|IMAGES IN RHEUMATOLOGY
|Ahead of print publication
Scurvy presenting as unilateral knee joint contracture
Harikrishnan Bhaskaran, Suma Balan
Department of Clinical Immunology and Rheumatology, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala, India
Department of Clinical Immunology and Rheumatology, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala
Source of Support: None, Conflict of Interest: None
Keywords: Diet, juvenile idiopathic arthritis, musculoskeletal, nutrition, scurvy
An 11-year-old boy, with suspected juvenile idiopathic arthritis, from Maldives, was admitted with an inability to stand due to right knee pain for 3 weeks. He had history of nonspecific leg pain in the past. There was no swelling, and it was episodic occurring once in 3–4 months, restricting his movements more after exertion. This used to settle on its own within few days, over the past 4 years. He was extensively evaluated during these years with rheumatoid factor, antinuclear antibody by immunofluorescence, complement levels, antineutrophil cytoplasmic antibodies, viral markers, and cryoglobulins which were not remarkable, but had mild anemia and raised erythrocyte sedimentation rate. His Vitamin D level was found to be very low 4 months before admission and his complaints were attributed to that. He was started on supplementation. However, his discomfort worsened progressively that he lost his ability to ambulate during the previous 3 weeks and was bedridden. He was admitted for further evaluation. On examination, he had hamstring contracture of the right side with knee in fixed flexion deformity [Figure 1]a and painful limitation of further examination. Neurological examination of other limbs and other systemic examination were within the normal limits. X-ray knee showed periarticular osteopenia. Magnetic resonance imaging (MRI) was done as an inability to bear weight was a “red flag sign” of malignancy. MRI of the thigh showed symmetrical short-tau inversion-recovery (STIR) T2 hyperintense signals in the proximal and distal metaphyses of the bilateral femur (white arrows in [Figure 1]b), with doubtful periosteal elevation in the left lower femur (yellow arrow in [Figure 1]b). MRI of the leg showed STIR T2 hyperintense signals in proximal and distal metaphyses of the bilateral tibia and fibula (white arrows in [Figure 1]c) with diffuse intramuscular edema in the gastrocnemius muscles bilaterally (yellow arrows in [Figure 1]c). Hypointense signals were seen in the corresponding areas in T1 images. Differential diagnoses considered were scurvy and acute myeloid leukemia. There are several case reports of scurvy presenting like juvenile idiopathic arthritis. The MRI findings in scurvy were also explained in the literature., Even though he does not belong to any high-risk category for nutritional deficiency or he did not have any petechiae or easy bruising, he had subtle gum hypertrophy and unhealthy hair. Further inquiry into the dietary history revealed a deficiency of vegetables and fruits in his diet. He had bizarre food habit that he existed exclusively on processed food such as noodles, biscuits, and milk and milk products. His previous extensive workup was negative; X-rays of the knees showed metaphyseal white line and submetaphyseal rarefaction. We thought of scurvy as the first possibility.
|Figure 1: (a) Right knee joint contracture at presentation. (b) Magnetic resonance imaging of the thighs short-tau inversion-recovery T2 showing hyperintense signals in the proximal and distal metaphyses of the bilateral femur (white arrows), with doubtful periosteal elevation in the left lower femur (yellow arrow). (c) Magnetic resonance imaging of the leg showing short-tau inversion-recovery T2 hyperintense signals in the proximal and distal metaphyses of the bilateral tibia and fibula (white arrows) with diffuse intramuscular edema in the gastrocnemius muscles bilaterally (yellow arrows). (d) The patient walks without support after 3 weeks of starting Vitamin C supplementation|
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Vitamin C supplementation (500 mg po Q8H) was started after sending the blood for Vitamin C level estimation. He could bear weight within 48 h of starting supplementation. Plasma Vitamin C level reports came after 2 weeks and it was <0.1 mg/dL (normal range: 0.2–2.1). His contracture disappeared and he could walk without support within 3 weeks [Figure 1]d. The follow-up test for Vitamin C level was not done as the serum levels generally reflect recent intake. Since he was on Vitamin C, we thought the level would be high. The diagnosis of scurvy was confirmed as he improved very well with Vitamin C supplementation alone. Now, after 10 months of follow-up, he is fine.
| Take-Home Messages|| |
It is true that children with developmental disorders with fastidious dietary habits have high chance of scurvy. However, it should be suspected even in an otherwise normal child, with bizarre food habits with very severe pain restricting ambulation, in the absence of other obvious possible causes.
In ambulatory children, musculoskeletal manifestations can precede gum changes and bleeding manifestations. Therefore, it is very important for a rheumatologist to consider scurvy in a child with musculoskeletal manifestations.
X-rays, initially, and response to Vitamin C supplementation are the cornerstone to diagnosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Harikrishnan B, Balan S. Scurvy mimicking juvenile idiopathic arthritis in two pediatric patients. Indian J Rheumatol 2018;13:273. [Full text]
Perkins A, Sontheimer C, Otjen JP, Shenoi S. Scurvy Masquerading as Juvenile Idiopathic Arthritis or Vasculitis with Elevated Inflammatory Markers: A Case Series. J Pediatr 2020;218:234-700.
Smith A, Di Primio G, Humphrey-Murto S. Scurvy in the developed world. CMAJ 2011;183:E752-5.