SCURVY P.W.Preston, S.D.Orpin & I.Zaki Department of Dermatology, Birmingham Heartlands and Solihull NHS Trust, West Midlands, United Kingdom Introduction What’s in the name A disease of the intrepid In a modern-day era of high-tech diagnostic investigations, complex disease classifications and a multitude of therapeutic interventions, scurvy remains a wonderfully simple disease to identify, understand and treat. We aim to enthuse the reader about the history of this most interesting disease. The word scurvy first appeared in print in Richard Hakluyt’s Principall navigations, voyages and discoveries of the English nation, published in 1589. The word is thought to derive from an attempt to render into English the French scorbut, which comes from the medieval Latin scorbutus. This word probably originated from the Middle Low German scorbuk coming in turn from schoren – a break; and buk – the belly; hence scurvy referred to a disease which ruptures or lacerates the belly. Indeed Anson described “a most extraordinary occurrence” whereby “the scars of old wounds healed for many years were forced open again”. Poor wound healing and scar dehiscence are further features of the disease resulting from defective collagen synthesis. Following its’ eradication at sea most cases of scurvy during the 19th and 20th centuries have occurred on terra firma. Soldiers serving in the Crimean War and World War I were afflicted just as sailors had been in the years before them. Rations often took several weeks to reach the front line and fresh fruit and vegetables would perish in this time. Scurvy – the disease Scurvy results from dietary deficiency of ascorbic acid better known as vitamin C. Man relies on a good dietary intake of the vitamin which is found in potatoes, berries, green vegetables and citrus fruit. It is an essential co-factor in the formation of mature collagen and when deficient particularly affects blood vessel integrity leading to the haemorrhagic manifestations of the disease. It also plays a role in the disulphide cross-linking of hair keratins. As a result, the classic clinical findings of perifollicular purpura and corkscrew hairs are well known to the dermatologist. The advent of evidence-based medicine James Lind, a Scottish physician serving in the Royal Navy was not the first to treat scurvy with citrus fruit, but his fame now endures because he conducted what is considered the first-ever clinical trial in medical and scientific history. It took place in 1747 on board H.M.S. Salisbury, a 4th rate ship of war during the War of Austrian Succession. Turkish soldiers being carried home from Balaclava 1855, many suffering from scurvy. Whaling fleets and polar explorers also suffered at the hands of the disease. Seal and polar bear meat consumed in large amounts contained reasonable amounts of vitamin C, but the liver and adrenal glands proved the best remedy for scurvy. One such whaler, having culled a polar bear noted: “that upon eating her liver, our very skins peeled off: for my own part, I being sick before, though I lost my skin, yet recovered I my health upon it”. This no doubt refers to the desquamation of skin that accompanies recovery from the disease. Lemon and subsequently lime juice remained regular sea-faring rations but were not always issued for polar sledging expeditions as they froze solid and could not be thawed in the extreme conditions. H.M.S. Salisbury Typical clinical findings of perifollicular purpura and corkscrew hairs in a contempory case of scurvy. Missing at sea Advances in ship design and navigation in the late 15 century onwards permitted pioneering extended voyages out of sight of land in the pursuit of territory and trade. The lack of fresh produce on board made scurvy an inevitable consequence of these voyages at sea and ravaged the crews of the early great explorers. th The ship had been at sea for 8 weeks and was patrolling the Bay of Biscay when about 35 of the crew showed features of scurvy. He selected 12 of them who were “as similar as I could have them”, and arranged for them to receive the same basic diet and to be accommodated in the same part of the ship. He then divided the men into groups of 2 and undertook a 6-armed comparative trial of some of the many treatments for scurvy then in use. These included cider, elixir of vitriol (sulphuric acid and aromatics), vinegar, sea-water, oranges and lemons and a purgative mixture. There was no control or placebo group. Within 10 days, the 2 men assigned to citrus fruit recovered. One returned to duty, the second helped nurse the others who remained ill. Capodimonte figurine of James Lind, presented to the Institute of Naval Medicine by Surgeon Vice Admiral Sir James Watt Magellan one of the early great maritime explorers. Lind reported the results in his publication A treatise on the scurvy in 1753 which last year celebrated its’ 250th anniversary. He concluded that oranges and lemons were the “most effectual remedies for this distemper at sea”. However he was unable to reproduce his initial findings using a condensate of lemon juice (in which most of the vitamin C had been lost by heating) and it took another 42 years before the Royal Navy adopted the daily provision of lemon juice on board ship to prevent the disease. The Royal Navy was similarly affected. A British Commodore George Anson reported losing 1300 ships’ crew to the disease during his 4-year 18th century circumnavigation of the world. Many believed that ‘blocked perspiration’ was a cause of many putrid diseases prevalent at that time. The skin was considered a major route of excretion of undesirable ‘vapors and humors’ from the body and at sea this route was blocked by the cold damp environment, leading to scurvy. This resulted in a number of different proposed treatments for the disease designed to purge the body of these toxins by inducing vomiting and diarrhoea. However anecdotal reports describing the remarkable benefits of citrus fruit in relieving the symptoms began to appear and would eventually lead to a young naval surgeon, James Lind, to undertake the first ever clinical trial in scientific history. Return of the 1875 Arctic sledging party which had suffered severly from scurvy. In the foreground is an emply ship’s 60-pound container of lime juice which had been brought to them by the rescue party. (Scott Polar Research Institute, Cambridge) The details of Robert Scotts’ second and final expedition to Antarctica are known to many. On reaching the South Pole the expedition party discovered the Norwegian flag left there 3 weeks previously by Amundsen. Their morale low, the party struggled back on very limited rations. Famously one night Captain Oates, who realising that he was slowing the group, deliberately walked out into a blizzard so that he would be frozen to death by morning. Oates had particular problems walking on one foot and it is postulated that the scar tissue from old wounds sustained in the Boer War may have been breaking up as a result of scurvy. Scott and the remainder of his team also died before reaching their final depot. Although no mention is made of scurvy by the search party that found their frozen bodies, many believe that scurvy significantly contributed to their ultimate demise. A disease of affluence One final group affected by scurvy were infants in middle and upper class families around the beginning of the 20th century. After weaning, these children were fed with proprietary food such as oatmeal and rusks with evaporated or condensed milk, which lacked vitamin C. Subperiosteal haemorrhage, first described in this condition by Thomas Barlow, led to pain in the extremities and an affected child would typically adopt the so-called frog position to minimise pressure on the swollen painful leg. Scurvy today Most contemporary cases of scurvy are seen in the setting of extreme dietary limitation. Danish beer no longer contains vitamin C as an antioxidant putting alcoholics at risk. The disease offers the clinician the opportunity to make a diagnosis of great historical interest and to observe a most rewarding rapid clinical improvement with the simplest explanation and cure. References A treatise on the scurvy and author James Lind 1. Carpenter KJ. The history of scurvy and vitamin C. Cambridge University Press. 1986. 2. Hirschmann JV, Raugi GJ. Adult scurvy. J Am Acad Dermatol 1999; 41(6): 895-910. 3. Black MM, Gawkrodger DJ, Seymour CA, Weismann K. Metabolic and nutritional disorders. In: Textbook of Dermatology 6th Edition. Blackwell Science; 3: 2660-2661. 4. The James Lind Library (www.jameslindlibrary.org) 2004. 5. Sutton G. Putrid gums and “Dead Men’s Cloaths’”: James Lind aboard the Salisbury. J R Soc Med. 2003 Dec; 96(12): 605-8.
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