eMedicine Specialties > Radiology > Pediatrics

Scurvy

Bruce M Rothschild, MD, Professor of Medicine, The Northeastern Ohio Universities College of Medicine; Director, Arthritis Center of Northeast Ohio; Adjunct Professor, Department of Biomedical Engineering, University of Akron
Jeno Imre Sebes, MD, Professor, Department of Radiology, University of Tennessee Health Science Center at Memphis

Updated: Dec 17, 2008

Introduction

Background

The term scurvy is derived from the Nordic word skyrbjugr, meaning swelling or edema. It has also been suggested that the term is derived from the Old Icelandic words skyrbugr, scarby, or skurvic.

Scurvy is caused by a lack of vitamin C and manifests as collagen defects, hemorrhagic diathesis, abnormalities in bone maturation, epiphyseal disease, lifting of the periosteum, and hemarthroses.^1,2,3

Related eMedicine topics:

Scurvy (Endocrinology)

Scurvy (Dermatology)
 

Frequency

United States

Scurvy is not common in the US, though in one study, vitamin C deficiency was present in up to 23% of respondents.^4,5 Young children and older persons are predisposed to scurvy because of their diet or the overpreparation of food (cooking destroys vitamin C). Smokers, non-Hispanic black males, and individuals who do not use vitamin supplements have an increased risk of vitamin C deficiency.⁴

International

Internationally, scurvy is rare. Young children and older persons are predisposed to scurvy because of their diet or the overpreparation of food (cooking destroys vitamin C).

Mortality/Morbidity

• Scurvy can lead to many other conditions, such as mental status alteration, gum disease and tooth loss, ecchymoses, hemarthrosis, the failure of wounds to heal, the breakdown of healed ulcers, heart and skeletal muscle damage, abruptio placentae, gastrointestinal blood loss, and arrested skeletal development.
• Epiphyseal separation at the epiphyseal plate is a complication.
• Sudden death has been reported.

Sex

Males and females are equally affected.

Age

Those most commonly affected are children aged 6-18 months; however, adults can also be affected. Unless the mother had a deficiency herself, the maternal contribution of vitamin C is generally protective of infants younger than 6 months.

Presentation

Presentation and natural history

Perivascular hemorrhage and lifting of the periosteum caused by hemorrhage are the main anatomic alterations depicted on radiographs. This hemorrhage is due to increased capillary fragility. In scurvy, there is normal mineralization of osteoids; however, the overall amount is decreased. This process is distinct from that in rickets, in which the lack of vitamin D results in defective mineralization of a normal organic matrix.

Scurvy is the direct result of vitamin C deficiency. Vitamin C is required for prolyl and lysyl hydroxylase activity and is essential for collagen synthesis. Defective collagen compromises skin, joint, bone, and vascular integrity. Vitamin C is also required for carnitine synthesis, and it is critical for fatty acid transportation into the mitochondria. This oxidative metabolism is also critical for muscle function.

Scurvy is usually caused by a lack of dietary vitamin C related to inadequate food intake, the destruction of vitamin C in food caused by cooking and canning, or the absence of fresh fruit in the diet. Malabsorption, tobacco use, chronic oxidative stress (eg, HIV infection, inflammatory bowel disease, endotoxemia, diabetes, heat stress), hemodialysis, and therapy with the folic acid antagonist aminopterin all reduce the level of vitamin C in the body. In studies of vitamin C deficiency, the most common associated causes included poor dentition, gastrointestinal disease, food fads, and alcoholism.^6,7

Clinical symptoms include the following:

• Lethargy, listlessness, mental confusion, and fatigue
• Pale, bloated complexion and dry, rough skin as a result of defective collagen
• Hair follicle enlargement and plugging; perifollicular congestion; proliferation of blood vessels; formation of lumps in hair follicles; fractured, coiled, or bent hairs; and perifollicular hemorrhage caused by defective collagen
• Swollen and purple gums, putrid and bleeding gums, or loosened teeth as a result of defective collagen
• Ecchymoses due to blood-vessel fragility (Purpura may become palpable.)
• Costochondral junction swelling; hemarthrosis; and pain, stiffness, or swelling of the knees or ankles as a result of bleeding
• Arrested skeletal development caused by defective collagen
• Impaired wound healing and breakdown of previously healed ulcers caused by defective collagen
• Dyspnea, chest pain, abruptio placentae, intraocular hemorrhage, diarrhea, and gastrointestinal blood loss caused by fragile blood vessels and defective collagen
• Femoral nerve compression by hematomas
• Normocytic anemia and macrocytic anemia related to osmotic fragility of the red blood cells
• Hypotension due to blood loss and vascular fragility
• Hematuria
• Subdural hemorrhage
• Sudden death

Individuals with renal complications who are receiving dialysis may be susceptible to vitamin C deficiency. Dietary restrictions associated with dialysis generally limit the amount of potassium that a patient may ingest in order to avoid hyperkalemia. Unfortunately, many foods rich in potassium, such as orange juice, strawberries, and broccoli, are also the best sources of vitamin C. In addition, the dialysis process removes a significant amount of vitamin C from the body. This is further compounded by the fact that the use of vitamin C supplements may lead to oxalosis, which can cause renal and liver problems. Often, physicians have avoided vitamin supplementation in order to safeguard against oxalosis. New research suggests that the benefits of vitamin C supplementation and advances in dialysis techniques may lead to an increased use of vitamin C to control anemia for dialysis patients.⁸

Treatment

• No radiologic interventions are indicated, although the dietary provision of recommended daily allowances of vitamin C is generally recommended.

Differential Diagnoses

Other Problems to Be Considered

Copper deficiency
Vasculitis
Clotting factor deficiency
Leukemia
Thrombocytopenic purpura
Henoch-Schönlein purpura
Meningococcemia

Radiography

Findings

Techniques and findings

Radiography is the preferred imaging examination for diagnosis. Serum vitamin C levels can be obtained to confirm the diagnosis of scurvy.

Some authors have suggested that the most diagnostic radiologic finding of vitamin C deficiency is a large, fluctuant, parietal swelling, which is apparently caused by subperiosteal hemorrhage. This author, however, considers long bone changes to be better clinical identifiers of vitamin C deficiency than parietal swelling. The epiphyses and periosteum also become easily detachable because of hemorrhage below the periosteum. Separation of the metaphyseal plate from the diaphysis, epiphyseal clefts, and malalignment of the metaphysis may also occur. A circular, opaque radiologic shadow often surrounds epiphyseal centers of ossification. This ring of increased opacity formed around the ossification center of long bone epiphyses is known as the Wimberger sign, which may result from bleeding or attachment movement.

Vitamin C deficiency is characterized by cortical thinning, which is sometimes described as a “pencil-point” cortex. Decreased trabeculae produce a decrease in radiopacity, resulting in a transparent aspect similar in appearance to ground glass (see Image below and Image 1 in Multimedia).

Anteroposterior radiograph of the lower extremities shows ground-glass osteopenia. Transverse metaphyseal lines of increased and decreased opacity (Trümmerfeld zone) are associated with lateral growth of the metaphyseal calcification zone and periosteal elevation, which produces the characteristic metaphyseal beaks known as Pelkan spurs.

Costochondral junctions of the first 6 or 8 thoracic ribs may be expanded; this change may be related to fracturing of the zone of provisional calcification during normal respiration. The costochondral junctions are rounded and appear smooth, knobby, and steplike. The enlargement of the costochondral junctions simulates that seen in rickets.

The zone of proliferating cartilage cells is distorted, producing spicules from the metaphysis into the epiphyseal plate region. The zone of temporary calcification broadens, producing a wide, radiopaque metaphyseal band. Subjacent to this is a zone of poor-quality trabeculae, which appears radiolucent. A steplike lateral projection is found at the epiphyseal line in patients who are severely affected. Scorbutic changes are radiologically more severe in the lower extremities, whereas scorbutic changes seen in rickets are allegedly more severe in the upper extremities.

Metaphyseal “beaks” and transverse lines of increased or decreased opacity may be seen in scurvy. The “beaks,” known as Pelkan spurs, are associated with fractures of the Trümmerfeld zone. They may be produced by lateral growth of the metaphyseal calcification zone and are associated with periosteal elevation. Subepithelial marginal clefts may also be present.

Skull changes may produce a porotic hyperostosis (“hair-on-end” appearance) or crew-cut appearance secondary to marrow hyperplasia in response to anemia. No sphenoid changes are reported. Sphenoid porosity has not been shown to be caused by scurvy.

Degree of Confidence

Periosteal elevation and epiphyseal separation both appear to be relatively specific for scurvy. Osteoporosis is a nonspecific finding.

The periosteal reaction of syphilis is more generalized than that of scurvy and is usually thick or multilaminated. Syphilis, often called the great imitator, produces metaphyseal beaking similar to that noted in scurvy; however, syphilis does not produce radiopaque metaphyseal lines. Although periosteal elevation may occur and produce a linear elevated area in patients, a spiculated periosteal reaction does not occur in scurvy.

Metaphyseal lesions caused by syphilis are beaklike, whereas those caused by scurvy involve epiphyseal separation. Epiphyseal separation is a known complication of scurvy; however, it is also seen in cases of child abuse. The periosteal reaction resulting from child abuse is more generally distributed and is associated with a fracture.

Costochondral beading is more common with rickets than with scurvy.

Skull-marrow hyperplasia is more likely to result from hemolytic anemia or anemia related to parasitic infestation.

Magnetic Resonance Imaging

Findings

Techniques and findings

• Focal areas of metaphyseal marrow edema on T1-weighted and PD/SPIR images, attributed to focal hemorrhage or infarcts, are seen. 
• Subperiosteal fluid and displacement of epiphyses has been reported.⁹
• Skull changes may be below the resolution of computed tomography (CT) or magnetic resonance imaging (MRI).
• Change in skull shape is not diagnostic, as this finding may also be seen with hemolytic anemia, other causes of bone marrow hyperplasia, and rickets.

Nuclear Imaging

Findings

Technetium bone scans are not routinely performed in patients with scurvy; however, increased uptake at the distal metaphyses and the epiphyses and formation of a rachitic rosary are hypothetically possible.

Intervention

Medicolegal Pitfalls

• The failure to diagnose copper deficiency, leukemia, and concomitant rickets or folate deficiency is a pitfall.
• Congenital syphilis and neuroblastoma produce findings similar to those of scurvy. The same findings may also be seen in a limb with residual palsy as a result of polio.

Multimedia

Media file 1: Anteroposterior radiograph of the lower extremities shows ground-glass osteopenia. Transverse metaphyseal lines of increased and decreased opacity (Trümmerfeld zone) are associated with lateral growth of the metaphyseal calcification zone and periosteal elevation, which produces the characteristic metaphyseal beaks known as Pelkan spurs.

References

1. Bohrer I, Roy M, Nager W, te Wildt B, Emrich HM, Ohlmeier MD. [Scurvy--a wrongly forgotten avitaminosis]. MMW Fortschr Med. Nov 8 2007;149(45):41-3. [Medline].

2. Léger D. Scurvy: reemergence of nutritional deficiencies. Can Fam Physician. Oct 2008;54(10):1403-6. [Medline].

3. Sommer A. Vitamin a deficiency and clinical disease: an historical overview. J Nutr. Oct 2008;138(10):1835-9. [Medline].

4. Hampl JS, Taylor CA, Johnston CS. Vitamin C deficiency and depletion in the United States: the Third National Health and Nutrition Examination Survey, 1988 to 1994. Am J Public Health. May 2004;94(5):870-5. [Medline].

5. Velandia B, Centor RM, McConnell V, Shah M. Scurvy is still present in developed countries. J Gen Intern Med. Aug 2008;23(8):1281-4. [Medline].

6. Burk CJ, Molodow R. Infantile scurvy: an old diagnosis revisited with a modern dietary twist. Am J Clin Dermatol. 2007;8(2):103-6. [Medline].

7. Olmedo JM, Yiannias JA, Windgassen EB, Gornet MK. Scurvy: a disease almost forgotten. Int J Dermatol. Aug 2006;45(8):909-13. [Medline].

8. Handelman GJ. Vitamin C neglect in hemodialysis: Sailing between Scylla and Charybdis. Blood Purification. 2007;25(1):58-61. [Medline].

9. Karthiga S, Dubey S, Garber S, Watts R. Scurvy: MRI appearances. Rheumatology (Oxford). Jul 2008;47(7):1109. [Medline].

10. Francescone MA, Levitt J. Scurvy masquerading as leukocytoclastic vasculitis: a case report and review of the literature. Cutis. Oct 2005;76(4):261-6. [Medline].

11. Rothschild BM, Martin LD. Paleopathology: Disease in the Fossil Record. London: CRC Press; 1993.

12. Willmott NS, Bryan RA. Case report: scurvy in an epileptic child on a ketogenic diet with oral complications. Eur Arch Paediatr Dent. Sep 2008;9(3):148-52. [Medline].

Keywords

scurvy, vitamin C deficiency, ascorbutism, scorbutism, Barlow disease, Barlow's disease, subperiosteal hematoma syndrome, hypovitaminosis C, collagen defects, hemorrhagic diathesis, abnormalities in bone maturation, epiphyseal disease, lifting of the periosteum, hemarthroses

Contributor Information and Disclosures

Author

Bruce M Rothschild, MD, Professor of Medicine, The Northeastern Ohio Universities College of Medicine; Director, Arthritis Center of Northeast Ohio; Adjunct Professor, Department of Biomedical Engineering, University of Akron
Bruce M Rothschild, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Rheumatology, American Federation for Clinical Research, American Heart Association, American Society for Clinical Pharmacology and Therapeutics, International Skeletal Society, New York Academy of Sciences, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Jeno Imre Sebes, MD, Professor, Department of Radiology, University of Tennessee Health Science Center at Memphis
Jeno Imre Sebes, MD is a member of the following medical societies: American College of Chest Physicians, American College of Radiology, American Medical Association, American Roentgen Ray Society, Association of University Radiologists, International Skeletal Society, New York Academy of Sciences, Radiological Society of North America, Sigma Xi, Society of Skeletal Radiology, Tennessee Medical Association, and Tennessee Radiological Society
Disclosure: Nothing to disclose.

Medical Editor

Michael A Bruno, MD, Associate Professor, Departments of Radiology and Medicine, Pennsylvania State University College of Medicine; Director, Radiology Quality Management Services, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine
Michael A Bruno, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, Association of University Radiologists, Radiological Society of North America, Society of Nuclear Medicine, and Society of Skeletal Radiology
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Marta Hernanz-Schulman, MD, FAAP, Professor, Radiology, Radiological Sciences, and Pediatrics, Director, Department of Pediatric Radiology, Radiologist-in-Chief, Director, Department of Diagnostic Imaging, Vanderbilt University Medical Center, Vanderbilt Children's Hospital
Marta Hernanz-Schulman, MD, FAAP is a member of the following medical societies: American Institute of Ultrasound in Medicine and American Roentgen Ray Society
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Felix S Chew, MD, MBA, EdM is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

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