Scurvy: a rare cause of haemarthrosis

Background

Scurvy is an under-recognised disease caused by vitamin C deficiency which can affect multiple body tissues and present in unexpected ways. This report presents the case of a patient with left knee pain and swelling due to hemarthrosis who was subsequently diagnosed with scurvy.

Haemarthrosis can occur in any joint, but is most common in the knee.1 Acute haemarthrosis can cause pain, swelling and anaemia.1 Severe or recurrent episodes may destroy intra-articular cartilage leading to degenerative arthritis.1 Thus, it is important to elucidate the underlying cause of haemarthrosis before permanent damage occurs.

Though haemarthrosis has several aetiologies, trauma is the most frequent precipitant.1 In the knee, multiple components can bleed when injured. Tears of the anterior cruciate ligament are the most common cause of traumatic haemarthrosis, occurring in approximately 70% of cases.1 Other common injuries include patellar subluxations, meniscal tears, osteochondral fractures and posterior cruciate ligament tears.1 Haemarthrosis can also occur as a postoperative complication and is seen in 1% of knee arthroplasties.1 Atraumatic causes of haemarthrosis include primary coagulopathies (haemophilia), platelet dysfunction, dysfibrinogenaemia or hyperfibrinolysis, acquired bleeding disorders and anticoagulant therapy.1 Haemophiliacs have an estimated 50% lifetime risk of developing haemarthrosis.1 Other non-traumatic causes include septic arthritis, neuropathic arthropathy (Charcot joint), ruptured aneurysms and tumours near the joint space.1 Scurvy is among the least common causes of haemarthosis in the present day.1

Case presentation

A Caucasian male in his 60s with a medical history notable for hypothyroidism and locally advanced squamous cell carcinoma of the head and neck, which was treated with curative intent surgery and radiation several years prior, presented to the emergency department (ED) with left knee pain and swelling of 3 weeks duration. Soon after the onset of the symptoms, the patient visited the ED and received a dose of intravenous ceftriaxone and oral amoxicillin/clavulanate for presumed cellulitis. Despite these antibiotics his left knee pain and swelling worsened. In the interim, he also developed oral gum bleeding and large bruises on his arms and legs. The patient denied any recent trauma or prior history of joint swelling or prolonged bleeding after surgeries or dental extractions. The only relevant family history the patient reported was a mother diagnosed with iron-deficiency anaemia. Home medications were oral levothyroxine 125 mcg daily and naproxen 220 mg, used no more than twice daily as needed for pain. The patient was a retired army veteran who enjoyed aerobic dance as a hobby. The patient reported a diet of only one meal per day, usually dinner, typically consisting of chili without beans, tortillas and tortilla chips. Rarely, he would also eat a bag of preprepared salad; otherwise, he did not consume fruits or vegetables. He also reported high levels of oral water intake due to xerostomia, a complication of his prior cancer treatment.

In the ED, the patient’s vital signs were normal. On physical examination, he was tall with a thin build. He had poor dentition, but the remainder of the head and neck and cardiac, respiratory and abdominal examinations were unremarkable. His left knee was swollen and painful to palpation, with decreased active and passive range of motion. He had several large ecchymoses on his forearms and posterior thighs.

Investigations

Initial laboratory values included haemoglobin 91 g/L (9.1 g/dL) (130-173 g/L or 13–17.3 g/dL; normocytic), platelets 263 K/µL (140–400) and total bilirubin 1.7 mg/dL (0.1–1.2; predominantly indirect). The remainder of his complete blood count and metabolic panel were unremarkable. Imaging was performed to evaluate his knee swelling.

Treatment

Oral vitamin C supplementation and dietary modifications.

Outcome and follow-up

It was determined that vitamin C deficiency, due to poor intake of fruits and vegetables over several years had predisposed the patient to tissue injury and haemarthrosis. Because of xerostomia, a long-term effect of prior radiation treatment, the patient found fruits and vegetables difficult to swallow. A confounder to this diagnosis was his use of naproxen which, as a non-steroidal anti-inflammatory drug (NSAID), carries an increased risk of bleeding due to interference with platelet function.2 However, doses of naproxen less than 600 mg per day convey a very low bleeding risk, and the patient consumed less than 440 mg per day.3 Additionally, other signs of heavy NSAID use, such as mucosal or kidney injury, were absent. Given the clinical setting of scurvy and weakened collagen structure, it was hypothesised that a mechanical injury incurred while dancing was the cause of his haemarthrosis. For treatment the patient was given oral vitamin C supplementation of 1 g per day, an artificial saliva substitute, and was referred to a nutritionist. To aid with knee pain and swelling, he was instructed to follow conservative measures including modified activity and icing of the knee, and to take acetaminophen 1 g up to three times daily as needed. Subsequent serum vitamin C measurements at 4 and 6 weeks were 1.1 and 1.2 mg/dL (normal). Pertinent laboratory values over the course of his treatment are shown in table 1. At his 6-week follow-up appointment, knee pain and swelling had improved significantly, suggesting partial recovery. However, he still had evidence of scurvy including poor dentition, thin skin and extensive bruising as shown in photos taken at this visit (figure 2). The patient continues to regularly see his primary care physician every 6 months and his haematology–oncology physician annually. At these clinic visits, the patient’s current diet is reviewed and dietary recommendations are provided. He continues to take a multi-vitamin with vitamin C daily.