eMedicine Specialties > Pediatrics: General Medicine > Nutrition

Scurvy

Bradley S Buckler, MD, Fellow in Neonatal-Perinatal Medicine, Medical College of Georgia
Anjali Parish, MD, Assistant Professor of Pediatrics, Department of Neonatology, Medical College of Georgia

Updated: Jul 16, 2009

Introduction

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. Use 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. Although 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.

Anteroposterior radiograph of the lower extremities shows ground-glass osteopenia, a characteristic of scurvy.

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 is rare 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.² Epidemic scurvy has been reported among refugee populations.

Mortality/Morbidity

Sudden death due to cardiac failure is reported in infants and adults with scurvy. Predominant morbidity is a result of hemorrhage into various tissues. Subperiosteal hemorrhage in the tibia and femur causes excruciating pain. Recent laboratory data suggest that the neonatal brain is particularly susceptible to vitamin C deficiency and that this condition may adversely affect early brain development.³

Race

No racial predilection is noted.

Sex

No sexual preponderance is noted.

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

History

• Initial symptoms of scurvy are nonspecific and include the following:
  • Loss of appetite
  • Peevishness (ill-tempered)
  • 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.
• Recently, an infant with diffuse nonscarring alopecia of the scalp and radiologic features of scurvy was reported.⁴

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

Differential Diagnoses

Other Problems to Be Considered

Acrodynia
Henoch-Schönlein purpura
Rheumatoid arthritis

Workup

Laboratory 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.
• WBC 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 Frãnkel. 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

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

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.

Ascorbic acid (Cecon, Ce-Vi-Sol, Dull-C)

Ascorbic acid (vitamin C) administered PO or parenterally effectively cures infantile and adult scurvy. Used by the body for collagen synthesis and tissue repair.

Dosing

Adult

100-200 mg PO q6h for 1 wk

Pediatric

25 mg PO q6h for 1 wk

Interactions

Large doses interfere with the absorption and metabolism of vitamin B-12; decreases effects of warfarin and fluphenazine; increases aspirin levels

Contraindications

Large doses may precipitate hemolysis in individuals with glucose-6-phosphate dehydrogenase deficiency; large doses are contraindicated in conditions aggravated by acid loading (eg, gout, renal tubular acidosis, cirrhosis, paroxysmal nocturnal hemoglobinuria)

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Large doses can cause renal stones and diarrhea; long-term high intake has been associated with metabolic tolerance/resistance and the expression, either clinically or biochemically, of vitamin C deficiency; for example, scurvy has been reported in infants born to mothers who had taken at least 400 mg/d of vitamin C during the entire pregnancy; healthy adults who take larger doses of vitamin C long term may manifest low serum ascorbic acid levels when they resume normal intake

Follow-up

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

Medicolegal 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.

Multimedia

Media file 1: Anteroposterior radiograph of the lower extremities shows ground-glass osteopenia, a characteristic of scurvy.

References

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15. 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].

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Keywords

scurvy, vitamin C deficiency, infantile scurvy, Barlow disease, Barlow's disease, Cheadle disease, Cheadle's disease, osteopathia hemorrhagica infantum, scurvy rickets, deficiency of ascorbic acid, impaired collagen synthesis, defective collagen, defective dentine formation, hemorrhaging

hemorrhaging into the gums, subperiosteal hemorrhage, pseudoparalysis, costochondral beading, scorbutic rosary, hyperkeratosis, corkscrew hair, sicca syndrome, whiteline of Frãnkel, treatment, diagnosis

Contributor Information and Disclosures

Author

Bradley S Buckler, MD, Fellow in Neonatal-Perinatal Medicine, Medical College of Georgia
Disclosure: Nothing to disclose.

Coauthor(s)

Anjali Parish, MD, Assistant Professor of Pediatrics, Department of Neonatology, Medical College of Georgia
Anjali Parish, MD is a member of the following medical societies: American Academy of Pediatrics and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center
Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

CME Editor

Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Jatinder Bhatia, MBBS, Professor of Pediatrics, Chief, Section of Neonatology, Department of Pediatrics, Medical College of Georgia
Jatinder Bhatia, MBBS is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Dietetic Association, American Federation for Clinical Research, American Pediatric Society, American Society for Clinical Nutrition, American Society for Parenteral and Enteral Nutrition, New York Academy of Sciences, Society for Pediatric Research, and Southern Society for Pediatric Research
Disclosure: Mead Johnson Consulting fee Consulting; Mead Johnson Honoraria Speaking and teaching; Dey LP Consulting fee Consulting; Dey LP Honoraria Speaking and teaching; Ovation Honoraria Speaking and teaching

The authors and editors of eMedicine gratefully acknolwedge the contributions of previous author Kumaravel Rajakumar, MD, to the original writing and development of this article.

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