CPC

1
CPC 3 The Mummified Finger
Bridging the Gap. Where Clinical and Basic
Sciences Meet

• Faiz Rehman, MD
• Fellow, Cardiovascular Diseases

Louisa Balazs, MD, PhD Associate Professor of
Pathology
Karl T. Weber, MD Professor of Medicine
2
October 1, 1951, and hamlets throughout the south
of France were still in the throes of economic
recovery ? the struggle in Vietnam further
drained French resources. Despite postwar
scarcities, falls harvest had provided a good
yieldan outcome in doubt given Springs heavy
rainfall and Junes hot sun.
3
October 1, 1951, and hamlets throughout the south
of France were still in the throes of economic
recovery the struggle in Vietnam further drained
French resources. ? Despite postwar scarcities,
falls harvest had provided a good yieldan
outcome in doubt given Springs heavy rainfall
and Junes hot sun.
4
October 1, 1951, and hamlets throughout the south
of France were still in the throes of economic
recovery the struggle in Vietnam further drained
French resources. Despite postwar scarcities,
falls harvest had provided a good yieldan
outcome in doubt given Springs heavy rainfall
and Junes hot sun.
5
Sustenance for destitute Mr. H., his wife and two
children, depended on pickled pork, cheese, honey
and well water. Spirits soared when they learned
baker J. would provide them with flour. Though
mottled gray and having a sticky texture that
made working batter difficult, H. beamed when
bread tasted just fine.
6
Within days all were brought to clinic, where
Nicole Monier, resident in internal medicine, was
working. In wife and children, throbbing calves
gave way to burning pains and discolored toes.
7
For H., painful fingers turned black. As Nicole
examined his right index finger, it fell off!
What caused this affliction in previously healthy
people? None had fever and others in their
village had not taken ill. This vivid experience
remained with Nicole for years. Comparisons were
inevitable. For example,
8
Mr. G., a 25-year-old Polish immigrant whose
religious preference made him a target of the
Nazi regime of terror. His family had fled to
the French countryside, where they followed their
religious customs and diet. An office clerk, G.,
complained of a painful right index finger and
left thumb worsened by cooler weather.
9
Weeks ago, his right hand became swollen and
painful. No trauma, fever or chills. Months
earlier, he had a painful right calf on whose
medial aspect was a several-centimeter-long red
cord. The left calf was involved weeks later. A
gangrenous digit of the right hand had been lost
last winter while stacking firewood. Dupuytrens
contractures were present bilaterally. Only in
Poland had he smoked cigarettes.
10
Blood pressure normal heart and abdomen
unremarkable. Normal renal function. After
Nicole had compressed his right radial and ulnar
arteries, she instructed him to open and close
his hand until it blanched restoration of color
was rapid upon release of the radial artery, but
delayed when this maneuver was repeated for the
ulnar artery.
11
Occlusions of digital arteries and ulnar artery
were found on arteriography. Buy why? Vasospasm
due to a circulating substance?
12
And then there was Mr. S., a 45-year-old service
station worker. For one month he noted pain and
numbness of distal digits of his hands with
blanching, followed by bluish discoloration and
then throbbing redness cooler temperatures were
provocative. ? In changing tires or fixing dented
fenders, he used a hammer or his left hand as a
hammer.
13
And then there was Mr. S., a 45-year-old service
station worker. For one month he noted pain and
numbness of distal digits of his hands with
blanching, followed by bluish discoloration and
then throbbing redness cooler temperatures were
provocative. In changing tires or fixing dented
fenders, he used a hammer or his left hand as a
hammer.
14
Normal examination except cool 4th and 5th digits
of the left hand with Dupuytrens contraction. A
cord-like mass was felt radial to hypothenar
muscles ulnar pulse was diminished and Allens
test positive.
15
Normal examination except cool 4th and 5th digits
of the left hand with Dupuytrens contraction. A
cord-like mass was felt radial to hypothenar
muscles ulnar pulse was diminished and Allens
test positive.
16
Arteriography revealed sparse vascularity of
digits with aneurysm of the ulnar artery adjacent
to the hamate bone. Was a local substance
responsible for vasospasm, perhaps released from
injured tissue?
17
The Mummified Finger

• Faiz Rehman MD
• And
• K.T Weber MD

18
Mr H (symptoms)

• History of ingestion of flour having asticky
  texture and being mottled gray
• Within days wife and children develop throbbing
  calves with burning pains and discolored toes.
• Mr H.s fingers are painful and then turn black

19
Mr H (exam)

• Mr H.s finger falls off

20
Mr G (symptoms)

• Painful right index finger and left thumb,
  worsened by cooler weather.
• Swollen right hand for weeks (in the absence of
  trauma, fever or chills)
• Months earlier, painful left calf with along red
  cord medially /duration several weeks
• Gangrenous digit of right hand lost last winter
• Smoker

21
Mr G (exam)

• BP normal
• Heart normal
• Abdomen normal
• Dupuytrens contracture bilaterally
• Compression of right ulnar and radial arteries
  followed by opening and closure of hand until
  blanching seen ,resulted in restoration of color
  upon release of radial artery but delayed for the
  ulnar artery

22
Exam

• The Allen test is abnormal in two-thirds of
  patient. To perform this test, both the radial
  and ulnar arteries are compressed while the hand
  is clenched and then opened. This activity causes
  palmar blanching. Release of compression from
  either pulse should normally produce palmar
  erythema if the palmar arches are patent.
• If they are occluded, pallor persists on the
  side where compression is maintained.
• Discrete, tender, erythematous subcutaneous
  cords, indicating a superficial thrombophlebitis,
  may be present on the distal aspects of the
  extremities

23

• Arteriography showed occlusion of right ulnar
  artery and digital arteries

24

• Angiogram of the Hand Showing Multiple Occlusions
  of the Digital Arteries, with Collateralization
  ("Corkscrew Collaterals") around the Areas of
  Occlusion (Arrows).

25
Mr S (symptoms)

• Pain and numbness in 4th and 5th digits with
  blanching.
• Followed by bluish discoloration and then
  throbbing rednesss. (Cooler temperatures were
  provacative)
• Occupation Changing tires/fixing dented fenders

26
Mr S (exam)

• Cool 4th and 5 th digits
• Dupuytrenss contracture
• Cord-like mass radial to hypothenar muscles.
• Ulnar pulse diminished
• Allens test positive

27
Mr S (arteriography)

• Sparse vascularity of digits
• Aneurysm of ulnar artery next to hamate bone

28
Differential diagnosis

• Ergotism
• Buergers disease
• Hammer-hypothenar syndromePolyarteritis Nodosa
  Raynaud Phenomenon Reflex Sympathetic Dystrophy
  Scleroderma Takayasu Arteritis

29
Ergotism

• The dry gangrene is a result of vasoconstriction
  induced by the ergotamine-ergocristine alkaloids
  of the fungus.
• It effects the more poorly vascularized distal
  structures, such as the fingers and toes.
  Symptoms include desquamation, weak peripheral
  pulse, loss of peripheral sensation, edema and
  ultimately the death and loss of affected tissues

30
Buergers disease
31
CLINICAL FINDINGS.

• Patients may have claudication of the hands,
  forearms, feet, or calves. The majority of
  patients with TAO have pain at rest and digital
  ulcerations. Often, more than one extremity is
  affected.
• Raynaud phenomenon occurs in approximately 45
  percent of patients, and superficial
  thrombophlebitis, which may be migratory, occurs
  in approximately 40 percent of patients.

32
CLINICAL FINDINGS.

• Patients may have claudication of the hands,
  forearms, feet, or calves. The majority of
  patients with TAO have pain at rest and digital
  ulcerations. Often, more than one extremity is
  affected.
• Raynaud phenomenon occurs in approximately 45
  percent of patients, and superficial
  thrombophlebitis, which may be migratory, occurs
  in approximately 40 percent of patients.

33
Hypothenar hammer syndrome

• Thrombosis of the ulnar artery in the hand is the
  most common type of upper extremity vascular
  occlusion. 
• It is commonly known as hypothenar hammer
  syndrome because it is often the result of a
  constant pounding on the ulnar side of the palm
  of the hand such as might be associated with
  roofing workers. 
• Multiple symptoms can result, including and not
  limited to pain, numbness and tingling, weakness
  of grip, discoloration of the fingers and even
  ulcers of the finger tips

34
ERGOTISM

• Ergotamine induced arterial
• constriction

35
Claviceps purpurea
36
Claviceps purpurea
37
Rye contaminated by fungus
38
Gangrenous ergotism
39
(No Transcript)
40
Gangrene-ergotism
41
Gangrene-ergotism
42
Buergers disease

• Thrombangiitis obliterans
• Gangrenes of extremities

43
Throbangiitis obliterans
44
Tobacco use-Buergers disease
45
Buergers disease
46
Dupuytrens contracture

• Benign fibroblast proliferation,
• Palmar/plantar

47
Dupuytrens contracture
48
In the Middle Ages, gangrenous ergotism appeared
west of the Rhine River the convulsive form to
the east. An outbreak of ergotism appeared in
the south of France some 40 years ago. With the
gangrenous form, limbs and/or digits become
swollen excruciating pain follows. Numbness
occurs suddenly and affected parts turn black
mummified, they are lost without pain or
hemorrhage.
49
A moist spring and dry June favor contamination
of rye by the fungus Claviceps purpurea, whose
hard, purple body, or sclerotium, gives infected
plant ovaries ? a cockspur (ergot in French)
appearance. Midwives had long given ergot to
hasten childbirth the alkaloid ergotamine was
used for migraine headache and pruritus of
hepatic origin. Toxic effects, due to intense
arterial constriction, resembled those seen in H.
and family.
50
A moist spring and dry June favor contamination
of rye by the fungus Claviceps purpurea, whose
hard, purple body, or sclerotium, gives infected
plant ovaries a cockspur (ergot in French)
appearance. ? Midwives had long given ergot to
hasten childbirth the alkaloid ergotamine was
used for migraine headache and pruritus of
hepatic origin. Toxic effects, due to intense
arterial constriction, resembled those seen in H.
and family.
51
A moist spring and dry June favor contamination
of rye by the fungus Claviceps purpurea, whose
hard, purple body, or sclerotium, gives infected
plant ovaries a cockspur (ergot in French)
appearance. Midwives had long given ergot to
hasten childbirth the alkaloid ergotamine was
used for migraine headache and pruritus of
hepatic origin. ? Toxic effects, due to intense
arterial constriction, resembled those seen in H.
and family.
52
A moist spring and dry June favor contamination
of rye by the fungus Claviceps purpurea, whose
hard, purple body, or sclerotium, gives infected
plant ovaries a cockspur (ergot in French)
appearance. Midwives had long given ergot to
hasten childbirth the alkaloid ergotamine was
used for migraine headache and pruritus of
hepatic origin. Toxic effects, due to intense
arterial constriction, resembled those seen in H.
and family. ?
53
A moist spring and dry June favor contamination
of rye by the fungus Claviceps purpurea, whose
hard, purple body, or sclerotium, gives infected
plant ovaries a cockspur (ergot in French)
appearance. Midwives had long given ergot to
hasten childbirth the alkaloid ergotamine was
used for migraine headache and pruritus of
hepatic origin. Toxic effects, due to intense
arterial constriction, resembled those seen in H.
and family.
54
Thromboangiitis obliterans (Buergers disease),
with inflammation and thrombosis of small- and
medium-sized arteries and veins of lower and/or
upper extremities, occurs predominantly in young
men whose ethnic origins may include northern
Slavic countries (e.g., Askenazium). Sour rye
bread is a dietary staple of these people
smoking is not a consistent habit. Migratory
superficial thrombophlebitis is frequent.
55
The hammer hypothenar syndrome involves the
superficial palmar branch of the ulnar artery, ?
as shown here for a person who stacked lumber.
Often associated with Raynauds phenomona, it
appears when the hand is used as a hammer and
where the hamate bone compresses the ulnar artery.
56
The hammer hypothenar syndrome involves the
superficial palmar branch of the ulnar artery, as
shown here for a person who stacked lumber.
Often associated with Raynauds phenomona, it
appears when the hand is used as a hammer ? and
where the hamate bone compresses the ulnar artery.
57
The hammer hypothenar syndrome involves the
superficial palmar branch of the ulnar artery, as
shown here for a person who stacked lumber.
Often associated with Raynauds phenomona, it
appears when the hand is used as a hammer and
where the hamate bone compresses the ulnar artery.
58
The fibrocontractive disorder of palmar fascia
known as Dupuytrens contracture, ? is based on
?-smooth muscle actin-containing myofibroblasts
whose contractility is induced by peptides (e.g.,
angiotensin II and endothelin-1). Such
substances, if released by these cells, could
promote vasospasm of involved vessels.
59
The fibrocontractive disorder of palmar fascia
known as Dupuytrens contracture, is based on
?-smooth muscle actin-containing myofibroblasts
whose contractility is induced by peptides (e.g.,
angiotensin II and endothelin-1). Such
substances, if released by these cells, could
promote vasospasm of involved vessels.