Scurvy is a condition caused by a dietary lack of vitamin C (ascorbic acid), hence is also called hypovitaminosis C, and is characterized by an increased bleeding tendency and impaired collagen synthesis resulting in osteoporosis and impaired wound healing.
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Epidemiology
Scurvy in adults is rare. Young children and older persons are predisposed to scurvy due to their diet or the overheating of food. It does not occur before six months of age because maternal stores are maintained until then. Males and females are equally affected. Patients at risk include elderly males, alcoholics, smokers and those with malabsorptive diseases.
Clinical presentation
Patients may present with lethargy and malaise, bone pain, bleeding diathesis (e.g. bleeding gums, petechiae), and impaired wound healing.
Pathology
Unlike most other animals, humans cannot produce their own vitamin C. In fact, vitamin C is synthesized by almost all vertebrates starting from D-glucose. Specifically, humans lack the enzyme gulonolactone oxidase (GULO), responsible for the transformation of L-gulonactone (D-glucose derivative) into 2-keto-L-gulonelactone. It will be this last metabolite that will finally be converted into L-ascorbate 10.
Lack of dietary vitamin C (ascorbic acid) may be related to inadequate food intake, the destruction of vitamin C in food caused by cooking and canning, or the absence of fresh fruit in the diet.
Vitamin C is essential for collagen synthesis, acting as a coenzyme to producing cross-linking of collagen fibers. Defective collagen cross-linking compromises skin, joint, bone, and vascular integrity.
Radiographic features
Pediatric
cortical thinning: “pencil-point” cortex
-
scorbutic rosary: expansion of the costochondral junctions
may relate to the fracturing of the zone of provisional calcification during normal respiration
similar to the rachitic rosary appearance as seen in rickets
Wimberger ring sign: circular, opaque radiologic shadow surrounding epiphyseal centers of ossification, which may result from bleeding
Frankel line: dense zone of provisional calcification
Trümmerfeld zone: lucent metaphyseal band underlying Frankel line
Pelkan spur: metaphyseal spurs that result in cupping of the metaphysis due to healing of microfractures 9 (note, these do not have the same pathogenesis or imaging appearance as the metaphyseal fractures encountered in non-accidental injury)
Adults
Other significant manifestations in both children and adults arise from the propensity for bleeding, including intra-articular, retrobulbar, and intracranial hemorrhage.
History and etymology
The term scurvy comes from various words used to describe the manifestations of the condition: covered with scabs, diseased, scorbutic.
scheurbuik (Dutch)
scorbut (French)
skybjugr (Old Norse): a swelling (bjugr) from drinking sour milk (skyr) on long sea voyages
Infantile scurvy, historically also known as Barlow disease, is named after Sir Thomas Barlow (1845-1945), Professor of Medicine at University College London 1895-1907 5.
Eugen Fraenkel (1853-1925), a German pathologist, was the first person to be appointed a full Professor of Pathology at the University of Hamburg in 1919 6,8.
Karl Francis Pelkan (1890-1992), an Austrian-American pediatrician described his eponymous spurs in a paper published in 1925 6,7.
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