eMedicine Specialties > Pediatrics: General Medicine > Nutrition

Scurvy

Author: Bradley S Buckler, MD, Fellow in Neonatal-Perinatal Medicine, Medical College of Georgia
Coauthor(s): Anjali Parish, MD, Assistant Professor of Pediatrics, Department of Neonatology, Medical College of Georgia
Contributor Information and Disclosures

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 extremiti...

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

Anteroposterior radiograph of the lower extremiti...

Anteroposterior radiograph of the lower extremities shows ground-glass osteopenia, a characteristic of 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. Guidelines for the evaluation of fractures in infants and young children have been established.1 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 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

More on Scurvy

Overview: Scurvy
Differential Diagnoses & Workup: Scurvy
Treatment & Medication: Scurvy
Follow-up: Scurvy
Multimedia: Scurvy
References

References

  1. [Guideline] Jenny C. Evaluating infants and young children with multiple fractures. Pediatrics. Sep 2006;118(3):1299-303. [Medline].

  2. 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].

  3. Tveden-Nyborg P, Lykkesfeldt J. Does vitamin C deficiency result in impaired brain development in infants?. Redox Rep. 2009;14:2-6. [Medline].

  4. Ragunatha S, Inamadar AC, Palit A, Sampagavi VV, Deshmukh NS. Diffuse nonscarring alopecia of scalp: an indicator of early infantile scurvy?. Pediatr Dermatol. Nov-Dec 2008;25(6):644-6. [Medline].

  5. Akikusa JD, Garrick D, Nash MC. Scurvy: forgotten but not gone. J Paediatr Child Health. Jan-Feb 2003;39(1):75-7. [Medline].

  6. 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.

  7. Barness LA. Vitamin C (Ascorbic Acid) (Scurvy). In: Nelson Textbook of Pediatrics. 14th ed. Philadelphia, Pa: W B Saunders Company; 1992:139-41.

  8. 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].

  9. Carpenter KJ. The History of Scurvy and Vitamin C. New York, NY: Cambridge University Press; 1986:1-288.

  10. Chaudhry SI, Newell EL, Lewis RR. Scurvy: a forgotten disease. Clin Exp Dermatol. Nov 2005;30(6):735-6. [Medline].

  11. 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].

  12. 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.

  13. Hess AF. Scurvy, Past and Present. Philadelphia, Pa: JB Lippincott Company; 1920:1-279.

  14. Jacob RA. Three eras of vitamin C discovery. Subcell Biochem. 1996;25:1-16. [Medline].

  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].

  16. Park EA, Guild HG, Jackson D. The recognition of scurvy with special reference to the early x-ray changes. Arch Dis Child. 1965;4:82-9.

  17. Rosati P, Boldrini R, Devito R. A child with painful legs. Lancet. Apr 16-22 2005;365(9468):1438. [Medline].

  18. Sauberlich HE. Human requirements. Vitamin C status: methods and findings. Ann NY Acad Sci. 1975;258:438-450. [Medline].

  19. Truswell AS. Vitamin C (Ascorbic acid). In: Davidson's Principles and Practice of Medicine. 13th ed. New York, NY: Churchill Livingstone; 1981:107-9.

  20. Wilson LG. The clinical definition of scurvy and the discovery of vitamin C. J Hist Med. 1975;30:40-60. [Medline].

  21. Woodruff CW. Ascorbic Acid-Scurvy. Prog Food Nutr Sci. 1975;1:493-506. [Medline].

Further Reading

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

 
 
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