ARTICLE
Auteur(s) : Emek
Kocatürk, Selin Aktas, Mukaddes Kavala, Feride Kocak, Merve
Sürücü, Aytekin Oguz
Göztepe Training and Research Hospital Department
of Dermatology, Nadiraga Sok. 25/9 Goztepe, 34073 Istanbul,
Turkey
Scurvy is considered by many a historical disease associated
with sailors traveling to distant countries. Today, with many food
products available, it is hard to believe it still exists in urban
areas. We report a housewife diagnosed with scurvy.
A 32-year-old woman was admitted to the internal medicine
department because of fatigue, myalgias, arthralgias and ecchymoses
on the legs, present for one month. Her medical history was
unremarkable except for weight loss (10 kilos in three months) and
painful chronic gingivitis, which resulted in a very limited diet,
consisting only of crumpets. The internal medicine department
investigations revealed severe anemia and a hematology consultation
suspected a systemic vasculitis. Prothrombin and partial
thromboplastin levels, hepatitis markers, antineutrophil cytoplasm
antibodies (c-ANCA), antineutrophil perinuclear antibodies
(p-ANCA), antinuclear antibodies (ANA), a comprehensive metabolic
panel including iron, folic acid and B12 vitamin were within normal
levels. The full blood count results were: hemoglobin
5.2 g/dL, hematocrit 16.9%, platelet 185 × 103/mL.
Physical examination revealed ecchymoses and edema on the lower
extremities, especially involving the joints, diffuse
perifollicular petechia with “corkscrew” hairs and hyperplastic
haemorrhagic gingivitis, resulting in tooth loss (figures 1A, B). In
the dermatology department, a punch biopsy was performed which
showed perivascular lymphocyte infiltration, extravasated
erythrocytes and hemosiderin deposition. Serum vitamin C levels
were at an almost undetectable level [< 0.1
(0.5-1.8 mg/dL)]. The diagnosis was scurvy and she was treated
with 1,000 mg/day vitamin C orally, which led to a dramatic
improvement within 2 weeks. She was also referred to a
psychiatrist, but found to be mentally healthy.
Vitamin C is a water-soluble essential vitamin with various
functions in the human body. It is responsible for the
hydroxylation of collagen, the metabolism of tyrosine, the
biosynthesis of carnitine and norepinephrine, and the amidation of
peptide hormones; it also promotes iron absorption by reducing
dietary iron from the ferric to the ferrous form [1]. Patients with
vitamin C deficiency produce an abnormal collagen that affects
blood vessel integrity, leading to capillary fragility,
perivascular edema and red cell extravasations [2].
Vitamin C is present mainly in fruit and vegetables and may
easily be lost during cooking and processing [3]. A daily
intake of 10 mg will maintain the total body vitamin C pool
above 300 mg. Scurvy develops after 60-90 days of a diet free
of vitamin C, when the total body pool is depleted below this value
[4]. At-risk groups include the poor, alcoholics, individuals with
allergies to multiple fruit and vegetables, cancer patients, people
with gastrointestinal disease, anatomical abnormalities,
psychiatric disorders or chronic dental problems [1].
Scurvy may develop with non-specific symptoms like weakness,
fatigue, shortness of breath and aching in the limbs. Skin changes
usually occur in the early phases of the disease and form the basis
for diagnosis. As a consequence of defective collagen formation,
blood vessel fragility manifests as petechiae, purpura and large
ecchymoses [5]. The initial skin change is follicular
hyperkeratosis with “corkscrew hairs”; later perifollicular
haemorrhages occur [6]. Patients with preexisting periodontal
disease have more pronounced complications on the oral mucosa like
gingivitis, bleeding gums and loosening of teeth [1, 5].
Normochromic and normocytic anemia is a common finding, due to
blood loss, concomitant folate deficiency, or decreased iron
absorption in the small bowel [1].
Our case had the typical clinical picture of scurvy (extensive
ecchymoses, follicular petechia, chronic gingivitis) but lacked the
typical history; she was a housewife living in the centre of a big
city; with no psychiatric or chronic illnesses, no drug abuse nor
alcohol intake but only an inadequate diet, due to chronic
gingivitis. This case also implies that dermatology consultations
may be more helpful than unnecessary laboratory work-ups for
patients with skin lesions.
Disclosure
Financial support: none. Conflict of interest: none.
References
1 Olmedo JM, Yiannias JA, Windgassen EB,
Gornet MK. Scurvy: a disease almost forgotten. Int J
Dermatol 2006; 45: 909-13.
2 Velandia B, Centor RM, McConnell V, Shah M. Scurvy is still
present in developed countries. J Gen Intern Med 2008 ; 23:
1281-4.
3 Nobile S, Woodhill JM. Where is vitamin C found? – vitamin C
in foods. In: Vitamin C. Lancaster: MTP press limited, 1981:
31-56.
4 Grandon JH, Lund CC, Dill DB. Experimental
human scurvy. N Engl J Med 1940; 223: 353-69.
5 Nguyen RT, Cowley DM, Muir JB. Scurvy:
A cutaneous clinical diagnosis. Aust J Dermatol 2003; 44:
48-51.
6 McKenna KE, Dawson JF. Scurvy occurring in a
teenager. Clin Exp Dermatol 1993; 18: 75-7.
|