Scurvy presenting as lower limb ecchymoses in the setting of metastatic colorectal cancer.

A 58-year-old woman presented with a 1-week history of lower limb bruising. She had a medical history of recurrent metastatic colon cancer with a sigmoid colectomy and complete pelvic exenteration leading to colostomy and urostomy formation. She had malignant sacral mass encroaching on the spinal cord. This caused a left-sided foot drop for which she used an ankle-foot orthosis. She was on cetuximab and had received radiotherapy to the sacral mass 1 month ago. On examination, there were macular ecchymoses with petechiae on the lower limbs. There was sparing of areas that had been compressed by the ankle-foot orthosis. Bloods showed mild thrombocytopaenia and anaemia with markedly raised inflammatory markers. Coagulation studies consistent with inflammation rather than disseminated intravascular coagulation. She was found to have Klebsiella bacteraemia secondary to urinary source. Skin biopsy showed dermal haemorrhage without vessel inflammation. Vitamin C levels were low confirming the diagnosis of scurvy.

The neuropsychiatric effects of vitamin C deficiency: a systematic review.

BACKGROUND: Vitamin C deficiency may be more common than is generally assumed, and the association between vitamin C deficiency and adverse psychiatric effects has been known for centuries. This paper aims to systematically review the evidence base for the neuropsychiatric effects of vitamin C deficiency. METHODS: Relevant studies were identified via systematic literature review. RESULTS: Nine studies of vitamin C deficiency, including subjects both with and without the associated physical manifestations of scurvy, were included in this review. Vitamin C deficiency, including scurvy, has been linked to depression and cognitive impairment. No effect on affective or non-affective psychosis was identified. CONCLUSIONS: Disparate measurement techniques for vitamin C, and differing definitions of vitamin C deficiency were apparent, complicating comparisons between studies. However, there is evidence suggesting that vitamin C deficiency is related to adverse mood and cognitive effects. The vitamin C blood levels associated with depression and cognitive impairment are higher than those implicated in clinical manifestations of scurvy. While laboratory testing for ascorbic acid can be practically difficult, these findings nonetheless suggest that mental health clinicians should be alerted to the possibility of vitamin C deficiency in patients with depression or cognitive impairment. Vitamin C replacement is inexpensive and easy to deliver, although as of yet there are no outcome studies investigating the neuropsychiatric impact of vitamin C replacement in those who are deficient.

Scurvy Masquerading as Juvenile Idiopathic Arthritis or Vasculitis with Elevated Inflammatory Markers: A Case Series.

Ten patients with scurvy were evaluated by rheumatology; we review their clinical, laboratory, and dietary presentations. Eight patients had developmental delay or autism. All had elevated inflammatory markers. These clinical and laboratory features with imaging findings can mimic rheumatic conditions such as arthritis, vasculitis, and chronic nonbacterial osteomyelitis (CNO).

Modern American scurvy - experience with vitamin C deficiency at a large children's hospital.

BACKGROUND: Until recently scurvy has been viewed in developed countries as a disease of the past. More recently there have been reports of case series of children with scurvy who have had a delayed diagnosis after an extensive diagnostic workup that included imaging. Most of these children have had underlying neurologic conditions such as autism. OBJECTIVE: To review the medical records of children diagnosed with vitamin C (ascorbic acid) deficiency based on serum ascorbic acid levels at a large pediatric health care system, to determine imaging findings and utility of imaging in management, and to identify at-risk pediatric populations. MATERIALS AND METHODS: We retrospectively identified cases of vitamin C deficiency in children tested for serum ascorbic acid levels during the last 5 years. We used the criteria of normal ascorbic acid >23 µmol/L and included children with ascorbic acid levels <23 µmol/L. We evaluated their clinical history, underlying medical condition, imaging studies obtained and imaging findings. RESULTS: We identified 32 children with vitamin C deficiency. All of these children had underlying medical conditions, most commonly iron overload from multiple transfusions related to sickle cell anemia or thalassemia (20), neurologic disorders (4) and bone marrow transplant/chemotherapy (3). No cases of scurvy from dietary deficiency in otherwise normal children were identified. All except two children had multiple imaging studies, primarily related to their underlying conditions. Three of these children had extensive imaging workups related to diffuse musculoskeletal pain. Imaging findings included ill-defined sclerotic and lucent metaphyseal bands (mainly at the knee) on radiography and MRI studies that showed diffuse increased T2-weighted signal in the bilateral lower-extremity long-bone metaphyses, periosteal reaction and adjacent soft-tissue edema. CONCLUSION: Vitamin C deficiency is not uncommon in large pediatric health care facilities, and it is frequently missed on clinical evaluation and diagnostic imaging. At-risk populations include those with iron overload, neurologic conditions and history of chemotherapy. Scurvy related to dietary deficiency in otherwise normal children was not encountered. When characteristic MRI findings are seen, particularly in children with a predisposing condition for vitamin C deficiency, scurvy should be considered and a serum ascorbic acid level checked to potentially confirm a diagnosis prior to further invasive tests.

[Atypical scurvy associated with anorexia nervosa]. / Scorbut atypique associé à une anorexie mentale.

INTRODUCTION: Scurvy, or "Barlow's disease", is a widely described disease involving cutaneous and mucosal lesions resulting from vitamin C deficiency. Herein, we report a case of scurvy in a 48-year-old woman that was unusual in its atypical cutaneous-mucosal presentation as well as its association with anorexia nervosa. PATIENTS AND METHODS: A 48-year-old woman treated for depression for several years was admitted to hospital for her impaired general state of health. Over the last year, she had presented palmoplantar rash and episodes of perimalleolar oedema. The clinical examination showed the patient to have wasting syndrome, with a BMI of 11.9kg/m2, lower-limb oedema, palmoplantar fissures, geographic tongue, telogen effluvium and purpuric petechiae on her right knee. However, no gingival bleeding was noted and there was no loss of tooth enamel. The remainder of the clinical examination was normal. Blood tests revealed extremely low vitamin C levels without any other associated deficiencies, as well as laboratory signs of cytolysis and anicteric cholestasis without inflammatory syndrome. The diagnosis of anorexia nervosa was made by psychiatrists, despite the unusual age of onset. Favorable clinical outcome was rapidly achieved via a one-month course of vitamin C supplements at a daily dose of 1g. DISCUSSION: The absence of classical buccal-dental symptoms and the presence of keratotic dermatosis with fissures and ulcers on the hands and feet are atypical in scurvy; however, this diagnosis was confirmed by the existence of purpura evoking capillary fragility, the patient's drastically low vitamin C level and the rapid subsidence of symptoms following treatment with oral vitamin C alone. Anorexia nervosa was doubtless the cause of deficiency. This situation is rare and a systematic review of the literature in Medline via PubMed showed that only three reports of scurvy associated with mental anorexia have been published since 1975.

Severe vitamin C deficiency in a critically ill adult: a case report.

Scurvy, a severe form of vitamin C deficiency, killed scores of people until its cause and treatment were firmly established at the end of the eighteenth century. Since then, cases have surged periodically around the world, mostly in developing countries and during times of war and famine. In developed countries, scurvy is still endemic and evidence is growing that vitamin C deficiency might affect up to 30 percent of the population. Low socio-economic status, alcoholism, severe psychiatric illness leading to poor nutrition and critical illness are significant risk factors. We hereby report the case of a patient admitted in a Swiss intensive care unit of a tertiary teaching hospital and presenting with clinical signs and symptoms of severe vitamin C deficiency.

Rapid and convenient detection of ascorbic acid using a fluorescent nitroxide switch.

Ascorbic acid is a small-molecule reductant with multiple functions in vivo. Reducing ascorbic acid intake leads to a lack of hydroxylation of prolines and lysines, causing a looser triple helix and resulting in scurvy. Ascorbic acid also acts as an antioxidant to prevent oxidative stress. Because ascorbic acid is related to disease states, rapid and convenient detection of ascorbic acid should be useful in diagnosis. Nitroxide is reduced to the corresponding hydroxylamine by ascorbic acid and a sensitive and novel approach to its detection employs covalent coupling of nitroxide with a fluorophore, leading to intramolecular quenching of fluorescence emission by electron-exchange interactions. Here, we developed a new fluorophore-nitroxide probe, Naph-DiPy nitroxide, for ascorbic acid. Naph-DiPy nitroxide rapidly reacted with ascorbic acid and showed fluorescence enhancement, but not in response to other reductants or reactive oxygen species. To confirm the practical usefulness of the fluorophore-nitroxide probe, we demonstrated the use of Naph-DiPy nitroxide for the measurement of ascorbic acid in the plasma of osteogenic disorder Shionogi rats when fed an ascorbic acid-deficient diet. The results suggest that this novel fluorophore-nitroxide probe could sensitively and easily detect ascorbic acid and be useful as a tool for the diagnosis of disease states.

Reduced platelet aggregation in a boy with scurvy.

Pediatric scurvy is a rare condition characterized by perifollicular petechiae and bruising, hemorrhagic gingivitis and musculoskeletal symptoms, all assumed to be predominantly related to abnormal collagen structure. We report on a 9-year-old autistic boy with vitamin C deficiency due to a highly limited food range presenting with multiple petechiae, gum bleeding and debilitating bone pain, in whom platelet aggregometry revealed a distinctly reduced thrombocyte aggregation, normalizing after vitamin C supplementation. This observation indicates that platelet dysfunction may additionally contribute to the hemorrhagic diathesis in scurvy, and demonstrates that ascorbic acid deficiency should be considered in children with an otherwise unexplained acquired thrombocytopathy.

[Eat a citrus fruit, stay healthy--a case report of scurvy].

Scurvy is a disease that results from a vitamin C deficient diet. Since vitamin C is available in many food products, and especially in citrus fruits, the disease is rare in developed countries. Clinical manifestations of scurvy include general weakness, cutaneous and gum bleeding, pain in the lower limbs and inability to stand and walk (pseudo paralysis). The diagnosis of scurvy requires a high level of clinical suspicion, typical radiographic features and low Levels of vitamin C in the plasma. We report a case of a 7-year-old patient with a medical history of hydrocephalus, failure to thrive and severe psychomotor retardation due to complications of prematurity. On admission she had gum bleeding, severe anemia, pain in the lower limbs and refused to stand and walk. According to her parents, her diet was restricted, without vegetables or fruit consumption. Our investigation ruled out coagulopathy, malignancy and infection. Serum vitamin C levels were low and radiographic findings were consistent with the diagnosis of scurvy. The patient improved rapidly after the initiation of vitamin C supplements. Despite being rare, scurvy should be considered in the differential diagnosis of bleeding and pain in the lower limbs, especially in a malnourished patient.

Scurvy in hospitalized elderly patients.

OBJECTIVES: The aim of this study was to systematically screen hospitalized elderly patients for clinical symptoms of scurvy and to confirm the diagnosis with biological measures. SETTINGS: Geriatric acute care ward. MEASUREMENTS: Scurvy symptoms (one or more among perifollicular hyperkeratosis, petechiae or bruises, haemorrhagic features caused by venous puncture, severe gingivitis). We compared associated diseases, nutritional status, need for assistance for feeding, serum albumin, transthyretin, B9 and B12 vitamins, iron status and Serum Ascorbic Acid Level (SAAL) and outcome (in-hospital mortality) between scurvy and scurvy free patients. RESULTS: 18 patients with clinical symptoms of scurvy (scurvy group) were identified out of 145 consecutive patients (12%). They were compared to 23 consecutive control patients with no clinical symptoms of scurvy (scurvy-free group). SAAL was significantly lower (1.09 +/- 1.06 vs 4.87 +/- 4.2 mg x L-1, p < .001) and vitamin C deficiency more frequent (94 vs 30 %, p < .001) in the scurvy group. Moreover, in scurvy group, coronary heart disease (39 vs 9 %, p=.028), need for assistance for feeding (56 vs 13 %, p=.006) and in-hospital deaths (44 vs 9 %, p=.012) were more frequent. CONCLUSION: Ninety-four percent of patients with clinical symptoms of scurvy had vitamin C deficiency. Our results suggest that in hospitalized elderly patients, clinical symptoms allow scurvy diagnosis. Scurvy could be a frequent disease in elderly patients admitted to acute geriatric ward.

Case report: scurvy in an epileptic child on a ketogenic diet with oral complications.

BACKGROUND: Epilepsy is a symptom of cerebral dysfunction, where there is a sudden and disorganised discharge of electrical activity from a group of neurones, producing symptoms that range from sensory absences to convulsive movements and unconsciousness. Fasting is recognised as reducing the frequency of epileptic seizures in difficult to control patients. The ketogenic diet is a high fat, low carbohydrate and adequate protein diet that mimics the biochemical effects of fasting. It is deficient in some essential elements that require supplementation. CASE REPORT: A 9-year old girl with learning difficulties, developmental delay and refractory epilepsy was placed on the ketogenic diet in 2003. Prior to starting the diet she had had as many as 12 tonic seizures/day, with prolonged periods of non-convulsive status epilepticus. Subsequent to being placed on the diet, the frequency of her seizures reduced markedly; there were long periods during which she had none. In late 2006, the patient inhaled a primary molar. This was retrieved by emergency bronchoscopy and at the same time the remaining primary teeth were extracted. Three weeks later she was admitted to hospital with low-grade fever, persistently bleeding sockets, oedema of her hands and feet, a petechial rash and bruising. A differential diagnosis included: liver disease, bleeding dyscrasia, oncological pathology or scurvy. The most striking finding amongst a number of investigations was a vitamin C level of 0.7 micromol/l (Deficiency: < 11 micromol/l). Accordingly a diagnosis of scurvy was made. TREATMENT: The patient was prescribed ascorbic acid 500 mg twice/day. Three weeks later the patient's vitamin C level was 141.5 micromol/l; the dose was therefore reduced to 250 mg once/day. FOLLOW-UP: At two-month review, the signs and symptoms of scurvy had resolved. CONCLUSION: Inhaling a tooth and scurvy are both rare occurrences. Paediatric dentists should be aware of the possible implications of a ketogenic diet.