Scurvy presenting primarily as gingival manifestation in a young child: a diagnostic dilemma

Vitamin C deficiency, historically known as scurvy, was associated with sailors in the Victorian times, however, a global review in 2020 suggests it still exists in certain at-risk groups. A case is presented of a young non-verbal child with learning difficulties and on a restricted diet, in which the primary symptom was gingival inflammation. It posed a diagnostic dilemma due to the non-specific symptoms, and a delay in the diagnosis, until vitamin C deficiency was confirmed. Gingival inflammation is one of the common findings in vitamin C deficiency and dental professionals may be the first point of contact. The importance of dietary evaluation, identifying and looking for other signs and liaising with the medical colleagues are discussed. This case highlights the role of the dentist in identifying latent cases of vitamin C deficiency and to consider this as a differential diagnosis especially in certain at-risk groups.


SUMMARY
Vitamin C deficiency, historically known as scurvy, was associated with sailors in the Victorian times, however, a global review in 2020 suggests it still exists in certain at-risk groups. A case is presented of a young non-verbal child with learning difficulties and on a restricted diet, in which the primary symptom was gingival inflammation. It posed a diagnostic dilemma due to the non-specific symptoms, and a delay in the diagnosis, until vitamin C deficiency was confirmed. Gingival inflammation is one of the common findings in vitamin C deficiency and dental professionals may be the first point of contact. The importance of dietary evaluation, identifying and looking for other signs and liaising with the medical colleagues are discussed. This case highlights the role of the dentist in identifying latent cases of vitamin C deficiency and to consider this as a differential diagnosis especially in certain at-risk groups.

BACKGROUND
Vitamin C deficiency has been historic, however, a 2008 UK survey revealed that 25% men and 14% of women from low-income populations had vitamin C deficiency (<11 µmol/L) and a further 20% of the population had levels in the depleted range (11-28 µmol/L). 1 A recent review of global vitamin C status indicated a high prevalence of vitamin C deficiency in individuals with lower socioeconomic status or higher deprivation particularly in low-income and middle-income countries. 2 The National Diet and Nutrition Surveys (2008-2017) have shown a significant 6% increase in vitamin C deficiency over the 9-year period in women aged 19-64. 2 Certain risk groups are associated with dietary insufficiency of vitamin C, such as alcoholics with poor diets, babies purely fed on cow's milk, individuals with eating disorders or restrictive diets, food allergies, children with autistic spectrum disorders with a limited food repertoire and individuals in nursing homes or care homes over the age of 65. [3][4][5][6] Vitamin C deficiency can be due to an underlying primary disease such as inflammatory bowel disease, individuals with iron overload which leads to wasting of vitamin C by kidneys, type 1 diabetes who have higher vitamin C requirements and not necessarily due to lack of vitamin C in their diet. 7

CASE PRESENTATION
A young Asian boy with severe learning difficulties, non-verbal and a wheel chair user, presented to the clinic with his mother and father. His mother was the main carer with limited understanding of English, and father gave the history. Their main concern was their son's swollen, bleeding gums, which had been present for 2-3 weeks. As tooth brushing was generally difficult, his parents assumed the bleeding gums was related to that and would resolve, but his condition had worsened and his mother stated that his behaviour had changed and he was more lethargic. They reported that his appetite was reduced. The patient showed signs of discomfort and his mother recognised that he was in pain and gave him analgesics regularly. They did not feel that his gums had been swollen before. The medical history included Cerebral Palsy with mixed tone pattern and truncal hypotonia, global developmental delay, central visual impairment, well controlled epilepsy, beta thalassaemia trait. Medication-included levetiracetam 100 mg two times per day and allergy to chicken and egg products. The diet was mainly pureed due to swallowing difficulties including supplements like pediasure and a cooked lentil diet. Toothbrushing was carried out by his mother twice daily using fluoridated toothpaste.
The patient had been seen by the community dental service pre-covid. There were deposits of calculus, which were removed by hand scaling of his teeth due to limited co-operation, and appropriate oral hygiene instructions were advised. The follow-up recall appointment did not take place due to COVID-19. The child lived with his mother and father and had two other siblings and attended a special needs school but was cared for at home due to COVID-19 restrictions at that time.
The patient was examined in his wheelchair assisted by his mum. Extraoral lymph nodes were not palpable and no other abnormalities were evident. However, he appeared very weak. Intraorally, examination was limited. Clinical examination revealed generalised erythematous swollen marginal gingiva involving the interdental papilla and extending to mucogingival junction. The consistency was spongy but palatally appeared like large gingival finger-like swellings with spontaneous bleeding. It was extremely tender to touch. Some calculus deposits were visible and the gingiva appeared to be detached. The vestibule and the tongue were not affected. No obvious ulceration was evident on examination (figure 1).
Treatment on that day was limited to the anterior teeth only due to limited cooperation. The gingiva was gently wiped using gauze soaked with chlorhexidine −0.2% mouthwash. This simple treatment involved a lot of bleeding and required pressure with gauze to achieve haemostasis. Parents were given oral hygiene instructions, to use a soft toothbrush and prescribed 0.2% chlorhexidine mouthwash with instructions to wipe the gums gently with a gauze soaked in mouthwash. They were advised to hydrate with fluids and to use analgesic prior to feeding the child to minimise discomfort. They were also advised to seek emergency assistance if the child became unwell. The patient was urgently referred to his general medical practitioner and oral medicine department for haematological investigations.

DIFFERENTIAL DIAGNOSIS
The differential diagnosis included necrotising ulcerative gingivitis, acute herpetic gingivostomatitis due to the severity of the inflammation, leukaemia, desquamative gingivitis seen in immune-mediated and autoinflammatory gingival lesions,bleeding disorders and gingival hyperplasia due to antiepileptic medication 8 presenting as reactive hyperplastic tissue.
Following immediate referral to oral medicine colleagues and his general medical practitioner, he was admitted to hospital for further investigations. The initial blood tests revealed very low haemoglobin levels, so an initial diagnosis of iron deficiency anaemia was made. Leukaemia was ruled out as white cell count and platelet count was in the normal range. His condition did show some improvement with antibiotics but the gingival inflammation persisted. Later, testing of vitamin C levels revealed severe vitamin C deficiency; less than 2.4 µmol(table 1). He was admitted for a total of 10 days.

TREATMENT
The patient received ascorbic acid tablets (250 mg) for a week and showed remarkable gingival improvement with in a few days of treatment. There was a concomitant vitamin D deficiency as well. He was also prescribed colecalciferol liquid (vitamin D deficiency) 6000 units once a day and sodium feredetate (iron-deficiency anaemia) 190 mg/5 mloral solution sugar free for 4 months.

OUTCOME AND FOLLOW-UP
Review 4 weeks later in dental clinic showed spontaneous resolution of the gingival tissues (figure 2). Follow-up 2 months later in clinic did not show any recurrence. He had regular follow-up appointments with his paediatrician and dietician to improve his diet.

DISCUSSION
This case demonstrated vitamin C deficiency presenting with gingival manifestations due to restricted dietary intake. Other symptoms such as pain in the limbs and difficulty in walking were not possible to assess as the child was non-verbal and nonmobile. Vitamin C deficiency manifestations are mainly associated with defective collagen synthesis and hence increased vascular fragility leads to a bleeding tendency which produces petechiae and ecchymosis.
In hindsight, a thorough dietary history would have given more information. His mother reported that he was mostly bottle-fed until the age of five due to difficulties with swallowing. A 5-day diet sheet showed severely restricted food intake, with allergy to egg products and chicken limiting his diet. He was fed cereals for breakfast and although his meals included cooked rice and lentils, there was no mention of fruits or juices in his diet. Liaison with the dietician revealed that his mother was boiling the lentils with vegetables at a high temperature to suit a pureed diet due to his swallowing difficulties; vitamin C being thermolabile was destroyed at high temperature, which lowered the nutritive value. His siblings did not have any reported problems as they had a more varied diet and no underlying medical problems.
Studies have investigated oral manifestations of scurvy in children with neurodevelopmental disorders, suggesting that in those with medical or developmental conditions like autistic spectrum disorders 9-13 maybe a presentation due to a restricted diet or food selectivity. They identified gingival swelling,pain,and bleeding as the classic oral features of scurvy.Limp, leg pain and refusal to walk, anaemia were some of the other common findings in these studies.    A Canadian study cited that scurvy should be a 'never event' in a healthy society and that a confident clinical diagnosis of scurvy was made in 12 cases out of a total of 52 admissions with low levels of vitamin c. 14 In a more recent systematic review of literature by Gicchino et al showed that 51% of patients presenting with scurvy were affected by neurodevelopmental disorders and 83% of these patients, presented with oral manifestations. The most common oral manifestation cited in their review were gingival bleeding, swelling and hypertrophy. 15 As oral manifestation is a common presenting feature in scurvy, we would like to highlight that dentists are in a unique position to identify nutritional deficiencies in children, especially in children with learning difficulties on a limited food repertoire and in those described as picky or fussy eaters. It is important to be vigilant and look for signs and symptoms of nutritional deficiencies through dietary evaluation.
We would also like to remind medical professionals to consider Scurvy as a potential diagnosis while evaluating patients presenting with musculoskeletal and cutaneous manifestations, to enquire for the common oral manifestations as well; to aid prompt diagnosis of vitamin C deficiency. Vitamin C deficiency still exists and as clinicians we should be aware of the early oral, cutaneous and musculoskeletal manifestations, especially in children with autism and developmental disorders and liaise with medical professionals to avoid the delay in diagnosis. Nonaccidental injuries should be considered in any case of bruising in atypical areas, and rule out physical injuries due to child abuse.
Hence this case presentation is to promote awareness among dentists and medical teams regarding nutritional deficiencies such as vitamin C presenting with non-specific symptoms and to consider this in our differential diagnosis.