BACKGROUND

Historically, scurvy has been associated with sailors undertaking long voyages. This disease has been known since ancient Egypt, but nowadays it is almost forgotten. Although its prevalence has decreased over the centuries, scurvy is still present in developed countries.1 Scurvy is the clinical manifestation of ascorbic acid deficiency and occurs in people at risk for chronically inadequate nutrition. Doctors should be aware of the clinical features because initial symptoms are non-specific. When suspected, scurvy is easily treatable.2 We report the case of a man with typical features of scurvy induced by obsessive–compulsive disorder.

CASE PRESENTATION

A 61-year-old man was referred to our hospital with a 30-day history of anorexia, fatigue, gingival bleeding and echimosis on his arms and legs. He also complained of a painful palatal lesion with severe halitosis. On physical examination, he was malnourished, with significant gingival hypertrophic lesions and signs of chronic periodontitis. The hard palate presented a painful friable lesion. Examination of his arms and legs showed petechial rash and several bruises at various stages of resolution (fig 1).

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Figure 1

Photograph showing vitamin C deficiency signs. Petechial rash on the left leg (A), gingival hypertrophic lesions (B,C), echimosis on the legs (D).

A food frequency questionnaire revealed a long history of poor diet, with no vegetables or fruit, and ascorbic acid ingestion of 1.5 mg/day. The patient had ingested only chocolate milk and cookies for the last 10 years due to fear of pesticides being present in foods of vegetable origin. In addition, he presented other signs of behaviour disorder such as he did not brush his teeth (he was afraid of chemical products), and he always stayed at home in winter despite adequate temperature. Consequently, the patient was evaluated by a psychiatrist who diagnosed obsessive–compulsive disorder.

INVESTIGATIONS

Laboratory data showed an activated partial thromboplastin time ratio of 0.98 (reference range (RR) up to 1.20), prothrombin time international normalised ratio (INR) 0.98, haemoglobin 10.4 g/dl (RR: 14–18 g/dl), haematocrit 31.6% (RR: 40% to 57%) and mean cell volume 84 fl. White blood cell count was 8300/mm³ and lymphocyte count 382/mm³. Reticulocyte count was 4.43% (RR: 0.5% to 2.0%). Lactate dehydrogenase was 805 UI/litre (RR: 313–618 UI/litre) and total bilirubin 2.0 mg/dl (RR: 0.2–1.3 mg/dl) due to indirect bilirubin elevation. Histopathology of the palatal lesion biopsy showed a pyogenic granuloma while bone marrow aspiration revealed megaloblastic haemopathy with bone marrow hypercellularity and haemosiderin deposits.

DIFFERENTIAL DIAGNOSIS

The clinical presentation is applicable to a range of differential diagnoses, such as haematological abnormalities, trauma, infections and autoimmune disorders.

TREATMENT

Based on clinical signs and alimentary history, treatment with vitamin C, 1 g orally on the first day and 500 mg three times daily, was started. Ferrous sulfate, folic acid, vitamin B complex and paroxetine were also prescribed.

OUTCOME AND FOLLOW-UP

Within 1 day of ascorbic acid therapy, there was marked diminishment of weakness. Within 2 weeks there were improvements in ecchymosis on the arms and legs, gingival swelling and palate lesion (fig 2).

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Figure 2

Photograph showing patient after treatment. A,D. Improvement of petechial rash and echimosis. B,C. Improvement of gingival swelling.

DISCUSSION

Scurvy has been known since ancient times, and historically it has been associated with sailors and soldiers, when only non-perishable food could be stored in ships sailing overseas. There is a report of scurvy in Egyptian hieroglyphs from around 1500 BC.2 Scurvy was very common in 19th century Europe, where climatic conditions were not favourable for growing fruit and vegetables, and was endemic in sailors who undertook great navigational journeys. However, scurvy prevention by citrus fruits was first described in 1753 by Sir James Lind, and ascorbic acid was first isolated in 1928.3 Nowadays, classical disease manifestations are rare. Scurvy has been described in people unaware of proper nutrition, or who follow diets with severe alimentary restriction. Gastrointestinal diseases that disable vitamin C absorption, alcoholism, patients submitted to chemotherapy and carriers of psychiatric disorders are also at risk for ascorbic acid deficiency.4 Our patient had a long history of a poor diet, with no vegetables or fruit, due to his obsessive–compulsive disorder.

Humans are one of the few mammals that cannot synthesise vitamin C, so they need an exogenous source of ascorbic acid, which is found primarily in citrus fruits and green vegetables. The daily ingestion of vitamin C recommended by the Food and Nutrition Board is 75 mg/day for men and 60 mg/day for women. Clinical features begin when daily ingestion is 6–10 mg or less for a 60–90 day period.5 In this report, the patient’s ingestion of vitamin C was only 1.5 mg/day.

Vitamin C is essential for several organic functions. It is responsible for collagen hydroxylation, and for the synthesis of carnitine, norepinephrine and tyrosine. Ascorbic acid also promotes iron absorption due to reduction of ferric iron to the ferrous form, and acts as antioxidant.6^,7 Vitamin C is a necessary cofactor in collagen biosynthesis. Collagen consists of three long polypeptide chains coiled into a triple helix. The synthesis of these polypeptides occurs initially in ribosomes as procollagen molecules; lysine and proline residues are then hydroxylated using vitamin C as a cofactor. Clinical manifestations of scurvy are attributable to depletion of pericapillary collagen and capillary fragility.6^,7

Early features include weakness, fatigue and myalgia. These symptoms could also be due to anaemia, which is usually normocytic normochromic, and occurs in 75% of patients with scurvy. Altered iron absorption, blood loss and folate deficiency are the principal causes of this anaemia. In our patient, bone marrow aspiration showed megaloblastic haemopathy with bone marrow hypercellularity and haemosiderin deposits. Subsequent classic features of scurvy, such as petechiae, ecchymosis, gingival hypertrophic lesions, pyogenic granuloma, haemarthroses and bleeding, occur later in the disease course. The clinical presentation is applicable to a range of differential diagnoses, such as haematological abnormalities, trauma, infections and autoimmune disorders. An accurate history, complete blood cell count and coagulation studies helped to exclude these diagnoses in our patient. Therefore, as recommended by the literature, a diagnosis of scurvy was made based on clinical characteristics, alimentary history and improvement of symptoms after treatment with vitamin C.2^,7

Ascorbic acid dosage is not necessary for diagnosis and is only made in atypical clinical presentations. The levels can be assessed in plasma, and values lower than 11 μmol/litre suggest scurvy. Measuring vitamin C levels in buffy coat leukocytes better reflects body stores of this vitamin than plasma levels.3 However, this process is not used in clinical practice because of high cost and technical difficulties. Therefore, the best evidence of the presence of scurvy is the resolution of the manifestations of the disease after ascorbic acid treatment.3

Treatment is based on ascorbic acid supplementation. The recommended dosage is 1 g/day for the first 5 days, followed by 0.5 to 1.5 g/day for 1 month. Vitamin C is usually safe and well tolerated, although ingestion higher than 3 g/day can cause renal lithiasis, diarrhoea and haemolysis in patients with G6PD deficiency. Despite vitamin C supplementation, other nutritional deficiencies should be treated, including iron-deficiency anaemia and deficiency of folate, zinc and other vitamins.5^,6

As occurred in our patient, weakness and fatigue usually improve in 24 h, gingival alterations in 2 weeks and petechiae and ecchymosis in 3–4 weeks.

This case report reinforces the fact that, although rare, scurvy is still present in people at risk for chronically inadequate nutrition. Even if vitamin C cannot be measured, empirical treatment should be implemented for this forgotten and easily curable disease.