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Vol. 20. Issue 5.
Pages 281-285 (May 2024)
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Vol. 20. Issue 5.
Pages 281-285 (May 2024)
Case Report
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Scurvy. A forgotten pseudovasculitis
Escorbuto. Una pseudovasculitis olvidada
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Noelia Cabaleiro-Rañaa,
Corresponding author
noeliamedicalcr@gmail.com

Corresponding author.
, Diego Santos-Álvareza, Lucía Romar de las Herasa, Carmen Álvarez-Regueraa, Evelin Cecilia Cervantes Péreza, Rosa María Hernández Cancelab, Susana Romero-Yustea
a Servicio de Reumatología, Complexo Hospitalario Universitario de Pontevedra, Pontevedra, Spain
b Servicio de Anatomía Patológica, Complexo Hospitalario Universitario de Pontevedra, Pontevedra, Spain
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Table 1. Laboratory test results.
Table 2. Review of scurvy cases in the 21st century.
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Abstract

Scurvy is a nutritional disease caused by ascorbic acid (vitamin C) deficiency. Althought currently it is a rare disease, we should considerer it in the differential diagnosis of purpura and arthritis in patients with restrictive diets.

We present the case of a 49-year-old man with a history of a nutritional disorder presented to our hospital with generalized purpura and hemarthros. Following the anamnesis and laboratory findings, rheumatological, infectious and hematological etiologies were excluded. Finally, the diagnosis of scurvy was made upon demostration poor levels of vitamin C and a spectacular response to nutritional supplements. We compare this case with 19 similar cases reported in the medical literature.

Keywords:
Purpura
Arthritis
Scurvy
Vitamin C
Restrictive diet
Resumen

El escorbuto es una enfermedad nutricional causada por el déficit de ácido ascórbico (vitamina C). Aunque actualmente es una enfermedad rara, debe tenerse en cuenta en el diagnóstico diferencial ante púrpura y artritis en pacientes con dietas restrictivas.

Presentamos el caso de un varón de 49 años con antecedentes de trastorno de la conducta alimentaria que presenta púrpura generalizada y hemartros. Tras la anamnesis y los hallazgos analíticos, se excluyeron enfermedades de etiología reumatológica, infecciosa y hematológica. Finalmente, se confirmó el diagnóstico de escorbuto tras objetivar niveles deficientes de vitamina C y una espectacular respuesta a los suplementos nutricionales. Comparamos nuestro caso con otros 19 similares, reportados en la literatura médica.

Palabras clave:
Púrpura
Artritis
Escorbuto
Vitamina C
Dieta restrictiva
Full Text
Introduction

Scurvy is a nutritional disease caused by vitamin C deficiency that was epidemic on sailing voyages from the 15th to the 18th centuries and is now rare in developed countries.1

Vitamin C is a water-soluble vitamin that acts as a reducing agent and is necessary for collagen synthesis. Humans rely on diet and foods high in vitamin C include tomatoes, potatoes, and citrus fruits. The recommended dose is 90 mg/day for men and 75 mg/day for women.2 When vitamin C levels fall to less than 0.15 mg/dL, the typical symptoms of scurvy can develop, characterised by asthenia, joint pain, and vascular fragility, which can lead to petechiae, bleeding gums, haematomas, or hemarthrosis.3,4

Case report

A 49-year-old male with a history of an eating disorder during adolescence was referred to rheumatology for purpura in his lower extremities and bilateral knee arthritis of one month’s duration. During the anamnesis, the patient reported asthenia and episodes of epistaxis. In recent years, his diet had consisted primarily of dairy products, biscuits, and cereals.

On examination, he appeared cachectic, pale, bradypsychic, oedema with pitting of the lower limbs, and purpuric lesions on his extremities and abdomen. Furthermore, he exhibited arthritis in both knees, for which arthrocentesis was performed and haematic fluid was obtained. In addition, dermatology confirmed perifollicular haemorrhage by dermoscopy and biopsied one of the purpuric lesions (Fig. 1A), the pathology examination of which revealed chronic superficial dermatitis and perifollicular extravasation of blood without vasculitis (Fig. 1C).

Fig. 1.

A. Lower extremities at the time of diagnosis. B. Lower extremities following treatment. C. Pathology study of the purpuric lesion biopsied.

(0.13MB).

Laboratory tests identified normocytic anaemia with associated iron deficiency and indirect evidence of malnutrition. The autoimmunity tests, coagulation study, serology, and radiographic studies were normal and the joint fluid culture was negative (Table 1).

Table 1.

Laboratory test results.

Laboratory results at the time of diagnosis
Parameter  Value 
Serology: Clostridium tetani, HAV, HBV, HCV, HIV, rubeola, varicella zoster, parvovirus B19, paramyxovirus, measles virus, Quantiferon, Rickettsia coronii, Treponema pallidum  Negative 
Autoimmunity: ANA (ELISA), ANCA-antimyeloperoxidase, ANCA-antiproteinase 3, anti-CCP, anti-cardiolipin antibodies, anti-streptolysin O, anti-β2-glycoprotein antibodies, cryoglobulins  Negative 
C3 and C4 complements  138 mg/dL (VR 88–201 mg/dL), 
  22 mg/dL (VR 10–40 mg/dL) 
Tumour markers: alpha-fetoprotein, carcinoembryonic antigen, CA 19.9, PSA, squamous cell carcinoma-associated antigen  Negative 
PCR  1.3 mg/dL (VR 0–0.5 mg/dL) 
ESR  26 mm/h (VR 0–10 mm/h) 
Haemoglobin, MCV  8.7 g/dL (VR 13.5–17.5 g/dL), 89.3 fL (VR 80–98 fL) 
Peripheral blood smear  No significant morphological alterations 
Direct Coombs  Negative 
Haemostasis: INR, fibrinogen, prothrombin time, activated partial thromboplastin time, factor II, factor V, factor VII, factor IV, factor X, factor XI, factor XII  Within range 
Factor VIII  200.8% (VR 50%–150%) 
ADP aggregation  64 s (VR 68–121 s) 
Epinephrine aggregation  73 s (VR 84–160 s) 
Folic acid  4.4 ng/mL (VR 4–20 ng/mL) 
Vitamin B12  337 pg/mL (VR 180–414 pg/mL) 
Vitamin K1  <0,05 µg/L (VR 0.13–1.5 µg/L) 
Vitamin A  0.04 mg/L (VR 0.3–1 mg/L) 
Vitamin C  <0.10 mg/dL (VR 0.4–2 mg/dL) 
Vitamin D (D2 + D3)  20 ng/mL (VR 10–30 ng/mL: deficiency) 
Albumin, prealbumin  3.4 g/dL (VR 3.5–5.2), 11 mg/dL (VR 18–45 mg/dL) 
Faecal occult blood  <30 (VR < 75: negative) 
Laboratory results of the articular fluid of the left knee
Parameter  Value 
Red blood cells  550,706 mm3 
Nucleated cells  428 mm3 
Polynuclear  27% 
Mononuclear  73% 
Glucose  80 mg/dL 
Crystals  No crystals observed 
Appearance  Haemorrhagic 

HAV, HBV, HCV: hepatitis A, B, and C virus; HIV: human immunodeficiency virus; ANA: antinuclear antibodies; ELISA: enzyme-linked immunosorbent assay; ANCA: antineutrophil cytoplasmic antibodies, Anti-CCP: anti-cyclic citrullinated peptide; CRP: C reactive protein, ESR = erythrocyte sedimentation rate; MCV: mean corpuscular volume.

In view of the high suspicion of scurvy, intravenous vitamin C was administered, and suppressed levels of vitamins C, A, K1, and zinc were confirmed in the following days (Table 1).

After two weeks of treatment, the patient improved clinically and analytically and was therefore discharged with vitamin complexes and remains asymptomatic at present (Fig. 1B).

Discussion

While scurvy is rare in developed countries, cases are still reported among individuals at risk for malnutrition. Vitamin C deficiency alters vascular collagen and leads to bruising, purpura, arthritis, or arthromyalgia that may mimic vasculitis.

A review of the literature revealed 19 cases of scurvy in adults published in the 21st century (Table 2). Of these, 63% were male and had a mean age of 50 years. In almost all cases, the person had some medical or psychiatric history, and in one case, vitamin C deficiency was reported as a complication of enteral nutrition.17 The most frequent clinical manifestations were dermatological, including haematomas or ecchymosis, rash with perifollicular distribution, gingival bleeding, and the characteristic “corkscrew” hairs. Approximately 60% of all cases presented with articular manifestations, the most commonly observed ones being hemarthrosis in the large joints of the lower limbs (mainly knees and ankles) and polyarthralgias. In the cardiorespiratory system, several patients had dyspnoea on minimal exertion secondary to anaemia and/or heart failure.3,11 Other less frequent symptoms were pulmonary hypertension,11 arterial hypotension, and presyncopal episodes that might be attributable to the greater resistance of the blood vessels to the action of adrenaline.18,20 As an atypical manifestation of vitamin C deficiency in connective tissue, Francescone and Levitt reported a case of a 59-year-old male with osteopenia of the sacrum.

Table 2.

Review of scurvy cases in the 21st century.

Author, year  Cases  Country  Sex  Age  History  Symptoms  Biopsy  Vitamin C treatment 
Martínez et al.6 (2004)1SpainF90Dependent for BADLHaematomas  NoID: 1000 mg/24 h (per os)
Perifollicular rash 
Bleeding of the gums 
Francescone and Levitt7 (2005)1USAF59Low socioeconomic levelHematomas  YesID: 1000 mg/12 h (per os)
Perifollicular rash 
Bleeding of the gums 
Corkscrew hairs 
Polyarthralgia 
Osteopenia sacrum 
Roé et al.8 (2005)1SpainF45Machado–Joseph diseaseEcchymosis  YesID: 500 mg/12 h (per os
Perifollicular rash  MD: 500 mg/week (per os)
Bleeding of the gums 
Corkscrew hairs 
Polyarthralgia 
Olmedo et al.9 (2006)1USAF77Food allergiesHematomas  NoID: 100 mg/8 h (per os
Perifollicular rash  MD: 100 mg/day (per os)
Bleeding of the gums 
Léger10 (2008)  1CanadaF47AlcoholismHematomas  YesNE
Léger10 (2008)Perifollicular rash 
Bleeding of the gums 
Corkscrew hairs 
Mertens and Gertner11 (2011)3USAF26Low socioeconomic levelEcchymosis  YesID: 1000 mg/24 h
Perifollicular rash 
Corkscrew hairs 
Knee hemarthrosis 
General syndrome 
F22CPEcchymosis  NoID: 1000 mg/24 h
Perifollicular rash 
Ankle hemarthrosis 
F74Deliriums about food sensitivitiesPerifollicular rash  NoID: 1000 mg/24 h
Bleeding of the gums 
Arthritic ankles 
Dyspnoea (PHTN) 
Núñez Fernández et al.12 (2001)1SpainF67AlcoholismHematomas  NoNE
Perifollicular rash 
Bleeding of the gums 
Abou Ziki et al.13 (2015)1USAFMANEHematomas  NoNE
Perifollicular rash 
Bleeding of the gums 
Corkscrew hairs 
Hemarthrosis 
Mintsoulis et al.3 (2016)1CanadaF68Food allergiesPerifollicular rash  YesID: 100 mg/12 h (iv) 
Hemarthrosis – ankle  MD: 250 mg/day (per os)
Bleeding of the gums 
Epistaxis 
Panniculitis 
Loureiro-Amigo et al.14 (2016)1SpainF28DepressionEcchymosis  NoID: 1000 mg/24 h (per os)
Perifollicular rash 
Bleeding of the gums 
Brandy-García et al.15 (2017)1SpainF42SchizophreniaPerifollicular rash  YesNE
Gingivitis 
Knee hemarthrosis 
Regehr et al.16 (2021)1USAF18CPHematoma  NoID: 1000 mg/24 h (iv) MD: 250 mg/24 h (per os)
Perifollicular rash 
Corkscrew hairs 
Bleeding of the gums 
Lanes Iglesias et al.17 (2020)1SpainF55DementiaHematomas  NoEnteral nutrition
Perifollicular rash 
Bleeding of the gums 
Thomas and Burtson18 (2021)1USAF69DepressionEcchymosis  NoMultivitamin complex
Bleeding of the gums 
Arterial hypertension 
General syndrome 
Rodríguez Falabella et al.19 (2023)1ArgentinaF48Parkinson’s diseaseHematomas  YesID: 500 mg/12 h (per os)
Perifollicular rash 
Bleeding of the gums 
Corkscrew hairs 
Oligoarthritic ankles 
Pope and Elder20 (2023)1USAF55AlcoholismHematomas  NoID: 250 mg/24 h (per os)
Perifollicular rash 
Arthritic knee 
Arterial hypertension 
Lu et al.21 (2023)1ChinaF25NSHematomas  No  ID: 200 mg/8 h (per os)
Oligoarthritis   
Cabaleiro Raña et al. (2024)1SpainF49EDPerifollicular rash  YesID: 500 mg/24 h (iv) 
Knee hemarthrosis  MD: multivitamin complex 

BADL: basic activities of daily living; ID: initial dose; MD: maintenance dose; PHTN: pulmonary hypertension; iv: intravenous; F: female; MA: median age; NS: not specified; CP: cerebral palsy; ED: eating disorder; M: male; per os: by mouth.

In our case, the history of an eating disorder, hemarthrosis, and desmoscopic findings enabled us to guide the diagnosis. Vitamin C levels are not routinely tested for in laboratory analyses and waiting for the result would delay diagnosis/treatment.

Differential diagnosis is made with purpura-related conditions (vasculitis, infections, idiopathic thrombocytopenic purpura, Ehlers–Danlos) or bleeding (coagulation disorders). Given that it is a pseudovasculitis, a biopsy is useful to rule out vascular inflammation.3

As regards treatment, the asthenia and skin lesions improved with vitamin C administration, whereas it took the arthritis several weeks to resolve. The anaemia disappeared after one month.5

Conclusion

Despite being an extremely rare deficiency disease in Western countries nowadays, scurvy continues to present in isolation in certain patients. The present case is a reminder of the importance of including this pseudovasculitis in the differential diagnosis of any patient with purpuric lesions or hemarthrosis.

Conflict of interests

The authors have no conflict of interests to declare.

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