Sailing the Low C’s: History Repeating Itself

Introduction

With the advent of proper nutrition, hygiene, and preventive medicine, many previously common medical conditions have become increasingly rare. The following case of scurvy in an elderly patient demonstrates the importance of recognizing a historically important disease that is currently rarely diagnosed and is easily misdiagnosed or overlooked.

Case Presentation

An 81-year-old white man presented to his internist with the chief complaint of a painless, nonpruritic, nonpalpable rash of the lower extremity that had been present for 1 week. The patient also noted mild fatigue and memory loss; however, he was recovering from an aortic valve replacement that had been performed 3 months earlier. His postoperative course had been complicated by pneumonia, sepsis, respiratory failure, complete heart block requiring pacemaker placement, and delirium. Ultimately, after a 4-week stay in the hospital, he improved significantly enough to be transferred to a long-term care facility for rehabilitation. After a 4-week stay in the long-term care facility, the patient returned home in a greatly improved condition. At that time, his appetite was poor, but he had not lost weight and, despite the rash that had developed 1 week prior to his current presentation, he was doing reasonably well.

At his current presentation, the patient appeared comfortable and was not acutely ill. Physical examination revealed a nonpalpable perifollicular rash, which coalesced in areas and was distal to the knees (Figure 1). The rash was neither painful nor pruritic. His vital signs were all unremarkable and, other than a rash of the lower extremity, his overall status appeared unchanged since his discharge from the long-term care facility. The examiner was unable to elicit any significant medical or dietary history from the patient.

Laboratory analysis revealed a normal complete blood count, comprehensive metabolic panel, erythrocyte sedimentation rate, C-reactive protein level, and fluorescent antinuclear antibody test. The patient’s rash was concerning for vasculitis, so a dermatologic consultation was sought and a punch biopsy was taken.

Skin biopsy revealed hair follicles with cystic dilation and hair shaft fracture, as well as perivascular and perifollicular inflammation and extensive dermal extravasation of red blood cells, consistent with a diagnosis of scurvy (Figure 2 and Figure 3).

Discussion

Scurvy is a condition caused by a lack of vitamin C in the diet. The name of the disease is derived from the Latin word scorbutus.¹ It was described in the Egyptian medical writing Ebers Papyrus circa 1500 BC. Historically, scurvy typically affected sailors and other sea voyagers, as the supplies of perishable fruits and vegetables were commonly exhausted long before these persons were able to reach land and resupply. The condition killed more sailors than all other diseases and disasters combined between 1500 BC and 1800 AD.² Throughout history, many epidemics of scurvy have been documented; particularly well-known epidemics include those experienced during the travels of Vasco da Gama in 1499 and Magellan in 1520.1 In the modern era, scurvy is thought to largely affect alcoholics, food faddists (persons following unusual diets for varied reasons), and the severely malnourished.³ Recent studies have suggested that vitamin C deficiency and scurvy are likely underestimated, especially in the acutely ill elderly.⁴

The diagnosis of scurvy is primarily clinical and is supported by dietary history and resolution of symptoms with vitamin C treatment.^2,5 Serum ascorbic acid level testing is available, and a low level supports the diagnosis of scurvy, but is not diagnostic.³ In pediatric patients, x-rays of long bones are also helpful in making diagnosis.

Typical symptoms of scurvy include perifollicular hemorrhage, which may appear as a rash (as in the case patient); follicular hyperkeratosis; dystrophic hair lesions; delayed wound healing; old scar breakdown; gingival changes; easy bruising; joint and back pain; and anemia and bleeding, commonly manifested as epistaxis, gastrointestinal bleeding, and hemorrhage into the joints and soft tissues.^6-8 Early in the course of the disease, nonspecific symptoms such as fatigue, weakness, weight loss, myalgia, and fever may also occur.^4,6 Ultimately, if not treated, scurvy is fatal, with death usually resulting from infection or sudden death.⁶ In general, all manifestations of scurvy are related to the abnormal formation of the collagen triple helix, weakening tissues throughout the body.⁶

The differential diagnosis of scurvy is broad, as the presentation is nonspecific. The perifollicular hemorrhage and purpura seen in scurvy can be mistaken for hematologic disorders such as idiopathic thrombocytopenic purpura, thrombotic thrombocytopenic purpura, disseminated intravascular coagulation, clotting disorders, and primary hematologic malignancies.^6,7 There are many cutaneous disorders that can mimic scurvy, including medication side effects, vasculitis, cellulitis, and infections such as Rocky Mountain spotted fever and meningococcemia.^5,6 The mucosal symptoms of scurvy may be mistaken for Sjögren’s syndrome or acute gingivitis.^6,7

Studies have suggested that scurvy may not be as rare as previously thought, especially in acutely ill and elderly hospitalized patients.^4,9-13 One study identified clinical symptoms of scurvy in 18 of 145 patients admitted to a geriatric acute care ward.4 These symptoms were manifested as petechiae, perifollicular purpura and hyperkeratosis, extensive lower-limb purpura, and gingivitis. It has also been suggested that deficiency in vitamin C is common in acutely ill, hospitalized patients,^9-13 with one study finding biochemical vitamin C deficiency in 60% of new admissions.¹³

Treatment is, very simply, oral replacement of vitamin C. Numerous dosing regimens have been suggested³; in the case patient, physicians elected to begin therapy with vitamin C 500 mg daily. Over the course of 2 weeks, the rash on the patient’s lower extremity resolved completely. The patient is now maintained on vitamin C 250 mg daily, and continues to do well with no recurrence. Although we do not believe that the case patient manifested the symptoms of later-stage disease, it should be noted that even more advanced scurvy may respond well to vitamin C replacement therapy.¹

Conclusion

Although scurvy is generally thought to be rare in the developed world, it may be commonly unrecognized and is likely to be misdiagnosed if symptoms are detected. This can potentially result in a myriad of testing and a delay in simple, inexpensive, and safe therapy that is rapidly restorative, especially if initiated early in the course of disease.

Acknowledgment
The authors would like to thank Anna Gieschen for her assistance in searching the medical literature and Jason Meyer, MD, for his assistance in preparing the manuscript. The authors report no relevant financial relationships. Dr. Larson is from the Department of Internal Medicine, Dr. Jassim is from the Department of Pathology, and Dr. McGrann is from the Department of Dermatology, Sanford School of Medicine, University of South Dakota, Sioux Falls.

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