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. 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
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
More on Scurvy |
 Overview: Scurvy |
| Differential Diagnoses & Workup: Scurvy |
| Treatment & Medication: Scurvy |
| Follow-up: Scurvy |
| References |
| Next Page » |
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].
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, siccasyndrome,whiteline ofFrãnkel
