you expect from the disease or treatment of the disease?

Left untreated, scurvy can result in death from bleeding complications (i.e., hemopericardium or cerebral hemorrhage) or sepsis.

Are additional laboratory studies available; even some that are not widely available?

How can scurvy be prevented?

The recommended minimum intake of vitamin C varies from 50 mg/day in infants to 110 mg/day in adolescents. Foods particularly high in vitamin C include oranges, pineapple, grapefruit, strawberrries, kiwi, mango, papaya, cantaloupe, broccoli, Brussels sprouts and cauliflower.

What is the evidence?

Heymann, W. “Scurvy in children”. J Am Acad Dermatol. vol. 57. 2007. pp. 358-9. Review of short case series of findings in children with scurvy.

Weinstein, M, Babyn, P, Zlotkin, S. “An orange a day keeps the doctor away: Scurvy in the year 2000”. Pediatrics. vol. 108. 2001. pp. e55Case report of child with scurvy describing associated MRI findings.

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