56-Year-Old Homeless Alcoholic

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This 56 year-old homeless, alcoholic male presented to your emergency department complaining of generalized malaise, diffuse bony aches and a rash to his lower extremities. The symptoms had been present for 2 weeks without any fever, chills or known sick contacts, and the rash was noticed 1 week ago and was progressing. It was nonpruritic, and the patient denied any traumatic event or prior history of a similar rash. The patient was malnourished but alert and oriented. His vital signs were stable. His exam was otherwise insignificant except for diffuse muscle/bony aches and a lower extremity rash (figures 1 and 2).

Figure 1 (click on image to enlarge)
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Figure 2 (click on image to enlarge
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What are your concerns as you examine this patient?

Dx: Scurvy

The patient was diagnosed with scurvy, a dietary disorder of vitamin C deficiency. Scurvy was first described by a Dutch physician in 1541, although the earliest recorded symptoms of this disease were documented in 1550 BC by the Egyptians. Afflicting many a sea explorer in the 16-18th century, a British naval surgeon named James Lind demonstrated in 1746 that oranges and lemons (hence the name limeys) were effective in not only preventing but also curing scurvy.

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Infantile scurvy was first described by Francis Glisson in the 17th century, and in1650, he was credited with the discovery of the simultaneous occurrence of scurvy and rickets in infants . Vitamin C is important for many metabolic processes in humans including collagen synthesis, hematopoiesis by promoting iron absorption, resistance to infection and the biosynthesis of carnitine and neurotransmitters. Because humans cannot synthesize vitamin C, this water-soluble vitamin must be included in one’s dietary regimen.

altAlthough rare in the United States, scurvy is more commonly seen in underdeveloped nations. Scurvy can occur in infants (uncommon in the neonate) and adults. Infantile scurvy was common in the United States during the 1950’s. Infants are prone to this disorder when they are exposed to pasteurized milk (due to the loss of vitamin C) or diets deficient in vitamin C. In addition, infants fed evaporated or condensed milk formulas are also more prone to scurvy.

Chronic alcoholics, elderly patients with poor diets, malabsorption, inflammatory bowel disease, cigarette smoking, patients on chemotherapy and people using fad diets are at risk. With a total body pool of 1500mg of vitamin C, it takes several months (usually 1-3 months) for signs and symptoms of scurvy to develop. At this point the body pool is usually below 350 mg.

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The daily requirement of vitamin C range from 30-40mg in infants to 45-60mg in children and adults, to 90-95 mg in lactating mothers. Significant amounts of vitamin C can be found in the following food sources: citrus fruits, broccoli, spinach, potatoes, tomatoes, cabbage, and berries.

Patients may complain of shortness of breath, aching limbs, fatigue and weakness. The earliest physical signs of scurvy usually involve the skin, especially the anterior tibial regions, which present with perifollicular hyperkeratotic papules surrounded by hemorrhagic halos. Purpura, which may coalesce, are often found on the posterior aspects of the legs. Central hairs become twisted and are often fragmented (corkscrew hairs). Spongy swelling of the gums precedes gingival petechiae and hemorrhage and permanent dental defects consisting of tooth loosening and infection occur.

Hemorrhage is a hallmark feature of scurvy and may occur in any organ or tissue. Cotton wool spots and flame hemorrhages as well as subconjunctival hemorrhages can occur. Sudden death, cardiomegaly, and hemopericardium as well as reversible nonspecific ST T-wave changes may occur. Dry mucosa and eyes may occur similar to patients having Sjogren’s syndrome.

Hematologic findings may include a normochromic normocytic anemia, painful hemarthrosis and deranged absorption and metabolism of folate and iron. Finally, poor wound healing may be present. Patients may be depressed, irritable and complain of diffuse body aches and pains.  Late stage changes include fever, neuropathy, jaundice, anasarca and seizures. Hemorrhaging into various tissues and organs is the usual cause of mortality.

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Treatment with either parenteral or oral vitamin C administration results in a relatively quick and effective cure of scurvy.

This patient had a serum ascorbic acid concentration of less than 0.10mg/dL, a level consistent with a significant deficiency in vitamin C. While levels of 0.02 mg/dL are nutritionally acceptable, a fasting level greater than 0.6 mg/dL rules out scurvy. In addition, he was noted to have chronic thrombocytopenia and a normochromic normocytic anemia. He responded well to oral vitamin C (initially 200 mg p.o. Q6h for 1 week) and was discharged to a long-term care facility.

REFERENCES
1. Carpenter KJ. The History of Scurvy and Vitamin C. New York, NY: Cambridge University Press; 1986:1-288
2. Reuler JB, Broudy VC, Cooney TG. Adult scurvy. JAMA 1985;253:805-7.
3. Oeffinger KC. Scurvy: more than historical relevance. Am Fam Physician 1993;48:609-13.

ABOUT THE AUTHOR

Dr. Effron is the assistant professor of emergency medicine at Case Western Reserve University and attending physician in the department of emergency medicine at the MetroHealth Medical Center, in Cleveland, Ohio.

1 Comment

  1. Wow, what an interesting way to write the article. On page one I was guessing my heart out, trying to diagnose this person, but other than what looked like heavy internal bleeding, I could not diagnose anything. Scurvy – very interesting. Wonder if he will keep getting Vitamin C when he is back on the streets.

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