Severe Scurvy: an Underestimated Disease

CLINICAL CASE REPORT Severe scurvy: an underestimated disease

M Levavasseur1, C Becquart1, E Pape1, M Pigeyre2, J Rousseaux2,3, D Staumont-Sallé1 and E Delaporte1

Scurvy is one of the oldest diseases in human history. Nowadays, although scurvy tends to become a forgotten disease in developed country, rare cases still occur, especially in people undergoing extreme diet, old people or children with poor diet and patients with malabsorption. We describe three cases of scurvy. The first case is a patient diagnosed with Crohn’s disease, the second one is in a context of anorexia nervosa and drug addiction, and the third case is in a context of social isolation. Early recognition of scurvy can be difficult because symptoms may appear nonspecific and can mimic more common conditions. In any patient with spontaneous hematoma and purpura, in the context of nutritional disorder, scurvy should be systematically considered. As this disease can lead to severe complications, such as bone pain, heart failure or gastrointestinal symptoms, nothing should delay vitamin C supplementation, which is a simple and rapidly effective treatment.

European Journal of Clinical Nutrition (2015) 69, 1076–1077; doi:10.1038/ejcn.2015.99; published online 17 June 2015

INTRODUCTION Clinical examination revealed purpura and perifollicular hyper- Scurvy is one of the oldest diseases in human history. The effects keratosis, hair dystrophy with corkscrew hair (Figure 1). Sponta- of a deﬁciency in vitamin C are well known and became a serious neous hematomas were also observed on the legs. The initial issue among mariners in the 15th and 16th century with diagnosis was scurvy. devastating consequences on long marine expeditions.1 The patient refused diagnostic investigations that led to a Nowadays, scurvy has become a forgotten disease in developed therapeutic test with 1 g of oral vitamin C daily for 1 month. Skin countries, yet cases still occur. lesions gradually resolved, conﬁrming the diagnosis of scurvy.

CASE REPORT Case 3 A 41-year-old man was hospitalized for confusion, in a context of Case 1 social isolation. We learned from his family that he was divorced, A 51-year-old woman with Crohn’s disease (since 1983) was unemployed and had a long history of alcohol intoxication and hospitalized in a nutrition unit. She had several ileal resections in major depressive syndrome with suicide attempt. He was not on her past leading to short bowel disease with need for a parenteral any medication. nutrition. Dermatological examination revealed purpura, perifollicular The patient was hospitalized in a nutrition unit. She was hyperkeratosis, corkscrew hair, hematomas on the legs and apyretic and asthenic. The clinical examination revealed perifolli- hemorrhagic gingivitis with tooth loss (Figure 2). Neurological cular purpura on the legs, with hematomas and corkscrew hair. findings included a polyneuropathy, confusion, disorientation with She was suffering from bone pain on the right knee. confabulation, false recognition and anosognosia. The rest of the Results of primary investigations revealed normal hemostasis, clinical examination was normal. − 1 normocytic normochromic anemia (hemoglobin: 9.6 g dl ). Result of primary investigations revealed a normocytic normo- Platelets, electrolytes, kidney function, vitamin B12, B9, albumin chromic anemia (hemoglobin: 7.4 g dl − 1) secondary to an iron and and iron status were in normal range. folate deficiency (folate: 3 ng ml − 1). Serum ascorbic acid was A dermatologic examination was solicited and scurvy was decreased (o3 μmol l − 1). diagnosed. Despite vitamin C supplementation in parenteral A diagnosis of scurvy was made and the patient was treated nutrition, ascorbic acid serum level was significantly decreased with enteral ascorbic acid at a dose of 1 g per day for 1 month, − 1 (o10 μmol l ). The patient was given enteral ascorbic acid at a associated with multivitamin supplementation, allowing a slow dose of 250 mg per day instead of 125 mg provided by the improvement of skin lesions. We noticed a significant improve- parenteral nutrition. The patient’s condition rapidly improved and ment of the polyneuropathy but a remanent confusion due to a the skin symptoms disappeared in 1 month. Korsakoff’s syndrome persisted.

Case 2 A 38-year-old woman was hospitalized for purpura and spontaneous DISCUSSION hematomas on the lower extremities. She had a history of drug Scurvy is a rare disease in developed countries and is conﬁned to addiction, chronic hepatitis C, iron deﬁciency and eating disorders at-risk patient groups: precarious situations (social isolation, including anorexia nervosa. She was not on any medications. unemployement and homeless), elderly persons, individuals with

1Department of Dermatology, Hospital Claude-Huriez, University Hospital of Lille, Lille, France; 2Department of Nutrition, University Hospital of Lille, Lille, France and 3Celiobe, Private Hospital La Louvière, Générale de Santé, Lille, France. Correspondence: Levavasseur M, Department of Dermatology, Hospital Claude-Huriez, University Hospital of Lille, 59037 Lille, France. E-mail: [email protected] Received 17 February 2015; revised 28 April 2015; accepted 11 May 2015; published online 17 June 2015 Scurvy: an underestimated disease M Levavasseur et al 1077 reduced production of carnitine. One to three months of inadequate intake can lead to more specific symptoms.5 Skin symptoms are characterized by follicular purpura with hyperkeratosis, corkscrew hair, spontaneous hematomas, subungual hemorrhage and petechiae that can lead to ecchymoses in 80% of the patients. Edema of the legs may occur. Oral manifestations are common and include a hyperplastic ulcerative gingivitis, rapidly progressive chronic periodontitis and widespread mucosal hemorrhages. In more advanced cases, patients may present bone pain (due to hemarthrosis or subperiosteal hemorrhage), gastrointestinal symptoms and heart failure (by hemopericardium). Ophthalmic manifestations include conjunctival hemorrhage, flame-shaped hemorrhage and cotton-wool spots at the ocular fundus. Laboratory investigations are not necessary for the diagnosis. The concentration of ascorbic acid may be checked, but this value Figure 1. Perifollicular purpura and corkscrew hair on the legs. tends to reflect recent dietary intake rather than actual storage of vitamin C. A serum level below 2 mg l − 1 suggests scurvy. Anemia occurs in 75% of patients because of blood loss and concomitant folate and iron deficiency. The treatment for scurvy consists of vitamin C intake: 300 mg to 1 g per day orally for an adult for 1 month.6 Subjective improvement in fatigue, pain and anorexia typically occurs rapidly within 24 h. Joint swelling resolves in days, the ecchymoses in 1 or 2 weeks. By 4 weeks, the corkscrew hair regain normal growth. Complete recovery occurs after about 3 months.2

CONCLUSION Early recognition of scurvy can be difficult because symptoms may appear nonspecific and can mimic other conditions. In any patient Figure 2. Upper hyperplastic hemorrhagic gingivitis with necrotic with spontaneous hematoma and purpura, especially in the interdental papillae. context of nutritional disorder or inflammatory bowel disease, scurvy should be systematically considered. As this disease can purported allergies to multiple fruits and vegetables products, lead to severe complications, nothing should delay vitamin C patients with psychiatric disorders or excessive alcohol consump- supplementation, which is a simple and rapidly effective tion as alcohol decreased the absorption of vitamin C. Other at-risk treatment. groups include persons with gastrointestinal diseases, patients with a past history of bariatric surgery, cancer patients on chemotherapy who suffer from nausea, diarrhea and patients on CONFLICT OF INTEREST 2 hemodialysis. The authors declare no conflict of interest. Humans do not have the ability to synthesize vitamin C and obtain 90% of their intake from fruits and vegetables. The total body pool of vitamin C is 1500 mg, and clinical manifestations of REFERENCES scurvy occur when this pool is reduced to o350 mg. To reach 1 Magiorkinis E, Beloukas A, Diamantis A, Scurvy. Past, present and future. Eur J Int such a low level, vitamin C must be completely eliminated from Med 2011; 22: 147–152. the diet for 60–90 days.3 Clinical manifestations of scurvy can be 2 Olmedo JM, Yiannias JA, Windgassen EB, Gornet MK. Scurvy: a disease almost seen within 8–12 weeks of irregular or inadequate intake. The forgotten. Int J Dermatol 2006; 45:909–913. recommended requirement of vitamin C is 75 mg per day for 3 Hodges RE, Hood J, Canham JE, Sauberlich HE, Baker EM. Clinical manifestations of 4 ascorbic acid deficiency in man. Am J Clin Nutr 1971; 24:432–443. women and 90 mg per day for men. fi Can Fam Physician The diagnosis of scurvy is based on clinical features, dietary 4 Léger D. Scurvy: reemergence of nutritional de ciencies. 2008; 54: 1403–1406. history and rapid resolution of symptoms after vitamin C 5 Hansen E, Metzsche C, Henningsen E, Toft P. Severe scurvy after gastric bypass supplementation. Patients with scurvy exhibit various systemic surgery and a poor postoperative diet. J Clin Med Res 2012; 4: 135–137. manifestations. Early stages are often characterized by weakness, 6 Khonsari H, Grandière-Perez L, Caumes E. Le scorbut n’a pas disparu: histoire d’une asthenia, sluggishness, emaciation and myalgias because of the maladie réémergente. Rev Med Interne 2005; 26: 885–890.