Poster Number-56 - A Forgotten Cause of Bleeding and Musculoskeletal Pain: A Case Report of Contemporary Scurvy

A Forgotten Cause of Bleeding and Musculoskeletal Pain: A Case Report of Contemporary Scurvy

Purpose:

Initial descriptions of scurvy, or vitamin C deficiency, date back to 1500 B.C., historically occurring in sailors, monks, and army men on long journeys with limited food access.¹ Scurvy resulted in more sailors’ deaths than all other diseases and disasters combined between 1500 BC and 1800 AD.² In 1747, Sir James Lind, a British naval surgeon, used lemons and oranges to treat sailors with scurvy.³ In 1931, the active substance in citrus fruits was identified as “hexuronic acid”, or vitamin C, and was renamed ascorbic acid because of its role in the prevention and treatment of scurvy.⁴ In 1937, Hungarian biochemist Albert von Szent-Györgyi won the Nobel Prize in physiology or medicine for this discovery.^5,6

With improvements in nutrition, hygiene, and preventive medicine over the last century, the incidence of scurvy decreased dramatically. Due to its rarity, scurvy is often misdiagnosed or underdiagnosed in current practice, though it continues to occur in vulnerable patient populations and is potentially fatal if left untreated.^1,7 Since the constellation of symptoms commonly includes musculoskeletal pain, pain providers may be faced with patients with underlying scurvy. We describe a case with contextualization within current literature to highlight disease findings that can help clinicians astutely make this diagnosis.

A 61-year-old female presented in March 2022 for evaluation of easy bruising, bleeding, arthralgias, myalgias, fatigue, loss of appetite with weight loss, and hypotension. Her past medical history included vertical band gastroplasty for obesity in 2001 requiring re-do and Roux-en-Y bypass in 2015 complicated by anastomotic ulcer and gastrointestinal (GI) bleeding. She also had right hemicolectomy with end ileostomy for ischemic right colon in 2018, chronic anemia, atrial fibrillation, chronic kidney disease stage 4, adrenal insufficiency, fibromyalgia, and depression. She was partially dependent on percutaneous-gastrostomy tube feeds for nutrition. Her outside dermatologic and hematologic workup for bruising and bleeding (in the post-operative and spontaneous settings) had been unrevealing, and her diffuse, daily, muscle and joint pains persisted despite acetaminophen, non-steroidal anti-inflammatory drugs (NSAIDs), and steroid injections, resulting in as needed oxycodone-acetaminophen use, impaired mobility, and intermittent wheelchair dependence. She was a retired phlebotomist and denied alcohol/substance use.



Methods:

Patient: 61-year-old woman with complex medical history including impaired nutritional and functional status

Setting: Outpatient hematology clinic

Consent: Patient provided verbal consent to use of de-identified patient information and images for medical research

Results:

Vital signs were notable for blood pressure of 84/54 mmHg. Body mass index was 30 kg/m². Physical examination showed diffuse purpura over the limbs with thin fragile skin, diffuse areas of small well-circumscribed ecchymoses in a similar distribution, and pedal edema.

Laboratory workup showed mild macrocytic anemia but otherwise normal blood counts, stable kidney function, normal electrolytes and normal liver function tests. Her comprehensive bleeding profile testing which included prothrombin time (PT), international normalized ratio (INR), activated partial thromboplastin time (aPTT), thrombin time, platelet function/aggregation testing, coagulation factors, and von Willebrand factor activity/antigen were unremarkable. We suspected scurvy as a vascular etiology of bleeding, and testing revealed low serum ascorbic acid level (0.2 mg/dL; normal range 0.4-2.0 mg/dL). Additional testing showed low zinc and low pyridoxic acid (vitamin B6). The patient was started on daily oral vitamin/mineral supplementation including ascorbic acid 1000mg daily, with referrals placed to GI and bariatric nutrition clinics in the interim.

Our patient had poor nutritional status and experienced bleeding, fatigue, and hypotension of unclear cause, with her arthralgias/myalgias previously attributed to fibromyalgia. Her case reminds us that scurvy is an underdiagnosed condition with a wide variety of manifestations. The most common findings are bruising, arthralgias, and joint swelling.⁸ Other reported symptoms in the literature include weakness, fatigue, myalgias, pedal edema, oral mucosal changes (swelling/bleeding), nausea, anorexia, and vasomotor instability. If untreated, scurvy can progress to heart failure and severe infections that can lead to death.

After one month of ascorbic acid supplementation, the patient demonstrated normalization of ascorbic acid level (0.5 mg/dl). Not all of her symptoms resolved, but her arthralgias, myalgias, and pedal edema improved and her weight stabilized. Fatigue and weakness remained significant and were felt to be driven by her comorbidities including adrenal insufficiency, debility, and poor oral intake. She did not have further bleeding and was cleared for further workup including endoscopy for her complex GI issues, provided she remained replete with vitamin C. She now follows with bariatric nutrition clinic to ensure appropriate vitamin levels.



Conclusion:

Scurvy occurs due to reduced dietary intake or absorption of vitamin C, thus presenting more commonly in patients with “fad diets”, reduced food access, alcoholism, GI disease or poor dentition, and patients on chemotherapy suffering from nausea/diarrhea.⁸ Vitamin C serves a variety of functions for the human body, including in collagen, carnitine, and norepinephrine synthesis. When defective, the abnormal synthesis of these products results in blood vessel fragility and poor wound healing (collagen), myalgias (carnitine), and vasomotor instability (norepinephrine), with reports of syncope and sudden death.⁸ A serum level of ascorbic acid below 0.4 mg/dl suggests scurvy.⁹ The signs and symptoms of scurvy rapidly respond to vitamin C supplementation. Subjective improvement in fatigue, pain, and anorexia typically occurs within 24 hours. Joint swelling and ecchymoses typically improve in days to weeks. Complete recovery occurs after about 3 months of treatment.¹⁰

Given the wide spectrum of scurvy manifestations, many of which can be dismissed as non-specific, it is important for providers of all specialties to recognize vitamin C deficiency. Given scurvy’s association with arthralgias and myalgias, pain providers can play an integral role in helping recognize scurvy and initiating appropriate therapy promptly.

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