Scurvy

What is scurvy?

Scurvy (scorbutus) is the clinical disease caused by Vitamin C (ascorbic acid) deficiency with characteristic mucocutaneous and musculoskeletal manifestations.

Ecchymoses

Perifollicular haemorrhage and ecchymoses

Corkscrew hairs and purpura

Who gets scurvy?

Scurvy can affect all age groups, both sexes, and all races. It remains surprisingly common worldwide in both developed and developing countries. The Vitamin C requirement is highest in children and during pregnancy, and lowest in the elderly.

In developed countries vitamin C deficiency affects up to 10% of women and 14% of men. Groups affected include:

• Children with very restricted diets such as may be seen with autism spectrum disorder, eating disorders, or food-fads; sickle cell disease or thalassaemia requiring blood transfusions resulting in iron overload; haemodialysis; bowel disease such as coeliac disease or Crohn disease
• Adults associated with:
  • Pregnancy (due to an increased requirement)
  • Psychiatric and behavioural disorders including depression, anorexia nervosa, alcoholism
  • Malabsorption due to bowel disease, iron overload, or alcohol excess
  • Cancer patients due to anorexia related to the malignancy, treatment, or depression.
• Elderly and others living alone, on a low income, homeless, or with poor dentition who are not eating a well-balanced diet

In developing countries such as northern India, the incidence can be as high as 73.9% due to limited access to Vitamin C-rich fruit and vegetables.

What causes scurvy?

Scurvy develops when sustained dietary deficiency of Vitamin C for several months reduces total body stores from 1500g to less than 300g. Unlike nearly all other animals, humans are unable to synthesise Vitamin C which must be obtained from the diet. 

Vitamin C is required for:

• Maintaining the triple helix structure of collagen, an essential component of connective tissue including support of blood vessels
• Anti-oxidant effect reducing free radicals, with effects on lipid metabolism, atherosclerosis and cardiovascular disease
• Iron absorption from the proximal small intestine.

What are the clinical features of scurvy? 

The initial clinical features of scurvy are non-specific and include:

• General malaise and lethargy
• Poor appetite and failure to thrive
• Ill temper
• Fever and tachypnoea.

As the condition progresses, shortness of breath, wasting, anaemia, oedema, mucocutaneous changes, ocular and musculoskeletal symptoms develop.

Mucocutaneous features of scurvy

• Follicular hyperkeratotic papules appear first on the upper arms, often spreading to the legs and buttocks
• Perifollicular haemorrhages, purpura (which can be palpable), and ecchymoses on the legs
• Hairs are often twisted and fragile (corkscrew hairs, swan-neck hairs)
• Poor wound healing and re-opening of old healed scars
• Splinter haemorrhages in the nails
• Red, swollen gums in patients with teeth (particularly around the upper incisors) which may later become purple or black
• Bleeding from the gums
• Loosening and loss of teeth

Purpura

Ecchymoses

Corkscrew hairs

Images in this gallery of ecchymoses and corkscrew hairs from: Esteves A, Teixeira da Silva F, Carvalho J, Felgueiras P, Laranjeira P. Scurvy, starvation, and flea infestation - a case report from 21st century Europe. Cureus. 2021;13(2):e13158.

Ocular effects of scurvy

• Eye dryness and irritation
• Subconjunctival, periorbital, or orbital haemorrhage

Musculoskeletal effects of scurvy

• Painful haemarthosis
• Subperiosteal haemorrhage, particularly femur and proximal tibia
• Costochondral junction beading – ‘scorbutic rosary’
• Intramuscular bleeding leading to woody oedema
• Severe pain of arms and legs (the major feature of scurvy in infants) and reluctance to walk

Other nutritional deficiencies are commonly associated with scurvy and clinical features may therefore be mixed and therefore confusing.

How do clinical features vary in differing types of skin?

Perifollicular haemorrhages and ecchymoses may appear dusky blue or a violaceous colour in patients with dark skin types.

What are the complications of scurvy?

• Haemorrhage and its consequences
• Cardiovascular disease
• Jaundice

How is scurvy diagnosed?

Scurvy should be considered on the clinical presentation and confirmed with recovery after Vitamin C supplementation.

Serum levels of Vitamin C reflect recent intake rather than body stores, so are unreliable. Investigation of other nutritional deficiencies should be undertaken.

Anaemia is common, and may be due to decreased iron absorption, bleeding, haemolysis, and other dietary deficiencies.

In children, characteristic changes are seen on X-ray:

• Frankel sign, a dense line in the metaphysis due to increased calcification of the cartilage matrix
• Trummerfeld zone due to subperiosteal haemorrhage
• Wimberger ring sign in the epiphysis
• Pelkan sign due to microfractures.

Frankel sign (green arrow), Trummerfeld zone (red arrow)

Image from: Gallizzi R, Valenzise M, Passanisi S, Pajno GB, De Luca F, Zirilli G. Scurvy may occur even in children with no underlying risk factors: a case report. J Med Case Rep. 2020;14(1):18.

What is the differential diagnosis for scurvy?

• Petechiae, purpura, and ecchymoses due to capillaritis, cutaneous vasculitis, thrombocytopaenia, or any other cause
• Folliculitis
• Gingivitis and periodontitis

What is the treatment for scurvy?

Prevention

The recommended daily intake of Vitamin C in Australia and New Zealand is 45 mg/d. Pregnant and lactating women need 55-85 mg/d. Smokers also require a higher Vitamin C intake due to increased metabolic turnover. A well-balanced diet including fresh fruit and vegetables provides sufficient Vitamin C for most people.

Vitamin C supplementation

Adults: Oral Vitamin C 1000 mg/d in divided doses for at least one week, then 300-500 mg/d for a further week, followed by the recommended daily allowance.

Children: The American Academy of Paediatrics recommends children receive 100 mg 3 times daily for at least 1 week, followed by 100 mg daily until symptoms have resolved.

Vitamin C can also be given intravenously, intramuscularly, or per rectum.

Treatment of complications

Transfusion is sometimes required for severe anaemia, especially if acute related to haemorrhage.

Children with bone disease may require surgery if symptoms do not resolve with supplements.

What is the outcome for scurvy?

Most symptoms of scurvy resolve rapidly within 24–72 hours of starting Vitamin C supplements. Bone changes may take several weeks to resolve. Full recovery is usual within 1–3 months. Loss of teeth and sequelae from haemorrhage are the only permanent disabilities.

If untreated, scurvy can cause sudden death.

Understanding the reason for developing scurvy, whether this be social or medical, and addressing this will reduce the risk of recurrence.