Scurvy

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Diagnosis and Differentials

Clinical

History:

• Symptoms of scurvy develop after 3 months of severe or total vitamin C deficiency.

• Patients may complain of weakness, fatigue, shortness of breath, and aching limbs. Left untreated scurvy progresses, with potentially fatal complications, including cerebral hemorrhage or hemopericardium.

• Infantile scurvy is uncommon before age 7 months, and clinical and radiographic manifestations rarely occur in infants younger than 3 months. Early clinical manifestations consist of pallor, irritability, and poor weight gain.

• In advanced infantile scurvy, the major clinical manifestation is extreme pain and tenderness of the arms and, particularly, the legs. The baby is miserable and tends to remain in a characteristic immobilized posture from subperiosteal pain, with semiflexion of the hips and the knees, as described by Thomas Barlow in 1884. The body is both wasted and edematous, and petechiae and ecchymoses are commonly present.

Physical:

• Symptoms and signs of scurvy may be remembered by the 4 Hs: hemorrhage, hyperkeratosis, hypochondriasis, and hematologic abnormalities. Patients may be miserable, irritable, depressed, resentful, and full of aches and pains. The body is both wasted and edematous, and petechiae and ecchymoses are commonly present.

• The earliest signs are found on the skin, often on the shins, after 3 months of severe or total vitamin C deprivation. Perifollicular hyperkeratotic papules are surrounded by hemorrhagic halos. The central hairs are twisted like corkscrews, and they may become fragmented. The posterior parts of the legs develop purpura that may coalesce.

• Soft, spongy swelling of the gums and gingival interdental papillae is followed by gingival hemorrhage, which is accentuated by coexistent poor oral hygiene and periodontal disease. Disrupted tooth formation and loosening of teeth may result in permanent defects of dentition.

• Ocular features include those of Sjögren's syndrome, subconjunctival hemorrhage, and bleeding within the optic nerve sheath. Funduscopic changes include cotton wool spots and flame-shaped hemorrhages.

• Bleeding into the joints causes exquisitely painful hemarthroses. Subperiosteal hemorrhage may be palpable, especially along the distal portions of the femurs and the proximal parts of the tibias of infants. In advanced cases, clinically detectable beading may be present at the costochondral junctions of the ribs. This finding is known as the scorbutic rosary. Bleeding into the femoral sheaths may cause femoral neuropathies, and bleeding into the muscles of the arms and the legs may cause woody edema.

• Heart complications include cardiac enlargement, ECG changes (reversible ST-segment and T-wave changes), hemopericardium, and sudden death.

• Anemia develops in 75% of patients, resulting from blood loss into tissue, coexistent dietary deficiencies (folate deficiency), altered absorption and metabolism of iron and folate, gastrointestinal blood loss, and intravascular hemolysis. The anemia is most often characterized as normochromic and normocytic. Vitamin C enhances iron absorption by reducing dietary iron from the ferric form to the ferrous form. Thus, vitamin C deficiency may reduce the availability of intracellular iron. Vitamin C is also necessary to convert folic acid to its active metabolite, folinic acid.

• Other problems include increased redness and swelling in recently healed wounds and the failure of new wounds to heal.

Causes:

• Scurvy is caused by a prolonged deficiency of vitamin C intake.

• Most animals can convert gluconate into ascorbate. Primates, including humans, and guinea pigs as well as a few other species cannot convert gluconate into ascorbate and, therefore, require exogenous ascorbic acid, otherwise known as vitamin C. Humans obtain 90% of their intake of vitamin C from fruits and vegetables, and cooking these sources decreases vitamin C content 20-40%. The US Food and Drug Administration recommends a daily dietary allowance of vitamin C of 75 mg for women and 90 mg for men.

• The total body pool of vitamin C is approximately 1500 mg. The absorbed vitamin is found ubiquitously in body tissues, with the highest concentrations in glandular tissue and the lowest concentrations in muscle and stored fat. Ascorbic acid is metabolized in the liver by oxidation and sulfation. The renal threshold for excretion by the kidney in urine is approximately 1.4 mg/100 mL plasma. Excess amounts of ascorbic acid are excreted unchanged or as metabolites. When body tissue or plasma concentrations of vitamin C are low, excretion of the vitamin is decreased. Scurvy occurs after vitamin C has been eliminated from the diet for at least 3 months and when the body pool falls below 350 mg.

Differentials

Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
Thrombophlebitis

Other Problems to be Considered:

Acute ulcerative gingivitis
Coagulation abnormalities
Collagen-vascular diseases
Deep vein thrombosis
Infection
Adverse medication effects
Platelet disorders
Septic arthritis
Systemic bleeding disorders
Trauma to legs and joints

Workup

Lab Studies:

• The diagnosis is mainly made on the basis of the historical features and the physical findings. Plasma ascorbic acid level may help in establishing the diagnosis, but this level tends to reflect the recent dietary intake rather than the actual tissue levels of vitamin C. Signs of scurvy can occur with low-normal serum levels of vitamin C.

• The level of vitamin C in leukocytes more accurately correlates to tissue stores compared with serum levels because these cells are not affected acutely by circadian rhythm or dietary changes. Recently, a specific and reproducible reverse-phase, high-pressure liquid chromatographic method has been found reliably to measure vitamin C in lymphocytes. This test is currently not clinically available but it might be useful for screening. A more commonly used method is the ascorbic acid tolerance test, which quantitates urinary ascorbic acid over the 6 hours following an oral load of 1 g of ascorbic acid in water. The best confirmation of the diagnosis of scurvy is still its resolution following vitamin C administration.

Imaging Studies:

• The earliest radiologic manifestation of infantile scurvy is generally seen at the distal ends of the radii where fuzziness of the lateral aspects of the cortices is present with slight rarefaction of the neighboring cancellous bone.

• As the disease progresses, radiographs demonstrate characteristic changes at the cartilage-shaft junctions of the long bones, especially at the distal ends of the femurs. Key imaging features show osteoporosis; increased density and widening of the zone of provisional calcification between the epiphysis and metaphysis (white line of Frankel); metaphyseal spurs or marginal fractures (Pelkan spur), a transverse band of radiolucency in the metaphysis (scurvy line or Trümmerfeld zone), which is subjacent to the zone of provisional calcification; ring of increased density surrounding the epiphysis (Wimberger ring); and periosteal elevation.

Histologic Findings: Noninflammatory perivascular extravasation of red cells and deposition of hemosiderin near hair follicles with intrafollicular keratotic plugs and coiled hair may be seen in a skin biopsy specimen.

• Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis)
• Thrombophlebitis

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