Scurvy is a disease rarely seen in developed countries where fortified food products and multiple supplements fill the market. However, poor diets devoid of fresh fruits and vegetables and low food variety can still cause this often forgotten disease.
Our patient’s initial diagnostic work-up considered conditions that are more common than scurvy, such as trauma, coagulopathies, vasculitides, and malignancy. We excluded leg trauma and physical abuse by history and a negative x-ray.
Subsequently deep vein thrombosis was ruled out with a normal vein duplex. An extensive work-up was done for dyspnea. Cardiac illness was excluded with a normal EKG, negative cardiac enzymes, and a normal chest x-ray and echocardiogram. Pulmonary embolism was in the differential with an increased D-dimer; the negative Doppler and CT angiograph of the chest excluded it. The history of tobacco use and incidental finding of bullous emphysema on CT, as well as a new-onset anemia, were considered contributory factors of dyspnea. Thrombotic thrombocytopenic purpura, idiopathic thrombocytopenic purpura, and hemolytic uremic syndrome were excluded by a normal platelet count, urinalysis, and basic metabolic panel. We also considered other important possibilities such as abnormal qualitative platelet function; drug hypersensitivity induced purpura, primary vasculitis, secondary vasculitis,21
Von Willebrand disease, vitamin K deficiency, and neoplasia. A similar clinical picture has been observed in patients with these different conditions.
Our patient’s Epi collagen test result was negative, as was his platelet antibodies, which ruled out purpura induced by drug hypersensitivity or neoplasia. A C-ANCA and P-ANCA came back negative, a urinalysis was normal, and a CT scan of the chest showed only bullous emphysema, ruling out primary vasculitis, such as Wegener’s granulomatosis and polyarteritis nodosa. In a similar way, we excluded secondary vasculitis induced by infectious diseases such as hepatitis C or HIV.
We considered vitamin K deficiency secondary to liver disease as our patient had a previous history of alcohol abuse. However, he had normal liver function tests and a normal coagulant profile (prothrombin time and partial thromboplastin time). We also considered the different types of Von Willebrand disease, but his Von Willebrand Ag was normal and his ristocetin cofactor ruled out a qualitative abnormal VIII factor.
Neoplastic disease can also present in a similar way. We considered diseases like leukemias, lymphomas, multiple myeloma, myelodysplasia, and disseminated malignancy. Our patient had a normal white blood cell count, normal peripheral smear, a normal protein electrophoresis, and a CT of the chest; abdomen, and pelvis were negative for malignancy.
As we could not find any common cause for his petechiae and hematoma, we reconsidered his presentation. We obtained a detailed dietary history in our patient that revealed poor dietary habits with lack of fresh fruit and vegetables, based on canned or overcooked food. He did eat what sounded like a daily intake of meat, so we did not check a prealbumin. His poor intake of vitamin C containing foods led us to consider scurvy in our differential diagnosis and consider less common diseases like senile purpura secondary to zinc deficiency.
Zinc deficiency can contribute to senile purpura, acrodermatitis enteropathica, cognitive decline, depression and alopecia.11,16,20
Our patient had purpura, male pattern baldness, and was initially irritable with a flat affect. We ordered a zinc level that came back low (0.62 mcg/mL) but not low enough to be significant. Alkaline phosphatase level was 62 IU, in the lower range of normal. It is interesting to note that after several months of treatment with multivitamins, his alkaline phosphatase went up to 92 u/L. Serum alkaline phosphatase is low in severe zinc deficiency and returns to normal after treatment.22,23
We did not order zinc levels in neutrophils and lymphocytes.
Our patient’s vitamin C was very low and his skin biopsy showed classic perifollicular hemorrhages that confirmed the diagnosis of scurvy.13
He also had the classic corkscrew hairs noted on biopsy. The biopsy excluded the possibility of leukocytoclastic vasculitis (small vessel vasculitis) triggered by Hepatitis C, cryoglobulinemias, or neoplasias.
Vitamin C participates in the transformation of lysine proline on hydroxylproline that is part of collagen synthesis. Patients with deficient Vitamin C produce an abnormal collagen that affects blood vessel integrity, leading to capillary fragility, perivascular edema, and red cell extravasations.7,14
Classical signs of vitamin C deficiency include weakness, anemia, tooth loss, gum bleeding, bruises, and petechiae.
Our patient had a significant anemia, which was not present at his earlier visits to the emergency department 2 weeks back. Reticulocyte count was mildly increased at 3.5%. This, along with a normal MCV, essentially ruled out any severe deficiency of nutrients required for the development of red blood cells, such as iron or vitamin B12
. Serum lactate dehydrogenase (LDH) and haptoglobin levels were normal and the patient’s anemia improved with vitamin C supplementation. Anemia is a frequent sign of scurvy secondary to blood loss and decreased iron absorption in the small bowel.
Decrease of the osteoid matrix formation and bone mineralization has been reported in children with scurvy and noted in our patient as evidenced by several bony infarcts and sclerotic lesions described on the tibia x-ray.23
Hemorrhagic manifestations and poor wound healing are evident symptoms after 3 to 6 months of decreased dietary intakes of vitamin C.9
Humans are unable to synthesize vitamin C and thus depend on external sources to fulfill these requirements.8
The usual sources of vitamin C include raw fresh fruits and vegetables. Boiling, cooking, and canning fruits and vegetables lower the vitamin C content by 33%.25,26
Many adults consume insufficient vitamin C. Eighty-five percent of the institutionalized elderly population is undernourished and 50% have vitamin and mineral intake that is less than the recommended dietary allowance.7,8
The most common population at risk for scurvy is an adult or older person living alone, edentulous or toothless, with poor self-care, lack of social support, having destructive behaviors such as alcoholism, drug abuse or smoking, and those adults who are mentally or chronically ill. They share a common denominator, a lack of food variety with chronic deprivation of vitamin C and other nutrients. Smokers require higher doses of vitamin C (110–125 mg/day) compared with normal population’s requirement of 60–90 mg/day because of increased demand as a result of increased oxidative stress.6
Some researchers feel that the elderly require more vitamin C for the same reason.6,17,18,24
The micronutrient and macronutrient deficiencies in the geriatric population cause new cases of scurvy, pellagra, and Zn deficiency.8,14,16
This patient showed classical symptoms of bleeding caused by vitamin C deficiency: petechiae, hematomas, weakness, anemia, as well as radiologic bone changes, giving a typical picture of scurvy.
We generally consider scurvy an uncommon disease in developed nations, but it is still present. As scurvy presents with clinical features similar to many other common conditions, we must remember it and consider it as part of an expanded differential diagnosis. We strongly recommend having a high index of suspicion for this disease in the specific populations at risk, described as the socially isolated adult or elderly patient with a history of destructive behavior, living alone, and mentally or chronically ill.