Approach
The diagnosis of scurvy is often elusive because symptoms, particularly early in the disease, are often non-specific; the diagnosis is not usually considered, owing to its rarity; and dietary history may not be elicited during initial evaluation. The diagnosis may escape detection for a prolonged period until more symptoms manifest themselves or the patient becomes severely ill.
Individual and environmental history
Scurvy is always due to insufficient consumption of vitamin C. It is important to identify historical factors that may contribute to low intake. Environmental influences include:
Famine
Poor economy with populations unable to purchase foods with sufficient vitamin C
War
Government policy, which can further influence these factors
Individual factors include:[1][3][7][16][17][18][19][20][39][40][43][44][45][52]
Behavioural or cognitive reasons leading to incomplete intake - atypical or idiosyncratic dieting; anorexia nervosa; impaired cognition or diminished hunger drive in older patients; or recent widowhood in patients previously dependent on their partner for food preparation
Alcohol use disorder
Living alone
Low income
Increased catabolism (e.g., in chronic infection, critical illness, acute respiratory distress syndrome, pancreatitis, patients who smoke)
Renal dialysis (because vitamin C is dialysed)
Children may additionally be at risk if their adult carers determine their diets and are constrained by the socioeconomic influences outlined above, or if they are fed cow's milk during infancy. Children with static encephalopathy, autistic spectrum disorder, depression, or idiosyncratic diets may also be at risk.
Dietary history
A detailed dietary history may obviate invasive or unnecessary diagnostic tests and shorten the time to treatment, and should include the contents of the diet with particular attention to absence of fruit, vegetables, non-cow animal milk, or organ meats (liver and kidney), and the time frame of the diet relative to the illness presentation. Given that scurvy is always due to insufficient consumption of vitamin C, symptoms of scurvy should follow a period of relative dietary inadequacy. Note that the likelihood is very low that a diet containing fruit and vegetables only from frozen or canned preparations will lead to scurvy.
Some patients cannot or will not give a full dietary history, and risk factors for scurvy should therefore be identified. Other concomitant dietary deficiencies may occur in patients presenting with scurvy and should increase the diagnostic suspicion.
Symptoms
Clinical manifestations of scurvy typically occur when body stores of vitamin C drop below 300 mg, but can begin when stores are as high as 490 mg. Initial signs of scurvy can appear as early as 29 days after the last vitamin C intake, with a more evident syndrome occurring after 2-4 months: a typical time of presentation when a diet is completely devoid of vitamin C.[2] Time to presentation may be longer if vitamin C is intermittently consumed yet overall dietary intake is deficient.
Early presenting symptoms may be vague, non-specific, or constitutional.[1][2][18] These may include fatigue, lassitude, weakness, nausea, emesis, weight loss, dry skin, depression, dyspnoea, and dysphagia. As vitamin C levels drop, the disease progresses and symptoms of endothelial dysfunction and haemorrhage develop, including easy bruising or bleeding, myalgia, arthralgias, limb pain, and joint swelling.[60] Poor wound healing may also be evident but overlooked by the patient.
Patients may present at any phase of disease, and outlining a progression of symptoms may be an important diagnostic clue.
Infants and children may present with other symptoms, most likely related to impaired bone growth (including bone and joint pain), gait impairment, or refusal to walk.[60]
Examination
Although no consistent order of presenting signs is established, the earliest signs of scurvy are often gingival abnormalities, and a comprehensive examination of the mouth when scurvy is suspected may be of the highest yield in patients presenting relatively early.
All signs associated with scurvy are related to endothelial dysfunction, leading to fluid extravasation and frank haemorrhage, and include the following.
Oral: mucosal changes (discoloration, petechiae, gum swelling); dental problems (loose teeth with haemorrhagic gingivitis affecting interdental papillae, though gums are typically normal in edentulous patients); and xerostomia as part of sicca-like or Sjogren-like syndrome.[1][18]
Dermatological: petechial and perifollicular haemorrhages (particularly of the legs and feet), poor wound healing, bruising, follicular hyperkeratosis, coiled hairs, alopecia and nodular or black ecchymoses at non-traumatic sites.
[Figure caption and citation for the preceding image starts]: Corkscrew hair and perifollicular petechiae which are commonly seen in scurvyShah V, et al. Case Rep Med 2021 Mar 30; 2021: 5519937; used with permission [Citation ends].
Ophthalmological: haemorrhages and petechiae in skin of lid; proptosis due to retrobulbar, periorbital, or orbital subperiosteal haemorrhage; conjunctival and subconjunctival haemorrhage (small ones are punctuate, triangular, or streaky and linear); linear and triangular varicosities of conjunctival venules; and keratoconjunctivitis as part of sicca-like or Sjogren-like syndrome.[61]
Musculoskeletal: swollen joints and joint effusions; children may assume a frog-leg position to accommodate for hip joint pain, which may be due to haemarthroses, periosteal haemorrhage, or metaphyseal structural changes; osteoporosis may occasionally be present.
Cardiac: the aetiology of temporary cardiac dysfunction in scurvy is poorly understood but in severe cases may be related to endocardial haemorrhage; pedal oedema may be a manifestation of cardiac failure or of local endothelial dysfunction.
Pulmonary: haemorrhagic pleural effusion (uncommon but potentially life-threatening); nail clubbing.
Neurological: manifestations of haemorrhagic stroke, entrapment neuropathies (particularly when peripheral oedema is evident).
Normal examination has also been reported, presumably when symptoms have developed in the setting of very low but not critical body stores.
Laboratory
Scurvy can be diagnosed on the basis of low blood ascorbate levels (whole blood, leukocyte, or serum) alone. Nonetheless, ascorbate levels are seldom ordered as part of an initial evaluation unless an astute examiner suspects the diagnosis. Comprehensive laboratory studies tailored to other diagnostic considerations are not discouraged; ascorbate levels may take several days to return and parallel diagnostic work-up during this time period may be critical. Other laboratory abnormalities related to scurvy may include only anaemia.
FBC is performed initially to look for other haematological disorders mimicking scurvy. For example, a normocytic anaemia may be expected with scurvy alone, but microcytic (i.e., with concomitant iron deficiency) or macrocytic (i.e., with concomitant B12 deficiency) anaemia may be found.
Serum ascorbic acid, leukocyte ascorbic acid, and whole blood ascorbic acid should be ordered on initial evaluation to help identify the amount and severity of vitamin C deficiency. European Society for Clinical Nutrition and Metabolism recommends measuring plasma vitamin C concentrations in all patients with clinical suspicion of scurvy or chronic low intake. However, they do not recommend measuring plasma vitamin C concentrations during critical illness or severe inflammation given challenges interpreting the results.[62]
Assessment for malignancy and infection should be strongly considered if a scurvy diagnosis is not firm on the basis of the dietary history or if other potential factors are identified to suggest an alternative diagnosis. A bone marrow biopsy is performed only when a scurvy diagnosis is in question, or if the patient's illness is rapidly progressing during initial treatment. Infection and haematological malignancy are the two most probable diseases in the differential diagnosis, and these should be pursued.
Imaging
On initial evaluation, an x-ray of the knee and wrist is recommended. In paediatric patients, an MRI may be useful, but findings may overlap with other diagnoses. Although several radiographic findings have been reported, they are often used as either supportive evidence when the diagnosis is established or as a diagnostic clue when scurvy is not considered in the initial differential diagnosis.
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