Scurvy

Definition/Description

Scurvy is a nutritional disease caused by a severe deficiency in vitamin C.[1]

Vitamin C, also known as ascorbic acid, is a water-soluble vitamin found in food that is necessary for healthy growth and development. A mutation in the gene coding for L-gulonolactone oxidase occurred, which no longer allows the human body to synthesize vitamin C. Thus, making it necessary for vitamin C to be taken in through the human diet to ensure the body is able to aid in wound healing, scar formation, repairing cartilage, bone, and teeth, and the ability to absorb iron.[2]  The vitamin acts as an antioxidant to help protect cells from damage induced by free radicals. Free radicals are formed when the body converts food into energy. The body is also exposed to free radicals in the environment such as smoke inhalation, air pollution, and ultraviolet light from sun exposure. For the body to make the collagen needed to increase wound healing and scar formation, vitamin C is needed in an everyday diet. Vitamin C improves the immune system by increasing the absorption of iron from plant-based foods which helps protect the body from disease.[3]  Signs of deficiency typically appear after one to three months and may overlap with infectious, hematologic, or rheumatologic diseases.


Scurvy was most prevalent during the Renaissance era in sailors on long voyages. A British naval surgeon, James Lind, found that citrus fruits--such as oranges, lemon, or lime juice--cured sailors with scurvy. A diagnosis of scurvy typically presents with weakness, anemia, gum disease, and skin hemorrhages.[4] Scurvy, if left untreated, can be fatal; however, it is very treatable once diagnosed. [5]

Prevalence

Today, scurvy is a much less common diagnosis and is typically seen in older adults who lack proper nutrition especially a diet with limited intake of foods enriched with vitamin C. According to an article, written by Anita Carr and Balz Frei, found in The American Journal of Clinical Nutrition, stated that approximately 25% of adults in the United States consume less than 60 mg/day of vitamin C and approximately 10% intake less than 30 mg/day.[6]  Consuming less than 10 mg/day can lead to the diagnosis of scurvy.[7] The following populations are at increased risk for a vitamin C inadequacy that could develop into a diagnosis of scurvy without appropriate dietary management.

  • Smokers[7] 
  • Infants fed evaporated or boiled milk[7] 
  • Children with restricted diets[8] 
  • Children with autism, developmental delay, and cerebral palsy[8] 
  • Sailors at sea for months[8] [4]  
  • Third world countries with limited foods [7]
  • Individuals with malabsorption [7]
  • Individuals with cancer [7]
  • Individuals with chronic diseases such as end-stage renal disease [7]

Characteristics/Clinical Presentation

Vitamin C deficiency can lead to the following signs and symptoms:[7]

  • Anemia
  • Bleeding gums
  • Decreased ability to fight infection
  • Decreased wound-healing rate
  • Dry and splitting hair
  • Easy bruising
  • Gingivitis (inflammation of the gums)
  • Nosebleeds
  • Possible weight gain because of slowed metabolism
  • Rough, dry, scaly skin
  • Swollen and painful joints
  • Weakened tooth enamel

Associated Co-morbidities

[5]

Osteopenia - osteopenia is defined by bone densitometry as a T score -1 to -2.5. There are many causes for osteopenia including calcium and vitamin D deficiency and inactivity. Genetics plays an important role in a person's bone mineral density and often Caucasian women with a thin body habitus who are premenopausal are found to have osteopenia.[9] 

Iron deficiency - As the name implies, iron deficiency anemia is due to insufficient iron. Without enough iron, your body can't produce enough of a substance in red blood cells that enables them to carry oxygen (hemoglobin). As a result, iron deficiency anemia may leave you tired and short of breath.[10]

Anemia

Folate deficiency - Folic acid (vitamin B9) works with vitamin B12 and vitamin C to help the body break down, use, and make new proteins. The vitamin helps form red and white blood cells. It also helps produce DNA. Folate is not stored in the body in large amounts, your blood levels will get low after only a few weeks of eating a diet low in folate.[11] 

Vitamin K deficiency - Vitamin K (VK) deficiency can occur in any age group but is encountered most often in infancy. VK, an essential, lipid-soluble vitamin that plays a vital role in the production of coagulation proteins[12]


Medications

At this time, the only effective treatment for scurvy is vitamin C replacement therapy. The same is also said for prevention. The best method to prevent scurvy is to ensure an adequate diet by consuming the recommended daily amounts of vitamin C. The Recommended Dietary Allowance can be found in the medical management section below, along with supplementation and food sources that have high amounts of vitamin C. [13][14]

Diagnostic Tests/Lab Tests/Lab Values

Plasma levels: A fasting serum ascorbic acid level greater than 0.6 mg/dL rules out scurvy. Scurvy generally occurs at levels below 0.1 mg/dL.[15]
Leukocyte levels: The level of vitamin C in leukocytes more accurately correlates to tissue stores compared with serum levels, because these cells are not affected acutely by circadian rhythm or dietary changes. A level of zero indicates latent scurvy. Levels of 0-7 mg/dL reflect a state of deficiency.[15]
Urinary levels: A more commonly used method is the ascorbic acid tolerance test, which quantitates urinary ascorbic acid over the 6 hours following an oral load of 1 g of ascorbic acid in water.[15]
Radiographic findings: in infantile scurvy are diagnostic and may show any of the following:[15]

  • Subperiosteal elevation
  • Fractures and dislocation
  • Alveolar bone reabsorption
  • Ground-glass appearance of cortex -- The cortex becomes thin and the trabecular structure of the medulla atrophies and develops a ground-glass appearance. The zone of provisional calcification becomes dense and widened, and this zone is referred to as the white line of Fränkel. The epiphysis also shows cortical thinning and the ground-glass appearance.
Image reprinted with permission from Bruce M. Rothschild, MD, Northeast Ohio Medical University, published by Medscape Drugs & Diseases (http://emedicine.medscape.com/), 2016, available at: http://emedicine.medscape.com/article/125350-overview.

Etiology/Causes

The primary cause of scurvy is inadequate intake of vitamin C. When intake level falls below approximately 10 mg/day, the risk for diagnosis of scurvy increases and typically symptoms will begin within one to three months once the total body vitamin C pool falls below 350 mg.[7][16]This level is reached when the diet is completely lacking vitamin C for 60-90 days.[16] Individuals with poor diet with limited intake of vitamin C are more susceptible to developing scurvy.[17] 

Systemic Involvement

Circulatory System:[18]

  • Hypotension may be seen during the late stages of scurvy in response to vessels inability to constrict in response to stimuli. Complications in the heart can arise which include cardiac enlargement, electrocardiographic (ECG) changes, hemopericardium, and sudden death. Heart failure due to high-output can be seen in response to anemia.
  • Anemia is present in 75% of the known cases of scurvy. Anemia appears in this population because of the blood loss into tissues, coexisting dietary deficiencies, malabsorption, and intravascular hemolysis.

Gastrointestinal System:[18]'

If teeth have been erupted, gum hemorrhage is possible. The tissue surrounding the upper incisors are most commonly affected and present with a blue or purple color and a spongy feeling.

  • Loose teeth can also be seen with a diagnosis of scurvy. Gum swelling, friability, bleeding, and infection can occur when loose teeth are present.
  • Anorexia is sometimes seen with scurvy diagnoses; therefore, weight loss is commonly seen. A submucosal hemorrhage may be shown with an upper endoscopy.

Integumentary System/Skeletal:[19] 

  • Several skin problems can be noted with a diagnosis of scurvy. An early symptom is appearance bluish spots similar to bruises surrounding hair follicles on the shins, known as perifollicular hyperkeratotic papules. The hair is usually twisted like a corkscrew and very brittle susceptible to breakage. As the disease progresses, large patches of papules can form larger areas of bruising known as ecchymoses or purpura shown in the picture below.
Purpura [Internet]. Wikipedia. 2016 [cited 5 April 2016]. Available from: https://en.m.wikipedia.org/wiki/Purpura














  • Vitamin C deficiency affects the production of collagen which can decrease efficiency of wound healing. This can affect new wounds and also decrease the integrity of old scars, leaving them at risk for skin breakdown.
  • Sometimes bleeding within the joints can occur leading to painful hemarthroses. A subperiosteal hemorrhage can be palpable along the femur and sometimes the tibia in infants. If bleeding occurs in the femoral sheath, neuropathies can be seen and if there is bleeding into the muscles, woody edema can be present.

Nervous System:[18]'

  • Protrusion of the eyeball occurs due to an orbital hemorrhage and can be seen in the diagnosis of scurvy. Other ocular impairments can be seen which include dry eye similar to the diagnosis of Sjögren syndrome, subconjunctival hemorrhage, and bleeding in the eyelid, retrobulbar space, and periorbital are. Late in the diagnosis scleral icterus can be caused by hemolysis along with pale conjunctiva.

Medical Management (current best evidence)

Treatment
According to current evidence, the only known effective treatment for scurvy is vitamin C replacement therapy which requires over infusing the body with ascorbic acid. In most adults, saturating the body with vitamin C can be achieved with 250 mg of vitamin C four times a day for at least a week. Typically, bleeding stops within 24 hours, the body becomes saturated within a few days and petechiae resolves within two weeks. Symptoms will resolve once the body stores of vitamin C are restored.[13][14]

Prevention
The Recommended Dietary Allowance (RDA) suggests the amount of each vitamin an individual should intake daily to prevent a vitamin C deficiency[6]  The amounts listed should be used as a general recommendation but it should be understood that the correct amount is different for each individual and could vary based age and gender. Pregnancy and illnesses can also affect the amount of vitamin C needed in one’s diet. The table below lists the recommended daily intake of vitamin C based on age, gender, pregnancy.[7] 

Table 1. Recommended Dietary Allowances (RDA’s) for Vitamin C

AGE MALE FEMALE PREGNANCY
0-6 Months 40 mg/day   40 mg/day
7-12 Months 50 mg/day 50 mg/day
1-3 Years 15 mg/day 15 mg/day
4-8 Years 25 mg/day 25 mg/day
9-13 Years 45 mg/day 45 mg/day
14-18 Years 75 mg/day
65 mg/day

80 mg/day

(breastfeeding:115 mg/day)

19 and up 90 mg/day 75 mg/day

85 mg/day

(breastfeeding:120 mg/day)

  • Smokers/Secondhand smoke exposure need an additional 35 mg/day
  • Ask your healthcare provider what amount is best 


Dietary management based off the above daily intake recommendations is the best way to prevent a vitamin C deficiency which will decrease the risk for developing scurvy. 


Supplementation Recommendations
Vitamin C supplementation is normally in the form of ascorbic acid and equivalent to the amount found in orange juice and broccoli. Other types of supplements include:[7]

  • Sodium ascorbate
  • Calcium ascorbate
  • Other mineral ascorbates
  • Ascorbic acid with bioflavonoids
  • 'Ester-C® (contains calcium ascorbate, dehydroascorbate, calcium threonate, xylonate and lyxonate) '


Dietary Management/Food Sources Vitamin C can be found in some foods naturally and other foods are fortified with vitamins. Table 2 lists the fruits and vegetables that contain naturally high in vitamin C content. Many cereals and beverages are fortified with vitamin C. Checking food labels can provide information on the amount of vitamin C contained in the product.

Table 2. Fruits and vegetables that contain high amounts of vitamin C. 

Fruits Vegetables
Cantaloupe Broccoli
Orange Brussel Sprouts
Grapefruit Cauliflower
Kiwi fruit
Spinach
Mango Sweet and white potatoes
Papaya
Tomatoes and tomato juice
Pineapple Winter squash
Strawberries
Green/red peppers
Raspberries
Turnip greens
Blueberries Cabbage
Cranberries
Watermelon

When choosing foods high in vitamin C, it is also important to consider how the food is prepared. Storing the food for long periods of time or cooking the product a certain way can reduce the amount of vitamin C the food contains. The best source of vitamin C is found most when consuming raw fruits and vegetables with high daily values. Cooking losses may be reduced by microwaving or steaming the food. Light exposure can also reduce the amount of vitamin C found in foods. According the NIH, juices kept in cartons should be chosen rather than juice contained in a clear bottle.[7]


Physical Therapy Management (current best evidence)

There are no direct physical therapy interventions for Scurvy. The patient may be referred to physical therapy for the treatment of impairments caused by Scurvy. These may include joint pain and stiffness, musculoskeletal pain, and musculoskeletal weakness, (particularly in the lower extremity).[7]

Differential Diagnosis

[20]

  • Meningococcemia
  • Osteomyelitis
  • Pediatric Poliomyelitis
  • Pediatric Syphilis
  • Retinoblastoma Imaging
  • Rheumatic Fever
  • Rheumatoid Arthritis
  • Rocky Mountain Spotted Fever
  • Septic Arthritis

Case Reports/ Case Studies

Cole JA, Warthan MM, Hirano SA, Gowen CW, Williams JV. Scurvy in a 10-Year-Old Boy. Pediatric Dermatology. 2011;28(4):444-6.

Authors: Cole JA, Warthan MM, Hirano SA, Gowen CW, Williams JV.


Abstract:

  • Scurvy, or hypovitaminosis C, is an uncommon condition that exists today primarily within certain unique populations - particularly elderly subjects, patients with neurodevelopmental disabilities or psychiatric illnesses, or others with unusual dietary habits.
  • This case highlights that the presence of scurvy should not be forgotten, because of its presence among susceptible populations. Scurvy can present with clinical manifestations that include dystrophic or corkscrew hairs, gingival hyperplasia, and weakened blood vessel walls, causing bleeding in the skin, joints, and other organs.

Patient Characteristics:

  • 10 year old boy with autism and slight developmental delay
  • Current bruising and leg swelling


Subjective: Chief complaints include:

  • Persistent lower extremity bruising and swelling
  • Refusal to walk
  • Pain in lower extremities


Examination:

  • Persistent bruising and swelling throughout lower extremity
  • Persistent petechial eruption on patient’s legs
  • Intermittent oozing of gums
  • Decreased hemoglobin and hematocrit and low serum iron (8.8 gdL and 26%)
  • Chest radiograph demonstrated mild diffuse osteoporosis
  • Hypertrophic and bleeding gingiva
  • MRI of knees revealed significant deep soft tissue inflammatory change.
  • Serum L-ascorbic acid level was deficient (<0.12 mg/dL)


Past Medical History:

  • Child was struck by a surfboard approximately 4 weeks earlier, causing bruising and swelling around his left knee.
  • Limited diet, eating only hamburgers, Wheat Chex, Pop Tarts, oyster crackers, and pancakes. Patient took no vitamins or supplements and drank only water.


Intervention: Pharmaceutical

  • Oral administration of 100 mg of L-ascorbic acid twice daily
  • Co-intervention:Daily multivitamin per nutrition consultation
  • Outcomes: Within 24 hours of starting the L-ascorbic acid patient showed clinical improvement.
  • Outpatient follow-up showed normalization of L-ascorbic acid levels and no remaining clinical features of scurvy.

(case studies should be added on new pages using the case study template)

Resources

[National Health Institute]
[Medscape Scurvy]

Recent Related Research (from Pubmed)

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References

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  1. Wax E, Zieve D, Ogilvie I. Vitamin C [updated Feb. 2, 2015; cited 2016 April 8]. Available from: https://www.nlm.nih.gov/medlineplus/ency/article/002404.htm.
  2. Carr AC, Frei B. Toward a new recommended dietary allowance for vitamin C based on antioxidant and health effects in humans. The American Journal of Clinical Nutrition. 1999;69(6):1086-107.
  3. Wax E, Zieve D, Ogilvie I. Vitamin C [updated Feb. 2, 2015; cited 2016 April 8]. Available from: https://www.nlm.nih.gov/medlineplus/ency/article/002404.htm.
  4. 4.0 4.1 Chaudhry SI, Newell EL, Lewis RR, Black MM. Scurvy: a forgotten disease. Clinical & Experimental Dermatology. 2005;30(6):735-6
  5. 5.0 5.1 Goebel L, July M. Scurvy Overview: Prognosis 2015 [updated Sep 23, 2015; cited 2016 April 5]. Available from: http://emedicine.medscape.com/article/125350-overview#a7.
  6. 6.0 6.1 Carr AC, Frei B. Toward a new recommended dietary allowance for vitamin C based on antioxidant and health effects in humans. The American Journal of Clinical Nutrition. 1999;69(6):1086-107.
  7. 7.00 7.01 7.02 7.03 7.04 7.05 7.06 7.07 7.08 7.09 7.10 7.11 7.12 Wax E, Zieve D, Ogilvie I. Vitamin C [updated Feb 2, 2015; cited 2016 April 8]. Available from: https://www.nlm.nih/gov/medlineplus/ency/article/002404.htm.
  8. 8.0 8.1 8.2 Alqanatish JT, Alqahtani F, Alsewairi WM, Al-kenaizan S. Childhood scurvy: an unusual cause of refusal to walk in a child. Pediatric rheumatology online journal. 2015;13-23.
  9. Karaguzel G, Holick MF. Diagnosis and treatment of osteopenia. Reviews in endocrine & metabolic disorders. 2010;11(4):237-51.
  10. Mayo Clinic Staff. Iron Deficiency Anemia 2014 [updated Jan. 2, 2014; cited 2016 April 8]. Available from: http://www.mayoclinic.org/diseases-conditions/iron-deficiency-anemia/basics/definition/con-20019327.
  11. Wax E, Zieve D, Ogilvie I. Folate Deficiency [updated July 14, 2015; cited 2016 APril 8]. Available from: https://www.nlm.nih.gov/medlineplus/ency/article/000354.htm.
  12. Nguyen-Khoa D-T, Patel P. Vitamin K Deficiency [updated Dec. 18, 2015; cited 2016 April 8]. Available from: http://emedicine.medscape.com/article/126354-overview.
  13. 13.0 13.1 Goebel L, July M. Scurvy Treatment and Management 2015 [updated Sep 23, 2015; cited 2016 April 5]. Available from: http://emedicine.medscape.com/article/125350-treatment.
  14. 14.0 14.1 Goebel L, July M. Scurvy Medication 2015 [updated Sep 23, 2015; cited 2016 APril 5]. Available from: http://emedicine.medscape.com/article/125350-medication.
  15. 15.0 15.1 15.2 15.3 Goebel L. July M. Scurvy Workup 2015 [updated Sep 23, 2015; cited 2016 April 5]. Available from: http://emedicine.medscape.com/article/125350-workup#showall.
  16. 16.0 16.1 Lau H, Massasso D, Joshua F. Skin, muscle and joint disease from the 17th century: scurvy. International Journal of Rheumatic Diseases. 2009;12(4):361-5.
  17. Goebel L, July M. Scurvy Background 2015 [updated Sep 23, 2015; cited 2016 April 5]. Available from: http://emedicine.medscape.com/article/125350-overview.
  18. 18.0 18.1 18.2 Goebel L, July M. Scurvy Clinical Presentation 2015 [updated Sep 23, 2015; cited 2016 April 5]. Available from: http://emedicine.medscape.com/article/125350-clinical.
  19. Ngan V. Scury 2005 [updated Nov. 9, 2015; cited 2016 April 8]. Available from: http://www.dermnetnz.org/systemic/scurvy.html.
  20. Goebel L, July M. Scurvy Differential Diagnosis 2015 [updated Sep 23, 2015; cited 2016 April 5]. Available from: http://emedicine.medscape.com/article/125350-differential.