Scurvy - British Association of Dermatologists

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.