Old World Meets Modern: A Case Report of ScurvyDepartment of Gastroenterology and Nutrition, Geisinger Medical Center, Danville, Pennsylvania Correspondence: Alan H. Wang, MD, Department of Gastroenterology and Nutrition, Geisinger Medical Center, 100 N. Academy Ave., Danville, PA 17822. Electronic mail may be sent to alanwang97{at}yahoo.com. Scurvy is a rarely seen disease resulting from a deficiency of vitamin C. We present a case of scurvy in a 65-year-old man. The patient reported heavy alcohol abuse over the last several years. He also reported that his diet consisted of cheese pizzas only. On physical examination, he was noted to have spontaneous ecchymosis of his lower extremities (denying any history of trauma); poor dentition; and corkscrew hairs on his chest, abdomen, and legs, with associated perifollicular petechia. Punch biopsy of his skin lesions revealed perivascular lymphohistiocytic inflammation, with some focal perifollicular erythrocyte extravasation. A serum ascorbic acid level was <0.12 mg/dL (normal range, 0.20–1.9 mg/dL). A diagnosis of vitamin C deficiency was made. The patient was successfully treated with 1 g/d vitamin C for the first 5 days, followed by a dose of 500 mg/d. Though scurvy is rarely seen in modern times, it is important to identify who is at risk and to recognize the clear and classic signs and symptoms associated with scurvy. Failure to diagnose this disease can potentially lead to expensive and unnecessary medical tests, as well as missing a very simple treatment that can prevent infection and even death. Scurvy is a disease originally described in ancient times that results from a deficiency of vitamin C. Because this ancient disease is rarely seen in modern times, we present a case of scurvy in an alcoholic man to review the clinical findings, diagnostics, and treatment of this disease.
A 65-year-old man was escorted to the emergency room by police for general poor health. The patient's neighbors informed the police that the patient lived alone and that his rental home was so dirty that the neighbors had noticed foul odors. The patient gave no medical history. He had not seen a medical doctor in over 20 years. He complained of 1–2 months of fatigue, loss of appetite, lethargy, and a progressive weight loss. He also complained of a 2-week history of lower-extremity swelling and easy bruising over his lower extremities. The patient denied any fevers, chills, nausea, vomiting, diarrhea, confusion, blurred vision or seizures, or any gastrointestinal bleeding. He denied any medication use, including aspirin, warfarin, anti-inflammatory medications, or clopidogrel bisulfate (Plavix, Bristol-Myers Squibb/Sanofi Pharmaceuticals, Bridgewater, NJ). The patient had not had any previous surgeries. The patient reported heavy alcohol abuse over the last several years. He consumed, on average, 10–15 drinks per day. He reported that his diet consisted of cheese pizzas only. He did not report any tobacco use or any illegal substance abuse or use. The patient also denied any recent trauma.
On physical examination, his vital signs were stable and he was alert and oriented to person, place, and time. His general appearance was that of an unkempt man, with long shoulder-length hair, who looked much older than his stated age. Examination of his head and neck revealed poor dentition, with gingivitis and contact bleeding of the gums. On skin examination, he was found to have corkscrew hairs over his chest, abdomen, and thighs (Figure 1). He also had severe ecchymosis and edema of his lower extremities, the right being worse than the left (Figures 2, 3). He had positive dorsal pedis, popliteal, and femoral pulses bilaterally. The areas were tender to palpation and had further contact ecchymosis after gentle palpation. Additionally, there were perifollicular petechias on his lower extremities. His chest, heart, abdominal, and neurologic examinations were unremarkable.
Initial laboratory analysis revealed a hemoglobin concentration of 6.3 g/dL, with a mean cell volume (MCV) of 95.6 and a platelet count of 252 K/µL. This normocytic anemia was attributed to bone marrow suppression from chronic alcohol consumption. A peripheral smear revealed slight anisocytosis, with moderate polychromasia and rare nucleated blood cells. Prothrombin time was 15.8 seconds, with an INR (international normalized ratio) of 1.46. This elevation of the INR was attributed to a nutrition deficiency of vitamin K. Basic serum chemistries revealed a blood urea nitrogen (BUN) of 45 mg/dL, creatinine of 1.9 mg/dL, and normal sodium, potassium, chloride, carbon dioxide, and glucose levels. The elevations of BUN and creatinine were attributed to dehydration on presentation. Liver function tests included a serum concentration of aspartate aminotransferase (AST) of 98 U/L, alanine aminotransferase (ALT) of 38 U/L, and an alkaline phosphatase of 41 U/L. The elevation of AST to more than 2 times the ALT is consistent with alcohol consumption. A serum ascorbic acid level was ordered and returned 9 days later; the level was <0.12 mg/dL, with the normal range being 0.2–1.9 mg/dL (Table 1).
The patient was admitted to the internal medicine inpatient service and a dermatologist was consulted for a biopsy of the above-noted skin lesions. Punch biopsy of his skin lesions revealed perivascular lymphohistiocytic inflammation, with some focal perifollicular erythrocyte extravasation. A diagnosis of vitamin C deficiency was made. The patient was treated with vitamin C at a dose of 1 g/d for the first 5 days and then 500 mg/d. The patient was also counseled and arrangements were made for the patient to enter an alcohol rehabilitation program. On a follow-up clinic visit 1 month after discharge, the patient had complete resolution of all his skin lesions.
Scurvy is a disease that was first described as early as 1500 BC in the Ebers papyrus.1 Through time, this disease was commonly found in groups of people living together in monasteries, traveling armies, and merchants, and the most severely affected were sailors. In 1499, on an expedition to India, Vasco da Gama lost almost two-thirds of his crew to scurvy.2 And in 1520, Magellan lost more than three-quarters of his crew while crossing the Pacific Ocean. For sailors, scurvy was responsible for more deaths than any other disease and disasters combined. Trying to decipher its etiology proved difficult for hundreds of years. Herman Boerhaave suggested that it was infectious.1 However, this theory could not explain why the upper class and the officers of naval ships never contracted scurvy after being exposed to it. Sir James Lind, who served as a surgeon for the British Navy, correctly identified the etiology, as well as the therapy, for scurvy. Aboard a ship, he conducted his own experiments, treating sailors afflicted by scurvy with either cider, vitriol, vinegar, sea water, or lemons and oranges. His experiments concluded that the lemons and oranges were the only effective treatment for scurvy.3 It was not until 1931 that ascorbic acid was found to be the key substance in citrus fruits that prevents and treats scurvy. The biochemists Albert Szent-Gyorgyi and Joseph Svirbely are credited with discovering ascorbic acid, or vitamin C. In modern times, the incidence of scurvy in industrialized countries is very rare. The last outbreak of scurvy was in 2002 in Afghanistan after a long season of drought. It was originally reported as a hemorrhagic illness that resulted in eventual deaths. But because scurvy is so uncommon, it was not initially suggested as a cause for the hemorrhagic illness.4 The clinical presentation of scurvy reflects the pathophysiologic role that vitamin C plays for collagen synthesis.5 In the early stages, between 3 and 6 months after vitamin C ingestion falls below 10 mg/d, symptoms can include fatigue, weight loss, irritability, generalized myalgias, and weakness. Later symptoms include the classic findings of petechiae, purpura, bleeding gums, hemarthroses (especially in children), corkscrew hairs, and poor wound healing.6,7 These findings are attributed to capillary fragility from a defect in a biochemical step of collagen synthesis. Cardiac manifestations are less common but include ST-segment elevations, as well as atrioventricular blocks. Eventually, if left untreated, scurvy is fatal. Death may follow infection or it may be sudden.8 Laboratory abnormalities include anemia usually hypochromic but possibly normocytic or even microcytic if there is associated blood loss. Usually, bleeding time, coagulation, and prothrombin times are normal; however, patients may have other nutrition deficiencies such as a vitamin K deficiency that could elevate the prothrombin time. A low plasma vitamin C or ascorbic acid level will confirm a clinical diagnosis of scurvy; generally, a level of <0.1 mg/dL confirms the diagnosis. However, patients with tuberculosis or rheumatic fever can also have low plasma levels of vitamin C.1,9 A vitamin C oral challenge can be administered, and if little vitamin C appears in the urine, scurvy should be considered. Furthermore, biopsies of petechial lesions classically will reveal extravasation of erythrocytes but can also show perivascular inflammation and may masquerade as leukocytoclastic vasculitis.8 Treatment is very simple. The recommended daily allowance (RDA) of vitamin C is 60 mg/d.10 Using the new dietary reference intake (DRI) guidelines, the recommendations are 75 mg/d for women and 90 mg/d for men.11 Additionally, stressors such as infection, trauma, hemodialysis, and smoking, increase the daily requirements. To treat scurvy, doses higher than the RDA are suggested and have been studied. Treating with 200 mg/d vitamin C has demonstrated improvement in symptoms within several days.12 However, there are also reports suggesting that 1 g/d for the first 3–5 days followed by 300–500 mg/d for another week can resolve symptoms in as fast as 3–5 days, with resolution of physical examination findings in 1–2 weeks.13,14 If vitamin C supplements are not available, consuming 5 servings of fruits and vegetables per day will exceed the RDA for vitamin C. Reports have also demonstrated that even in cases of severe late-stage scurvy that have progressed to multi-organ failure, there is a dramatic improvement with vitamin C therapy.15 Though scurvy is a rare and ancient disease, it should not be left in the pages of a history book. The clinical features, physical examination findings, laboratory confirmations, and treatments are classic, easy to recognize, and easy to administer. People at risk for scurvy include the economically challenged, the elderly, alcoholics, people living alone, young children, and inner-city inhabitants. Healthcare professionals need to recognize the signs and symptoms of scurvy, especially when they occur in at-risk populations. This may avoid unnecessary and expensive testing to rule out other diseases and allow for the faster implementation of a simple treatment that may be lifesaving.
Nutrition in Clinical Practice, Vol. 22, No. 4,
445-448 (2007) This article has been cited by other articles:
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