Scurvy Clinical Presentation
- Author: Lynne Goebel, MD; Chief Editor: George T Griffing, MD more...
Symptoms and signs of scurvy may be remembered by the 4 Hs: hemorrhage, hyperkeratosis, hypochondriasis, and hematologic abnormalities.
The initial symptoms of scurvy are nonspecific and include the following:
Loss of appetite
Poor weight gain
After 1-3 months of severe or total vitamin C deficiency, patients develop shortness of breath and bone pain. Myalgias may occur because of reduced carnitine production. Skin changes with roughness, easy bruising and petechiae, gum disease, loosening of teeth, poor wound healing, and emotional changes occur. Dry mouth and dry eyes similar to Sjögren syndrome may occur.
Other symptoms include the following:
Pain and tenderness of the legs
Swelling over the long bones
In the late stages, jaundice, generalized edema, oliguria, neuropathy, fever, and convulsions can be seen. Left untreated scurvy progresses, with potentially fatal complications, including cerebral hemorrhage or hemopericardium.
Infantile scurvy is uncommon before age 7 months, and clinical and radiographic manifestations rarely occur in infants younger than 3 months. Early clinical manifestations consist of pallor, irritability, and poor weight gain.
In advanced infantile scurvy, the major clinical manifestation is extreme pain and tenderness of the arms and, particularly, the legs. The baby is miserable and tends to remain in a characteristic immobilized posture from subperiosteal pain, with semiflexion of the hips and the knees ("frog leg posture"), as described by Thomas Barlow in 1884.
The body is both wasted and edematous, and petechiae and ecchymoses are commonly present. Hyperkeratosis, corkscrew hair, and sicca syndrome are typically observed in adult scurvy but rarely occur in infantile scurvy. The case of an infant with diffuse, nonscarring alopecia of the scalp and radiologic features of scurvy was reported in India in 2008. . Three cases of scurvy presenting as difficulty with walking have been reported in the US, with only 1 out of 3 patients having classic gingival lesions at presentation.
Hypotension may be observed late in the disease. This may be due to an inability of the resistance vessels to constrict in response to adrenergic stimuli. Heart complications include cardiac enlargement, electrocardiographic (ECG) changes (reversible ST-segment and T-wave changes), hemopericardium, and sudden death. Bleeding into the myocardium and pericardial space has been reported. High-output heart failure due to anemia can be observed.
Two case reports of pulmonary hypertension in patients with vitamin C deficiency have been described, with complete reversal after vitamin C replacement.[25, 26] .
Anemia develops in 75% of patients, resulting from blood loss into tissue, coexistent dietary deficiencies (folate deficiency), altered absorption and metabolism of iron and folate, gastrointestinal blood loss, and intravascular hemolysis. The anemia is most often characterized as normochromic and normocytic.
Ocular features include those of Sjögren syndrome, subconjunctival hemorrhage, and bleeding within the optic nerve sheath. Scleral icterus (late, probably secondary to hemolysis); and pale conjunctiva are seen. Funduscopic changes include cotton flame-shaped hemorrhages, and cotton-wool spots may be seen. Bleeding into the periorbital area, eyelids, and retrobulbar space also can be seen. Proptosis of the eyeball secondary to orbital hemorrhage is a sign of scurvy.
Integumentary and skeletal system
Perifollicular hyperkeratotic papules, perifollicular hemorrhages, purpura, and ecchymoses are seen most commonly on the legs and buttocks where hydrostatic pressure is the greatest. The central hairs are twisted like corkscrews, and they may become fragmented. Poor wound healing and breakdown of old scars may be seen. Capillary fragility can be checked by inflating a blood pressure cuff and looking for petechiae on the forearm. In the nails, splinter hemorrhages may occur.
Alopecia may occur secondary to reduced disulfide bonding.
In advanced cases, clinically detectable beading may be present at the costochondral junctions of the ribs. This finding is known as the scorbutic rosary (ie, sternum sinks inward) and may occur in children. The scorbutic rosary is distinguished from rickety rosary (which is knobby and nodular) by being more angular and having a step-off at the costochondral junction. Fractures, dislocations, and tenderness of bones are common in children.
Bleeding into the joints causes exquisitely painful hemarthroses. Subperiosteal hemorrhage may be palpable, especially along the distal portions of the femurs and the proximal parts of the tibias of infants. Bleeding into the femoral sheaths may cause femoral neuropathies, and bleeding into the muscles of the arms and the legs may cause woody edema.
A case of a 6-year-old boy with feeding difficulties and a monoarticular lesion of the distal femur mimicking a bone tumor was reported in India. After full assessment and investigation, he was found to have scurvy with significant improvement following vitamin C replacement.
Gum hemorrhage occurs only if teeth have erupted and usually involve the tissue around the upper incisors. The gums have a bluish-purple hue and feel spongy. Gum swelling, friability, bleeding, and infection with loose teeth also occur, as do mucosal petechiae.
Loss of weight secondary to anorexia is common. Upper endoscopy may show submucosal hemorrhage. Rarely, hematuria, hematochezia, and melena are noted.
In both animal and human studies, vitamin C deficiency has been linked to possible pathogenesis of nonalcoholic fatty liver disease, given its anti-oxidant properties and inverse correlation with BMI.
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