Scurvy Masquerading As Reactive Arthritis
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CASE LETTER Scurvy Masquerading as Reactive Arthritis Karen L. Christopher, MD; Kelly K. Menachof, MD; Ramin Fathi, MD (Figure); well-demarcated desquamated patches on the PRACTICE POINTS weight-bearing aspects of the plantar feet; and a 2.0-cm, • Patients with scurvy often pose a diagnostic dilemma well-demarcated, thinly raised erythematous patch of the because their presenting symptoms can lead physi- inferolateral penile shaft. Oralcopy examination was notable cians down a laborious and costly road of unnec- for multiple discrete areas of ulceration on the lateral essary tests including vasculitic, infectious, and aspects of the tongue. Ophthalmic examination revealed rheumatologic workups. conjunctival injection and photophobia. The ankles were • The diagnosis of scurvy is clinical and typically is edematous and tender (the left ankle more than the based on signs such as perivascular hemorrhage, right), and range of passive motion was limited by pain. bleeding gums, anemia, impaired wound healing, and Laboratory values were remarkable for a hemoglobin ecchymoses in the setting of vitamin C deficiency count of 13.1 g/dL (reference range, 14.2–18 g/dL), erythrocyte with rapid resolution upon vitamin C supplementation. not sedimentation rate of 31 mm/h (reference range, 0–10 mm/h), To the Editor: Do A 28-year-old recently homeless white man with a his- tory of heroin abuse was admitted with a worsening rash and left ankle pain of 1 week’s duration, as well as subjective fever after 3 weeks of a productive cough, sore throat, hoarse voice, and general malaise. Six days prior to presentation, he developed redness and swelling of the A dorsal aspects of both hands with accompanying rash, and 2 days prior to presentation he developed a similar rash on the legs with associated left ankle pain, redness, and swelling. He also reported eye redness, pain, pho- tophobia, crusty eye discharge, and a pins and needles sensation on the soles of both feet. Additionally, he had noted difficulty with urination over several days. He had been homelessCUTIS for less than 1 month prior to admission. On physical examination, the patient appeared to be well nourished. Skin examination was notable for B scattered perifollicular hemorrhagic and hyperkeratotic A and B, Scattered perifollicular hemorrhagic and hyperkeratotic pap- papules ranging in size from 3 to 6 mm with associ- ules with associated alopecia on the thighs. ated nummular alopecia of the bilateral medial thighs Drs. Christopher and Fathi are from and Dr. Menachof was from the University of Colorado, Denver. Dr. Christopher is from the Department of Ophthalmology and Dr. Fathi is from the Department of Dermatology. Dr. Menachof currently is from The Indian Health Service, Rockville, Maryland. The authors report no conflict of interest. Correspondence: Karen L. Christopher, MD, 1675 Aurora Ct, F731, Aurora, CO 80045 ([email protected]). WWW.MDEDGE.COM/DERMATOLOGY VOL. 103 NO. 3 I MARCH 2019 E21 Copyright Cutis 2019. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. SCURVY MASQUERADING AS ARTHRITIS and C-reactive protein level of 5.4 mg/dL (reference requiring 110 to 125 mg daily because of increased oxida- range, 0–0.8 mg/dL). Urinalysis was unremarkable, blood tive stress.6-9 Although access to these foods in the mod- cultures were negative, and a chest radiograph was nor- ern United States is high, as many as 10% of males and mal. Human immunodeficiency virus and rapid plasma 6.9% of females are vitamin C deficient, and in the subset reagin tests were negative, with normal levels of IgG, IgA, of generally healthy middle-class Americans, as many as and IgM. IgE was elevated at 572 IU/mL (reference range, 6% are deficient.8,10 The highest risk groups tend to be 0–100 IU/mL). Ultrasonography of the leg was negative smokers and individuals with low incomes.8 Although for deep vein thrombosis, and a left ankle radiograph vitamin C deficiency does not automatically equate to was negative for fracture. The patient previously was scurvy, early studies on experimentally induced scurvy found to have antinuclear antibodies of 1:40 and nega- in prisoners showed that signs of scurvy may begin tive antineutrophil cytoplasmic antibodies, anti–double- to develop in as few as 29 days of complete vitamin C stranded DNA, anti–Sjögren syndrome antigens A and B, deprivation, with overt scurvy developing after approxi- and cryoglobulins, as well as normal complement levels. mately 40 to 90 days.11,12 The constellation of rash, arthritis, conjunctivitis, and dif- Patients with scurvy often pose a diagnostic dilemma ficulty with urination raised a high suspicion for reactive for physicians because their presenting symptoms, such arthritis; however, the patient was found to be HLA-B27 as fatigue, anemia, and rash, are nonspecific and can lead negative with a negative urine chlamydia test. physicians down a laborious and costly road of unneces- The patient was mildly hypokalemic at 2.9 mmol/L sary tests including vasculitic, infectious, and rheumato- (reference range, 3.5–5.0 mmol/L) and hypoalbuminemic logic workups to determine the cause of the symptoms. at 3.6 g/dL (reference range, 3.9–5.0 g/dL). He had a Increased awareness of thecopy current prevalence of hypovi- slightly elevated international normalized ratio of 1.4 (ref- taminosis C may help to decrease these unnecessary costs erence range, 0.9–1.2). Further questioning revealed that by putting scurvy higher on the differential for patients his diet consisted mostly of soda and energy drinks; his with this spectrum of symptoms. vitamin C level was subsequently checked and found to Scurvy has been called the eternal masquerader be 0 mg/dL (reference range, 0.2–2.0 mg/dL). A diagnosis because its nonspecific signs and symptoms have often of scurvy was made, and his symptoms improved at the led to misdiagnosis.13 Cases of scurvy mimicking dis- hospital while maintaining a diet with normal levels of eases ranging from bone tumors14 to spondyloarthritis15 not16 vitamin C. His rash had markedly improved by hospital and vasculitis have been reported. The typical patient day 2, joint swelling decreased, and the conjunctival injec- at risk for scurvy tends to fall in one of the following tion and eye pain had resolved. Upon outpatient follow- categories: psychiatric illness, gastrointestinal disorders, up, his rash and joint swelling continued to improve, and malnourishment, chronic alcoholism, drug use, elderly he had not experienced any further areas of hair loss. age, infants, restrictive dietary habits or food allergies, Scurvy, a condition caused by vitamin C deficiency,Do is a or those in developing countries.17-20 Our patient did disease of historical importance, as it ravaged ships full of not fit particularly well into any of the aforementioned sailors in days past; however, its incidence has decreased high-risk categories; he had only recently become drastically since Lind1 first described its treatment using homeless and had a history of intravenous drug use but citrus fruits in 1753. Nonetheless, even with modern day had not been using drugs in the months prior to the access to foods rich in vitamin C, scurvy is far more com- development of scurvy. Additionally, his salient symp- mon than expected in the developed world. toms were more consistent with reactive arthritis than Vitamin C (ascorbic acid) plays a crucial role in human with classic scurvy. biochemistry. Although many plants and animals can Although he had many symptoms consistent with synthesize ascorbic acid, humans and other animals such scurvy such as generalized malaise, perifollicular hemor- as guinea pigs lack the required enzyme, making vita- rhage and hyperkeratosis, spongy edema of the joints, min C an essential nutrient required in dietary intake. 2-4 and mild anemia on laboratory testing, he was missing Hypovitaminosis C leads to scurvy when collagen pro- several classic scurvy symptoms. Unlike many patients duction becomes impaired due to lack of ascorbic acid as with scurvy, our patient did not describe any history a required CUTIScofactor for its synthesis, which leads to tissue of bruising easily or dental concerns, and examination and capillary fragility, causing hemorrhage and peri- was notably absent of ecchymoses as well as spongy vascular edema.4 The diagnosis of scurvy is clinical and or bleeding gums. He did, however, present with eye typically is based on signs such as perivascular hemor- irritation and photophobia. These symptoms, consistent rhage, bleeding gums, anemia, impaired wound healing, with keratoconjunctivitis sicca, are lesser known because and ecchymoses in the setting of vitamin C deficiency ocular findings are rarely found in scurvy.21 Patients with (<11 μmol/L or <0.2 mg/dL) with rapid resolution upon scurvy can report eye burning and irritation, redness, vitamin C supplementation.5 blurry vision, and sensitivity to bright light secondary to Important sources of vitamin C include citrus increased dryness of the corneal surfaces. Horrobin et al22 fruits, strawberries, broccoli, spinach, and potatoes. postulated that this symptom may be mediated by regu- Recommended daily intake is 75 to 90 mg, with smokers lation of prostaglandin E1 by vitamin C. E22 I CUTIS® WWW.MDEDGE.COM/DERMATOLOGY Copyright Cutis 2019. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. SCURVY MASQUERADING AS ARTHRITIS Another less common sign of scurvy found in our 8. Schleicher RL, Carroll MD, Ford ES, et al. Serum vitamin C and patient was patchy alopecia. Alopecia most often is seen the prevalence of vitamin C deficiency in the United States: 2003- 11,23 2004 National Health and Nutrition Examination Survey (NHANES). in association with concomitant Sjögren syndrome.