Unrecognised scurvyBMJ 2009; 339 doi: http://dx.doi.org/10.1136/bmj.b3580 (Published 17 September 2009) Cite this as: BMJ 2009;339:b3580
- Clarisa T P Choh, CT2 in general surgery1,
- S Rai, clinical fellow 1,
- M Abdelhamid, research fellow1,
- W Lester, consultant haematologist2,
- R K Vohra, consultant vascular surgeon1
- 1Department of Vascular Surgery, University Hospitals Birmingham NHS Foundation Trust, Selly Oak, Birmingham B29 6JD
- 2Department of Haematology, University Hospitals Birmingham NHS Foundation Trust, Edgbaston, Birmingham B15 2TH
- Correspondence to: C Choh
- Accepted 30 April 2009
Scurvy, first described by Hippocrates, has troubled sailors and soldiers since 460 BC, and consumption of citrus fruit was shown to be a cure by James Lind, a Scottish naval surgeon.1 Scurvy is a deficiency of vitamin C and commonly occurs in people with poor social status, malnutrition, and alcoholism, especially in those with peculiar dietary habits.2 3 It is thought to be rare in the developed world, but emerging literature has shown otherwise.4 5 6 Poor vitamin C status is relatively common in the United Kingdom, especially in adults living on a low income, with a prevalence of 46% in men and 35% in women.4 Scurvy has also been described in reports from the United States,7 Canada,8 Spain,9 and Italy.10 Patients usually present with fatigue, gum swelling or bleeding, and skin discolouration.7 11 12
Here, we discuss a case of a young man who presented with unilateral leg swelling and pigmentation, in association with other symptoms such as gastrointestinal bleeding and epistaxis, which resolved after the oral administration of vitamin C.
A 30 year old white law clerk presented to the orthopaedic team with a two week history of non-traumatic left leg swelling and bruising. It had started with pain and swelling on the medial aspect of the left knee, which progressed to extensive bruising and swelling on the posteromedial aspect of the left thigh and calf. He was a non-smoker with no relevant medical history and was not on any medication. He looked well, and examination was unremarkable. His haemoglobin level was 105 g/l, mean cell volume 78 fl, mean cell haemoglobin 26 pg, with no thrombocytopaenia. A colour-flow Duplex-Doppler ultrasound excluded deep vein thrombosis but detected tissue oedema. He was discharged with ruptured left gastrocnemius muscle as a provisional diagnosis.
A fortnight later he presented to the medical assessment unit after a follow-up blood test arranged by his general practitioner showed a haemoglobin level of 37 g/l. He reported breathlessness, with no history of haematemesis, haemoptysis, or melaena, but he mentioned frequent episodes of epistaxis that resolved spontaneously after his first admission. On examination, he had generalised swelling and bruising of his left leg with a full complement of palpable pulses. No other bruises or petechiae were found on the rest of the body. His laboratory investigations showed that platelet count, prothrombin time, activated partial thromboplastin time, fibrinogen concentration, and renal function were all normal, but that his D-dimer concentration was raised at 2559 ng/ml.
On this admission, a repeat venous Duplex-Doppler ultrasound of the left leg showed a haematoma in the left distal thigh and deep vein thrombosis in the superficial femoral vein extending down to the ankle. Another repeat ultrasound by a consultant radiologist excluded evidence of deep vein thrombosis, and therefore anticoagulation was not started. Despite multiple blood transfusions, the patient’s haemoglobin level stayed low. A gastroscopy revealed multiple duodenal ulcers, which were injected with adrenaline, and triple therapy with amoxicillin, clarithromycin, and omeprazole was started for Helicobacter pylori infection.
Since the patient’s haemoglobin level remained low, between 65 g/l and 75 g/l, and a new onset of gum bleeding was noted, he was referred to gastroenterology and haematology. Meanwhile, an immune mediated haemolytic anaemia was excluded by vasculitic screen and Coombs test. Meckel’s scan for ectopic gastric mucosa was negative. A bone marrow biopsy was normal apart from showing mild erythroid hyperplasia consistent with his recent history of blood loss. Scurvy was then considered as a differential diagnosis, as further questioning revealed that the patient’s diet was deficient in fruits or vegetables. Given the symptom presentation of epistaxis, gum bleeding, and haemorrhage in the lower limbs, oral supplementation with vitamin C was started. Subsequently, his haemoglobin level improved to 85 g/l, and he had no further symptoms on follow-up. This was a diagnosis of exclusion, as no confirmatory investigation such as serum ascorbic levels was available.
This patient’s anaemia was secondary to gastrointestinal and limb haemorrhage, which, together with recurrent epistaxis and gum bleeding, was due to scurvy.
Scurvy is caused by a deficiency of vitamin C (ascorbic acid), a nutrient that is abundant in citrus fruits, green vegetables, tomatoes, and peppers13 and that is essential for normal collagen formation.11 Unlike many other animals, humans cannot synthesise the vitamin, so a deficiency, most often because of poor diet, can lead to abnormal collagen formation. Abnormal collagen formation leads to increased vascular fragility, which results in extravasation of red blood cells into the skin, especially in the legs where hydrostatic pressure is highest. Smokers have greater vitamin C requirements than non-smokers, which predisposes them to scurvy.14 15 However, the common factor described in the literature was that of a particular diet,16 as in our patient’s case.
Patients with a mild form of scurvy may initially present with fatigue, nausea, and weight loss.17 Common clinical signs are gingival swelling,7 poor wound healing,17 skin discolouration, and follicular hyperkeratosis16—excess keratin around hair follicles that results in skin eruptions.18 Scurvy can also present as purpuric swelling on the abdominal wall,9 gastrointestinal haemorrhage, bleeding into the soft tissue and joints, haemorrhagic ulceration of the lower limbs,19 or rarely, compartment syndrome of the leg.8
Diagnosis is based on history and clinical findings, such as poor intake of food rich in vitamin C, and examination findings of cutaneous haemorrhagic lesions on the limbs or body. Skin biopsy may be performed, but it will only exclude vasculitis.10 16 Adults require 40 mg/day of vitamin C13 and concentrations in serum should be 4-15 mg/l.(11) Measurement of serum level of ascorbic acid before and after treatment, although seldom done, can confirm the diagnosis when symptoms improve or resolve within weeks.(7) (11) However, serum measurements may not correlate well with levels in tissue.20
Scurvy is unusual yet important, and delayed diagnosis can have serious consequences such as gastrointestinal or lower limb haemorrhage. Treatment is simple, with oral supplementation of ascorbic acid 300-400 mg daily, maintained with a daily intake of fruits and green leafy vegetables.13 As patients with scurvy are often deficient in other nutrients, close attention is needed to prevent the development of refeeding syndrome, which is a result of profound hypophosphataemia and is common in patients after prolonged starvation. Refeeding syndrome can produce rhabdomyolysis, hypotension, arrhythmias, seizures, and may result in multiorgan failure and death in 0.43% to 34% of these patients if untreated.21 22 Therefore electrolytes, especially serum phosphate levels, need to be monitored at least three times a week during hospital treatment and managed according to National Institute for Health and Clinical Excellence guidelines.23
Cite this as: BMJ 2009;339:b3580
Acknowledgments: We thank the Department of Vascular Surgery at Selly Oak Hospital for their support in this report.
Contributors: CTPC collected, analysed, and interpreted the data, drafted the article, and critically revised it, and had final approval of the version to be published. SR analysed and contributed to the final diagnosis, interpreted data, critically revised the article, and had final approval of the version to be published. MA critically revised the article and had final approval of the version to be published. WL contributed to the final diagnosis and treatment of the case, critically revised the article, and had final approval of the version to be published. RKV conceptualised the information, critically revised the article, and had final approval of the version to be published.
Competing interests: None declared.
Patient consent obtained.
Provenance and peer review: Not commissioned; externally peer reviewed.