August 2006
Fig.6
Diagnosis: Scurvy
Labs
Plasma Ascorbic Acid level <0.1 (normal range of 0.6-2.0)
Histopathology: Left leg biopsy showed normal epidermis, with superficial perivascular inflammatory infiltrate composed of lymphocytes, histiocytes, occasional plasma cell and eiosinophills (Figures 6,7). There was no evidence of leukocytoclastic vasculitis. No perifollicular fibrosis or follicular plugging was noted.
Discussion:
Vitamin C deficiency, better known as scurvy has been known
for centuries as a potentially fatal disease. In 1747 the British
navy officer James Lind, recognized that scurvy could be cured
by eating oranges or lemons. Albert Szent-Györgyi and Charles
G. King in the early 20th century independently extracted hexuronic
acid that is now known as vitamin C. Vitamin C was the first
vitamin to be artificially synthesized in 1935 by the Polish-Swiss
endocrinologist, Dr. Tadeusz Reichstein.
Vitamin C is a reducing agent/electron donor or antioxidant
needed for reduction of metal ions in several human enzymatic
pathways, three of which participate in collagen hydroxylation
and two in carnitine biosynthesis. The formation of hydroxyproline
is important for the stability of the triple-helix structure of
collagen in skin and other tissues. Carnitine is an amino acid
responsible for the transport of fatty acids and hence vitamin
C plays a role in fat storage and metabolism.
In addition, Vitamin C also stabilizes folate in food and in plasma.
A deficiency of both results in anemia. Vitamin C also promotes
absorption of soluble non-heme iron possibly by chelation or simply
by maintaining iron in its reduced form, which explains why deficiency
of Vitamin C might result in iron deficiency anemia.
The incidence of scurvy in the United States is rare, affecting mostly elderly, alcoholic and indigent patients. Other risk groups include individuals with anorexia nervosa, type I diabetes, the renal dialysis population, and patients with small bowel disease where vitamin C is absorbed. In adults, scurvy presents 3 to 6 months after vitamin C consumption in the diet falls to less than 10 mg/day. Symptoms can be preceded by lassitude, weakness, irritability, weight loss, and vague myalgias and arthralgias. Skin findings include perifollicular hyperkeratotic papules, corkscrew hairs, splinter hemmorages of the nails, perifollicular hemorrhages, purpura, and ecchymoses. Other common findings include: gum swelling, friability, bleeding, mucosal petechiae; and pale conjunctiva. Conjunctival hemorrhage, flame-shaped hemorrhages, and cotton-wool spots may be seen. Systemic effects include high-output heart failure due to anemia and submucosal hemorrhages in gastrointestinal tract. Anemia develops in 75% of patients, secondary to multiple factors including altered absorption of iron and folate, gastrointestinal blood loss, and intravascular hemolysis. Bleeding in the muscles and joints which can be quite painful, has been observed.
Diagnosis
Plasma ascorbic acid falls from the normal range of 0.6 to
1.4 mg/dL to less than 0.2 mg/dL. Ascorbic acid levels in the
WBC-platelet layer of centrifuged blood are more significant;
normal levels more than 16 µg/108 cells are reduced to less
than 2.0 µg/108 cells.
A capillary fragility test, also known as the Rumpel-Leede test,
can be performed at the bedside where a pressure tourniquet is
applied above the elbow, and pressure midway between the systolic
and diastolic is applied for 15 minutes, after which the tourniquet
is removed. The appearance of greater than 20 petechial lesions
on the forearm is abnormal.
Radiographic studies of the joints and long bones can aid in
the diagnosis with characteristic findings of cortical thinning,
which is sometimes described as a pencil-point cortex, and decreased
radiopacity.
Treatment
Supplementation with Vitamin C 100 mg 3-5x/day until
a total of 4g is reached then 100mg qd results in quick resolution
of symptoms. Patients should also be encouraged to have diet rich
in fruits and vegetables such as grapefruit, lemons, broccoli,
green peppers, tomatoes, and cabbage.
References:
1. Andrew's Diseases of the Skin (10th Edition). Elsevier Inc,
2006.
2. Dermatology. Jean Bolognia, Joseph L Jorizzo, Ronald P Rapini.
Elsevier Inc., 2003.
3. Carpenter KJ: The History of Scurvy and Vitamin C. New York,
NY: Cambridge University Press; 1986:1-288.
4. The Merck Manual of Diagnosis and Therapy. 18th edition.
2006.
Presented by Drs. Liana Abramova, Margaret Mann and Beatriz Tapia.
