Scurvy is a nutritional disease caused by vitamin C deficiency that was epidemic on sailing voyages from the 15th to the 18th centuries and is now rare in developed countries.1

Vitamin C is a water-soluble vitamin that acts as a reducing agent and is necessary for collagen synthesis. Humans rely on diet and foods high in vitamin C include tomatoes, potatoes, and citrus fruits. The recommended dose is 90 mg/day for men and 75 mg/day for women.2 When vitamin C levels fall to less than 0.15 mg/dL, the typical symptoms of scurvy can develop, characterised by asthenia, joint pain, and vascular fragility, which can lead to petechiae, bleeding gums, haematomas, or hemarthrosis.3,4

A 49-year-old male with a history of an eating disorder during adolescence was referred to rheumatology for purpura in his lower extremities and bilateral knee arthritis of one month’s duration. During the anamnesis, the patient reported asthenia and episodes of epistaxis. In recent years, his diet had consisted primarily of dairy products, biscuits, and cereals.

On examination, he appeared cachectic, pale, bradypsychic, oedema with pitting of the lower limbs, and purpuric lesions on his extremities and abdomen. Furthermore, he exhibited arthritis in both knees, for which arthrocentesis was performed and haematic fluid was obtained. In addition, dermatology confirmed perifollicular haemorrhage by dermoscopy and biopsied one of the purpuric lesions (Fig. 1A), the pathology examination of which revealed chronic superficial dermatitis and perifollicular extravasation of blood without vasculitis (Fig. 1C).

Fig. 1.

A. Lower extremities at the time of diagnosis. B. Lower extremities following treatment. C. Pathology study of the purpuric lesion biopsied.

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Laboratory tests identified normocytic anaemia with associated iron deficiency and indirect evidence of malnutrition. The autoimmunity tests, coagulation study, serology, and radiographic studies were normal and the joint fluid culture was negative (Table 1).

In view of the high suspicion of scurvy, intravenous vitamin C was administered, and suppressed levels of vitamins C, A, K1, and zinc were confirmed in the following days (Table 1).

After two weeks of treatment, the patient improved clinically and analytically and was therefore discharged with vitamin complexes and remains asymptomatic at present (Fig. 1B).

While scurvy is rare in developed countries, cases are still reported among individuals at risk for malnutrition. Vitamin C deficiency alters vascular collagen and leads to bruising, purpura, arthritis, or arthromyalgia that may mimic vasculitis.

A review of the literature revealed 19 cases of scurvy in adults published in the 21st century (Table 2). Of these, 63% were male and had a mean age of 50 years. In almost all cases, the person had some medical or psychiatric history, and in one case, vitamin C deficiency was reported as a complication of enteral nutrition.17 The most frequent clinical manifestations were dermatological, including haematomas or ecchymosis, rash with perifollicular distribution, gingival bleeding, and the characteristic “corkscrew” hairs. Approximately 60% of all cases presented with articular manifestations, the most commonly observed ones being hemarthrosis in the large joints of the lower limbs (mainly knees and ankles) and polyarthralgias. In the cardiorespiratory system, several patients had dyspnoea on minimal exertion secondary to anaemia and/or heart failure.3,11 Other less frequent symptoms were pulmonary hypertension,11 arterial hypotension, and presyncopal episodes that might be attributable to the greater resistance of the blood vessels to the action of adrenaline.18,20 As an atypical manifestation of vitamin C deficiency in connective tissue, Francescone and Levitt reported a case of a 59-year-old male with osteopenia of the sacrum.

In our case, the history of an eating disorder, hemarthrosis, and desmoscopic findings enabled us to guide the diagnosis. Vitamin C levels are not routinely tested for in laboratory analyses and waiting for the result would delay diagnosis/treatment.

Differential diagnosis is made with purpura-related conditions (vasculitis, infections, idiopathic thrombocytopenic purpura, Ehlers–Danlos) or bleeding (coagulation disorders). Given that it is a pseudovasculitis, a biopsy is useful to rule out vascular inflammation.3

As regards treatment, the asthenia and skin lesions improved with vitamin C administration, whereas it took the arthritis several weeks to resolve. The anaemia disappeared after one month.5

Despite being an extremely rare deficiency disease in Western countries nowadays, scurvy continues to present in isolation in certain patients. The present case is a reminder of the importance of including this pseudovasculitis in the differential diagnosis of any patient with purpuric lesions or hemarthrosis.

The authors have no conflict of interests to declare.