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, a state of dietary deficiency of vitamin C (ascorbic acid) is an ancient disease. Egyptians recorded its symptoms as early as 1550 BC. Scurvy was the scourge of the sea explorers of the Renaissance era (16th-18th centuries). In 1746, James Lind, a British naval surgeon, established the fact that oranges and lemons were effective in curing scurvy. The incidence of scurvy among the British sailors sharply declined upon routine provision of lemon juice on board. During the 19th century, people who experienced the Great Potato Famine, armies of the Crimean War and American Civil War, Arctic explorers, and California Gold Rush communities were prominent victims of "land" scurvy.

Francis Glisson is credited with the earliest description of infantile scurvy. In 1650, Glisson observed the co-occurrence of scurvy in infants with rickets. After Glisson's description, infantile scurvy was not reported for another 200 years. By the end of the 19th century, infantile scurvy was readily recognized and frequently observed in Britain and the United States. The increased incidence of infantile scurvy during this era was attributed to the consumption of heated milk and proprietary foods deficient in vitamin C.

In 1912, Holst and Frolisch induced and cured scurvy in guinea pigs through dietary modification. Utilization of an animal model for induction and treatment of scurvy was a major advance. Soon, human experiments followed. In 1914, Alfred Hess, a pediatrician practicing at the Hebrew Asylum in New York, observed an increase in scurvy among the infants at the asylum. This increase in scurvy coincided with the introduction of pasteurized milk and exclusion of orange juice from the infants' diet.

Modifying the infants' diet, with the provision of raw milk or orange juice or potatoes, Hess reversed the scurvy and effected a cure. Hess had demonstrated that pasteurization results in the loss of vitamin C. He recommended the provision of fresh fruit or vegetable juice for the prevention of scurvy in infants fed on heated formulas. This practice of supplementing the diet of infants receiving heated formulas with fresh fruit or vegetable juices eventually led to the eradication of infantile scurvy in the United States.

The human body lacks the ability to synthesize and make vitamin C and therefore depends on exogenous dietary sources to meet vitamin C needs. Consumption of fruits and vegetables or diets fortified with vitamin C are essential to avoid ascorbic acid deficiency. Even though scurvy is uncommon, it still occurs and can affect adults and children who have chronic dietary vitamin C deficiency.

Pathophysiology

Vitamin C is functionally most relevant for collagen synthesis, and a vitamin C deficiency results in impaired collagen synthesis. The typical pathologic manifestations of vitamin C deficiency are noted in collagen-containing tissues and in organs and tissues such as skin, cartilage, dentine, osteoid, and capillary blood vessels. Pathologic changes are a function of the rate of growth of the affected tissues; hence, the bone changes are often observed only in infants during periods of rapid bone growth.

Defective collagen synthesis leads to defective dentine formation, hemorrhaging into the gums, and loss of teeth. Hemorrhaging is a hallmark feature of scurvy and can occur in any organ. Hair follicles are one of the common sites of cutaneous bleeding.

Bone involvement is typical for infantile scurvy. The bony changes occur at the junction between the end of the diaphysis and growth cartilage. Osteoblasts fail to form osteoid (bone matrix), resulting in cessation of endochondral bone formation. Calcification of the growth cartilage at the end of the long bones continues, leading to the thickening of the growth plate. The typical invasion of the growth cartilage by the capillaries does not occur. Preexisting bone becomes brittle and undergoes resorption at a normal rate, resulting in microscopic fractures of the spicules between the shaft and calcified cartilage. With these fractures, the periosteum becomes loosened, resulting in the classic subperiosteal hemorrhage at the ends of the long bones. Intra-articular hemorrhage is rare because the periosteal attachment to the growth plate is very firm.

Besides being essential for collagen synthesis, ascorbic acid is important for biosynthesis of carnitine and neurotransmitters and in hematopoiesis by promoting iron absorption.

Frequency

United States

Currently, scurvy occurs very rarely in the United States. Patients who are elderly or alcoholic and who subsist on diets devoid of fresh fruits and vegetables are vulnerable. Infants and children on restrictive diets because of medical, economic, or social reasons are at risk for scurvy. Occurrence of scurvy is uncommon in those younger than 7 months.

International

International occurrence is unknown. A case series from Thailand reviewed 28 cases of scurvy in infants and children (10 mo to 9 y and 7 mo; median age, 29 mo) hospitalized over a 7-year period (1995-2002) and noted prolonged consumption of heated milk (ultra-high temperature [UHT] milk) and inadequate intake of vegetables and fruits as the risk factors for the development of scurvy. Epidemic scurvy has been reported among refugee populations.

Mortality/Morbidity

• Sudden death due to cardiac failure is reported in infants and adults.
• Predominant morbidity is a result of hemorrhage into various tissues. Subperiosteal hemorrhage in the tibia and femur causes excruciating pain.

Race

No racial predilection exists.

Sex

No sexual preponderance exists.

Age

Scurvy can occur at any age. Most cases of infantile scurvy occur when the infant is aged 6-24 months. Scurvy is uncommon in the neonatal period.

CLINICAL

Section 3 of 10  

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

History

• Initial symptoms are nonspecific and include the following:
• • Loss of appetite
  • Peevishness
  • Poor weight gain
  • Diarrhea
  • Tachypnea
  • Fever
• Specific symptoms include the following:
• • Irritability
  • Pain and tenderness of the legs
  • Pseudoparalysis
  • Swelling over the long bones
  • Hemorrhage

Physical

• The infant is apprehensive, anxious, and progressively irritable. Upon handling and changing of diapers, severe tenderness over the thighs is present. The excruciating pain results in pseudoparalysis. The infant assumes the frog leg posture (ie, keeping hips and knees slightly flexed and externally rotated) for comfort.
• Hemorrhages of the gums usually involve the tissue around the upper incisors. The gums have a bluish-purple hue and feel spongy. Gum hemorrhage occurs only if teeth have erupted.
• Subperiosteal hemorrhage is a typical finding of infantile scurvy. The lower ends of the femur and tibia are the most frequently involved sites. The subperiosteal hemorrhage is often palpable and tender in the acute phase.
• Petechial hemorrhage of the skin and mucous membranes can occur. Rarely, hematuria, hematochezia, and melena are noted.
• Proptosis of the eyeball secondary to orbital hemorrhage is a sign of scurvy.
• Costochondral beading or scorbutic rosary is a common finding. The scorbutic rosary is distinguished from rickety rosary (which is knobby and nodular) by being more angular and having a step-off at the costochondral junction. The sternum is typically depressed.
• Low-grade fever, anemia, and poor wound healing are signs of scurvy.
• Hyperkeratosis, corkscrew hair, and sicca syndrome are typically observed in adult scurvy but rarely occur in infantile scurvy.

Causes

• Inadequate intake of vitamin C
• Long sea voyages (historically)
• Famines
• Food faddism
• Ignorance (eg, boiling of fruit juices)
• Neglect (eg, self-imposed restrictive diets in anorexia nervosa)
• Restrictive diets imposed by food allergies
• Neurodevelopmental disabilities associated with compromised oral intake of foods

DIFFERENTIALS

Section 4 of 10  

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

Other Problems to be Considered

Acrodynia
Henoch-Schönlein purpura
Rheumatoid arthritis

WORKUP

Section 5 of 10  

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

Lab Studies

• Laboratory tests are usually not helpful to ascertain a diagnosis of scurvy. Presentation of an infant with the typical clinical and radiologic picture of scurvy, along with a supportive history of dietary deficiency of vitamin C, is often sufficient to diagnose infantile scurvy.
• Serum ascorbic acid levels
• • A fasting serum ascorbic acid level greater than 0.6 mg/dL rules out scurvy.
  • Serum ascorbic acid levels of 0.2 mg/dL or greater are considered nutritionally acceptable.
  • Levels of 0.10-0.19 mg/dL are considered low.
  • Levels less than 0.10 mg/dL are considered deficient.
• White blood cell ascorbic acid concentration is a more accurate measure of a vitamin C nutritional state.
• • A level of zero indicates latent scurvy.
  • Levels greater than 15 mg/dL reflect a state of nutritional adequacy.
  • Levels of 8-15 mg/dL are considered low.
  • Levels of 0-7 mg/dL reflect a state of deficiency.

Imaging Studies

• Radiologic findings in infantile scurvy are diagnostic.
• The characteristic radiologic changes occur at the growth cartilage-shaft junction of bones with rapid growth. The knee joint, wrist, and sternal ends of the ribs are typical sites of involvement.
• Radiologic diagnosis of scurvy is based on the following specific changes:
• • In the early phase of scurvy, the cortex becomes thin and the trabecular structure of the medulla atrophies and develops a ground-glass appearance. The zone of provisional calcification becomes dense and widened, and this zone is referred to as the white line of Fränkel. The epiphysis also shows cortical thinning and the ground-glass appearance.
  • As scurvy becomes advanced, a zone of rarefaction occurs at the metaphysis under the white line. The zone of rarefaction typically involves the lateral aspects of the white line, resulting in triangular defects called the corner sign of Park. This area has multiple microscopic fractures and may collapse with impaction of the calcified cartilage onto the shaft. The lateral aspect of the calcified cartilage can project as a spur. Subperiosteal hemorrhages are not visualized in the active phase. With healing, they become calcified and are readily observed.

TREATMENT

Section 6 of 10  

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

Medical Care

• Vitamin C administered by mouth or the parenteral route is effective in curing infantile scurvy.
• Orange juice is an effective dietary remedy for curing infantile scurvy and was the standard treatment before the discovery of vitamin C.
• Upon instituting dietary or pharmacologic treatment, the clinical recovery is impressive. The appetite of the infant is recovered within 24-48 hours. The symptoms of irritability, fever, tenderness upon palpation, and hemorrhage generally resolve within 7 days.

Diet

• A diet adequate in vitamin C can prevent the development of scurvy. The dietary requirements of vitamin C sufficient to prevent deficiency vary with the age of the individual. The following are the Food and Nutrition Board of the National Academy of Sciences, National Research Council's minimum recommended daily dietary allowances of vitamin C:
• • Infants - 30-40 mg
  • Children and adults - 45-60 mg
  • Pregnant women - 70 mg
  • Lactating mothers - 90-95 mg
• Food sources rich in vitamin C include the following:
• • Citrus fruits
  • Berries
  • Cantaloupe
  • Broccoli
  • Cauliflower
  • Cabbage
  • Spinach
  • Potatoes
  • Tomatoes

MEDICATION

Section 7 of 10  

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

Drug Category: Vitamins

Vitamins are organic substances required by the body in small amounts for various metabolic processes. Vitamins may be synthesized in small or insufficient amounts in the body or not synthesized at all, thus requiring supplementation. They are classified as either fat soluble or water soluble. Vitamins A, D, E, and K are fat soluble, whereas biotin, folic acid, niacin, pantothenic acid, B vitamins (ie, B-1, B-2, B-6, B-12), and vitamin C are generally water soluble.

Vitamin deficiency may result from an inadequate diet, from increased requirements (eg, pregnancy, lactation), or secondary to disease or drugs. Vitamin supplements are used clinically for the prevention and treatment of specific vitamin-deficiency states.

FOLLOW-UP

Section 8 of 10  

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

Further Inpatient Care

• Because sudden death may occur in patients with scurvy, ensuring adequate vitamin C replenishment in patients with vitamin C deficiency is the hallmark of therapy. Restoration of body stores of vitamin C is essential to achieve complete resolution of symptoms. In most adult patients, provision of 250 mg of vitamin C 4 times a day for 1 week aids in achieving this goal.
• Provision of a balanced and liberal diet to meet the nutritional needs of the patient aids in recovery.
• Identifying and treating comorbid nutritional deficiencies (eg, iron deficiency anemia, folate deficiency, other vitamin deficiencies) are integral parts of management.

Further Outpatient Care

• Supervising the diet to ensure adequate intake of vitamin C to effect complete resolution and prevent recurrence.

Deterrence/Prevention

• Scurvy is a preventable disease. Provision of the required daily allowance of vitamin C in the diet or as a supplement prevents scurvy.

Complications

• Scurvy, if left untreated, can be fatal and has been associated with sudden death.
• Morbidity associated with hemorrhages depends on the site of involvement. Subperiosteal hemorrhages cause pain and tenderness resulting in pseudoparalysis. Loss of function at the site of the hemorrhage and anemia are typical sequelae of the hemorrhages observed in scurvy.

Prognosis

• Typically, scurvy carries an excellent prognosis if diagnosed and treated appropriately.

MISCELLANEOUS

Section 9 of 10  

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

Medical/Legal Pitfalls

• The bony pathology associated with scurvy could potentially be confused with physical abuse of a child. A good dietary history and the response to treatment with vitamin C establish the diagnosis and clear confusion. Nevertheless, in the developed world, vitamin C deficiency in children may be associated with neglect (ie, nutritionally inadequate diet) as a form of child abuse, even in the absence of overt signs of physical trauma.

REFERENCES

Section 10 of 10

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

• Akikusa JD, Garrick D, Nash MC. Scurvy: forgotten but not gone. J Paediatr Child Health. Jan-Feb 2003;39(1):75-7. [Medline].
• Barlow T. On cases described as 'acute rickets' which are probably a combination of scurvy and rickets, the scurvy being essential, and rickets a variable element. Med Chir Trans (London). 1883;66:159-220.
• Barness LA. Vitamin C (Ascorbic Acid) (Scurvy). In: Nelson Textbook of Pediatrics. 14th ed. Philadelphia, Pa: W B Saunders Company;1992:139-141.
• Bingham AC, Kimura Y, Imundo L. A 16-year-old boy with purpura and leg pain. J Pediatr. May 2003;142(5):560-3. [Medline].
• Carpenter KJ. The History of Scurvy and Vitamin C. New York, NY: Cambridge University Press;1986:1-288.
• Chaudhry SI, Newell EL, Lewis RR. Scurvy: a forgotten disease. Clin Exp Dermatol. Nov 2005;30(6):735-6. [Medline].
• Cheung E, Mutahar R, Assefa F. An epidemic of scurvy in Afghanistan: assessment and response. Food Nutr Bull. Sep 2003;24(3):247-55. [Medline].
• Greene HL. Disorders of the water-soluble vitamin B-complex and vitamin C. In: Textbook of Pediatric Nutrition. 2nd ed. New York, NY: Raven Press;1993:86-88.
• Hess AF. Scurvy, Past and Present. Philadelphia, Pa: JB Lippincott Company;1920:1-279.
• Jacob RA. Three eras of vitamin C discovery. Subcell Biochem. 1996;25:1-16. [Medline].
• Kocak M, Akbay G, Eksioglu M. Case 2: sudden ecchymosis of the legs with feelings of pain and weakness. Diagnosis: adult scurvy. Clin Exp Dermatol. May 2003;28(3):337-8. [Medline].
• Park EA, Guild HG, Jackson D. The recognition of scurvy with special reference to the early x-ray changes. Arch Dis Child. 1965;4:82-89.
• Ratanachu-Ek S, Sukswai P, Jeerathanyasakun Y. Scurvy in pediatric patients: a review of 28 cases. J Med Assoc Thai. Aug 2003;86 Suppl 3:S734-40. [Medline].
• Rosati P, Boldrini R, Devito R. A child with painful legs. Lancet. Apr 16-22 2005;365(9468):1438. [Medline].
• Sauberlich HE. Human requirements. Vitamin C status: methods and findings. Ann NY Acad Sci. 1975;258:438-450. [Medline].
• Truswell AS. Vitamin C (Ascorbic acid). In: Davidson's Principles and Practice of Medicine. 13th ed. New York, NY: Churchill Livingstone;1981:107-9.
• Wilson LG. The clinical definition of scurvy and the discovery of vitamin C. J Hist Med. 1975;30:40-60. [Medline].
• Woodruff CW. Ascorbic Acid-Scurvy. Prog Food Nutr Sci. 1975;1:493-506. [Medline].

Article Last Updated: Aug 15, 2006