Scurvy is one of the oldest diseases in human history. Nowadays, although scurvy tends to become a forgotten disease in developed country, rare cases still occur, especially in people undergoing extreme diet, old people or children with poor diet and patients with malabsorption. We describe three cases of scurvy. The first case is a patient diagnosed with Crohn’s disease, the second one is in a context of anorexia nervosa and drug addiction, and the third case is in a context of social isolation. Early recognition of scurvy can be difficult because symptoms may appear nonspecific and can mimic more common conditions. In any patient with spontaneous hematoma and purpura, in the context of nutritional disorder, scurvy should be systematically considered. As this disease can lead to severe complications, such as bone pain, heart failure or gastrointestinal symptoms, nothing should delay vitamin C supplementation, which is a simple and rapidly effective treatment.
Scurvy is one of the oldest diseases in human history. The effects of a deficiency in vitamin C are well known and became a serious issue among mariners in the 15th and 16th century with devastating consequences on long marine expeditions.1
Nowadays, scurvy has become a forgotten disease in developed countries, yet cases still occur.
A 51-year-old woman with Crohn’s disease (since 1983) was hospitalized in a nutrition unit. She had several ileal resections in her past leading to short bowel disease with need for a parenteral nutrition.
The patient was hospitalized in a nutrition unit. She was apyretic and asthenic. The clinical examination revealed perifollicular purpura on the legs, with hematomas and corkscrew hair. She was suffering from bone pain on the right knee.
Results of primary investigations revealed normal hemostasis, normocytic normochromic anemia (hemoglobin: 9.6 g dl−1). Platelets, electrolytes, kidney function, vitamin B12, B9, albumin and iron status were in normal range.
A dermatologic examination was solicited and scurvy was diagnosed. Despite vitamin C supplementation in parenteral nutrition, ascorbic acid serum level was significantly decreased (<10 μmol l−1). The patient was given enteral ascorbic acid at a dose of 250 mg per day instead of 125 mg provided by the parenteral nutrition. The patient’s condition rapidly improved and the skin symptoms disappeared in 1 month.
A 38-year-old woman was hospitalized for purpura and spontaneous hematomas on the lower extremities. She had a history of drug addiction, chronic hepatitis C, iron deficiency and eating disorders including anorexia nervosa. She was not on any medications.
Clinical examination revealed purpura and perifollicular hyperkeratosis, hair dystrophy with corkscrew hair (Figure 1). Spontaneous hematomas were also observed on the legs. The initial diagnosis was scurvy.
The patient refused diagnostic investigations that led to a therapeutic test with 1 g of oral vitamin C daily for 1 month. Skin lesions gradually resolved, confirming the diagnosis of scurvy.
A 41-year-old man was hospitalized for confusion, in a context of social isolation. We learned from his family that he was divorced, unemployed and had a long history of alcohol intoxication and major depressive syndrome with suicide attempt. He was not on any medication.
Dermatological examination revealed purpura, perifollicular hyperkeratosis, corkscrew hair, hematomas on the legs and hemorrhagic gingivitis with tooth loss (Figure 2). Neurological findings included a polyneuropathy, confusion, disorientation with confabulation, false recognition and anosognosia. The rest of the clinical examination was normal.
Result of primary investigations revealed a normocytic normochromic anemia (hemoglobin: 7.4 g dl−1) secondary to an iron and folate deficiency (folate: 3 ng ml−1). Serum ascorbic acid was decreased (<3 μmol l−1).
A diagnosis of scurvy was made and the patient was treated with enteral ascorbic acid at a dose of 1 g per day for 1 month, associated with multivitamin supplementation, allowing a slow improvement of skin lesions. We noticed a significant improvement of the polyneuropathy but a remanent confusion due to a Korsakoff’s syndrome persisted.
Scurvy is a rare disease in developed countries and is confined to at-risk patient groups: precarious situations (social isolation, unemployement and homeless), elderly persons, individuals with purported allergies to multiple fruits and vegetables products, patients with psychiatric disorders or excessive alcohol consumption as alcohol decreased the absorption of vitamin C. Other at-risk groups include persons with gastrointestinal diseases, patients with a past history of bariatric surgery, cancer patients on chemotherapy who suffer from nausea, diarrhea and patients on hemodialysis.2
Humans do not have the ability to synthesize vitamin C and obtain 90% of their intake from fruits and vegetables. The total body pool of vitamin C is 1500 mg, and clinical manifestations of scurvy occur when this pool is reduced to <350 mg. To reach such a low level, vitamin C must be completely eliminated from the diet for 60–90 days.3 Clinical manifestations of scurvy can be seen within 8–12 weeks of irregular or inadequate intake. The recommended requirement of vitamin C is 75 mg per day for women and 90 mg per day for men.4
The diagnosis of scurvy is based on clinical features, dietary history and rapid resolution of symptoms after vitamin C supplementation. Patients with scurvy exhibit various systemic manifestations. Early stages are often characterized by weakness, asthenia, sluggishness, emaciation and myalgias because of the reduced production of carnitine. One to three months of inadequate intake can lead to more specific symptoms.5 Skin symptoms are characterized by follicular purpura with hyperkeratosis, corkscrew hair, spontaneous hematomas, subungual hemorrhage and petechiae that can lead to ecchymoses in 80% of the patients. Edema of the legs may occur.
Oral manifestations are common and include a hyperplastic ulcerative gingivitis, rapidly progressive chronic periodontitis and widespread mucosal hemorrhages.
In more advanced cases, patients may present bone pain (due to hemarthrosis or subperiosteal hemorrhage), gastrointestinal symptoms and heart failure (by hemopericardium). Ophthalmic manifestations include conjunctival hemorrhage, flame-shaped hemorrhage and cotton-wool spots at the ocular fundus.
Laboratory investigations are not necessary for the diagnosis. The concentration of ascorbic acid may be checked, but this value tends to reflect recent dietary intake rather than actual storage of vitamin C. A serum level below 2 mg l−1 suggests scurvy. Anemia occurs in 75% of patients because of blood loss and concomitant folate and iron deficiency.
The treatment for scurvy consists of vitamin C intake: 300 mg to 1 g per day orally for an adult for 1 month.6 Subjective improvement in fatigue, pain and anorexia typically occurs rapidly within 24 h. Joint swelling resolves in days, the ecchymoses in 1 or 2 weeks. By 4 weeks, the corkscrew hair regain normal growth. Complete recovery occurs after about 3 months.2
Early recognition of scurvy can be difficult because symptoms may appear nonspecific and can mimic other conditions. In any patient with spontaneous hematoma and purpura, especially in the context of nutritional disorder or inflammatory bowel disease, scurvy should be systematically considered. As this disease can lead to severe complications, nothing should delay vitamin C supplementation, which is a simple and rapidly effective treatment.
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The authors declare no conflict of interest.
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Levavasseur, M., Becquart, C., Pape, E. et al. Severe scurvy: an underestimated disease. Eur J Clin Nutr 69, 1076–1077 (2015). https://doi.org/10.1038/ejcn.2015.99
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