Pseudovasculitis: an etiology not to miss

A Correction to this article was published on 01 February 2021

This article has been updated

Presentation

A 63-year-old male with dementia was referred to dermatology with a rash concerning for vasculitis. He denied itch or pain from the rash but noted pain at his right calf. On examination, there were peri-follicular purpuric macules and hyperkeratotic papules with corkscrew hairs. Conjunctival hemorrhage, hemorrhagic gingivitis, splinter hemorrhages, and a large ecchymosis with ultrasound-confirmed hematoma of the right calf were noted (Fig. 1a, b). On review, his wife shared that his diet had been limited since he developed dementia, consisting exclusively of grains and dairy. Given the physical findings and history of restricted diet, a presumptive diagnosis of scurvy was made. An undetectable serum ascorbic acid level, < 0.1 mg/dL confirmed the diagnosis. The patient was started on ascorbic acid 1000 mg daily; his rash improved after 2 weeks of supplementation and resolved after 6 weeks.

Fig. 1
figure1

Cutaneous manifestations of vitamin C deficiency. a Peri-follicular purpuric macules and hyperkeratotic papules with corkscrew hairs. Splinter hemorrhages. b Gingival hemorrhage

Discussion

Vitamin C is an essential water-soluble vitamin exclusively obtained through diet, it is essential for collagen synthesis, and its deficiency leads to impaired collagen synthesis and capillary fragility [1]. Vitamin C deficiency is rare in the developed world and is typically seen in patients with alcohol use disorder, restrictive dieting, psychiatric disease, dementia, and malabsorptive diseases. The first manifestation of vitamin C deficiency is typically fatigue. Corkscrew hairs with peri-follicular petechiae are pathognomonic for scurvy [2]. Musculoskeletal manifestations including arthralgias, particularly in the knees, ankles, and wrists, as well as myalgias, are seen in up to 80% of patients. Hemarthroses are also commonly seen and may be the initial chief complaint of the patient [3]. Hemarthroses have been found to occur mainly in the hips, knees, and ankles and are believed to be the result of damage to synovial blood vessels and microfractures [4]. More rare musculoskeletal manifestations include regional transient osteoporosis [5]. Early recognition of key physical findings is important to ensure prompt treatment.

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Change history

  • 01 February 2021

    A correction to this paper has been published: 10.1007/s10067-021-05615-9

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All authors contributed equally to the conception and design of this submission. SAE primarily drafted the manuscript. DRM and JFM both read, edited, and approved the submission in its final form.

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Correspondence to Joseph F. Merola.

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The original online version of this article was revised: In the original published version of the above article, the legend of Fig. 1 was revised. The legend for Figure 1b mentioned splinter hemorrhages. However, there are no splinter hemorrhages in Figure 1b, only in Figure 1a.

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Elman, S.A., Mazori, D.R. & Merola, J.F. Pseudovasculitis: an etiology not to miss. Clin Rheumatol (2021). https://doi.org/10.1007/s10067-020-05548-9

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