Scurvy

Key Points:

  • Caused by nutritional deficiency of vitamin C
  • Patients may have petechiae, ecchymosis, bone pain, weakness, poor wound healing, and radiographic changes
  • Treatment is oral dietary vitamin C supplementation

Description:

Scurvy is a disease caused by nutritional deficiency of vitamin C (ascorbic acid). It is a rare condition, but sporadic reports still surface regarding children with unusual diets, such as those with anorexia nervosa, developmental delay or autism.

Epidemiology:

Scurvy is rarely observed in developed countries.

Clinical Findings:

Children with scurvy are irritable, may have petechiae, ecchymosis, bone pain, weakness, and poor wound healing.  Loss of appetite, irritability, and failure to thrive may be present.  The gums become bluish and swollen and may hemorrhage. Because Vitamin C is essential to the structure of collagen, all body parts are affected. Vascular fragility is a major concern in these patients.  Overall bone formation is reduced, and any bone that is formed lacks tensile strength.  The limbs may be swollen, bruised and tender.  In infants, pseudoparalysis may be present due to bone pain whereas older children may refuse to walk.

Imaging Studies:

Radiographic findings have been well described, and include osteopenia with cortical thinning. Bony changes are greatest during periods of maximum growth.  For this reason, infancy is the time period during which radiographic changes will be most impressive.  Classically, a line of radiodensity is apparent just on the metaphyseal side of the physis, called the line of Frankel.  (Figure 1) Wimburger’s sign is a radiodense ring around the epiphysis. Cupping of the distal femoral metaphysis has been reported. Subperiosteal hemorrhage along the femur, tibia, or humerus may occur and calcify with treatment.


Figure 1a: A 13-year-old male with severe autism and very restricted diet presented with refusal to walk.  Radiographs of the knee demonstrate osteopenia, physeal widening, and a sclerotic band in the distal femoral metaphysis (the “line of Frankel”).   


Figure 1b: MRI of the knee (sagittal and coronal STIR) noted abnormal heterogenous increased T2 signal in the distal femoral and proximal tibial metaphysis.  The physis is wide and striated in appearance.  The patient was subsequently diagnosed with scurvy and the symptoms improved with vitamin C supplementation.  (Images courtesy of Melissa Hilmes, MD)

Etiology:

Scurvy is caused by a deficiency of vitamin C, which cannot be synthesized de novo in humans. The basic defect is failure of hydroxylation of proline and lysine, an essential step in collagen formation, which is dependent on Vitamin C.  As a redox agent, Vitamin C protects DNA, protein, and vessel walls from damage due to free radicals.
 
Infants whose intake includes only cow’s milk for the first year of life will display symptoms.  In certain segments of the population such as refugees, scurvy may still be evident.  The body’s concentration of Vitamin C can be depleted in as short a time period as 1-3 months.  Certain individuals have higher requirements of Vitamin C including smokers, and patients with Type I diabetes, AIDS, iron overload disorders, renal failure requiring hemodialysis, or with diseases affecting the small intestine.

Treatment:

Treatment is oral dietary vitamin C, ascorbic acid.  Scurvy may be avoided by adequate daily vitamin C intake: infants 25 mg/day, children 30 – 40 mg/day, adults 40-75 mg /day.  (Kim, 2014) Intoxication does not occur as vitamin C is not fat soluble.
 

Complications:

Untreated, scurvy may result in failure to thrive, anemia, hypertension, and poor wound healing.

References:

  1. Hallel T, Malkin C, Garti R. Epiphyseometaphyseal cupping of the distal femoral epiphysis following scurvy in infancy. Clinical Orthopaedics & Related Research 1980(153): 166-8.
  2. Kim HK. Metabolic and Endocrine Bone Diseases.  In: Herring JA, editor.  Tachdjian’s Pediatric Orthopaedics. Philadelphia: Elsevier; 2014. P e582-e642.
  3. McKenna KE, Dawson JF. Scurvy occurring in a teenager. Clinical & Experimental Dermatology 1993; 18(1): 75-7.
  4. Nerubay J, Pilderwasser D. Spontaneous bilateral distal femoral physiolysis due to scurvy. Acta Orthopaedica Scandinavica 1984; 55(1): 18-20.
  5. Quiles M, Sanz TA. Epiphyseal separation in scurvy. Journal of Pediatric Orthopedics 1988; 8(2): 223-5.
  6. Sprogue PL. Epiphyseo-metaphyseal cupping following infantile scurvy. Pediatric Radiology 1976; 4(2): 122-3.
  7. Tamura Y, Welch DC, Zic JA, Cooper WO, Stein SM, Hummell DS. Scurvy presenting as painful gait with bruising in a young boy. Archives of Pediatrics & Adolescent Medicine 2000; 154(7): 732-5.
  8. Zaleske DJ. Metabolic and endocrine abnormalities. In: Morrissy RT, Weinstein SL, editors. Pediatric Orthopaedics. Philadelphia: Lippincott-Raven; 1996. p. 137-201.

Top Contributors:

Robert F. Murphy, MD