CPC #3: The Mummified Finger Bridging the Gap. Where Clinical and Basic Sciences Meet CPC #3: The Mummified Finger Faiz Rehman, MD Fellow, Cardiovascular Diseases Louisa Balazs, MD, PhD Associate Professor of Pathology Karl T. Weber, MD Professor of Medicine

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, fall’s harvest had provided a good yield—an outcome in doubt given Spring’s heavy rainfall and June’s hot sun.

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, fall’s harvest had provided a good yield—an outcome in doubt given Spring’s heavy rainfall and June’s hot sun.

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, fall’s harvest had provided a good yield—an outcome in doubt given Spring’s heavy rainfall and June’s hot sun.

Sustenance for destitute Mr. H 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.

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.

For H. , painful fingers turned black 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,

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.

Weeks ago, his right hand became swollen and painful 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. Dupuytren’s contractures were present bilaterally. Only in Poland had he smoked cigarettes.

Blood pressure normal; heart and abdomen unremarkable 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.

Occlusions of digital arteries and ulnar artery were found on arteriography. Buy why? Vasospasm due to a circulating substance?

And then there was Mr. S. , a 45-year-old service station worker 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.

And then there was Mr. S. , a 45-year-old service station worker 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.

Normal examination except cool 4th and 5th digits of the left hand with Dupuytren’s contraction. A cord-like mass was felt radial to hypothenar muscles; ulnar pulse was diminished and Allen’s test positive.

Normal examination except cool 4th and 5th digits of the left hand with Dupuytren’s contraction. A cord-like mass was felt radial to hypothenar muscles; ulnar pulse was diminished and Allen’s test positive.

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?

Faiz Rehman MD And K.T Weber MD The Mummified Finger Faiz Rehman MD And K.T Weber MD

Mr H (symptoms) History of ingestion of flour having a”sticky 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

Mr H (exam) Mr H.’s finger falls off

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

Mr G (exam) BP normal Heart normal Abdomen normal Dupuytren’s 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

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

Arteriography showed occlusion of right ulnar artery and digital arteries

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

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

Mr S (exam) Cool 4th and 5 th digits Dupuytrens’s contracture Cord-like mass radial to hypothenar muscles. Ulnar pulse diminished Allen’s test positive

Mr S (arteriography) Sparse vascularity of digits Aneurysm of ulnar artery next to hamate bone

Differential diagnosis Ergotism Buerger’s disease Hammer-hypothenar syndrome Polyarteritis Nodosa Raynaud Phenomenon Reflex Sympathetic Dystrophy Scleroderma Takayasu Arteritis

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

Buerger’s disease

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.

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.

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

Ergotamine induced arterial constriction ERGOTISM Ergotamine induced arterial constriction

Claviceps purpurea

Claviceps purpurea

Rye contaminated by fungus

Gangrenous ergotism

Gangrene-ergotism

Gangrene-ergotism

Thrombangiitis obliterans Gangrenes of extremities Buerger’s disease Thrombangiitis obliterans Gangrenes of extremities

Throbangiitis obliterans

Tobacco use-Buerger’s disease

Buerger’s disease

Dupuytren’s contracture Benign fibroblast proliferation, Palmar/plantar

Dupuytren’s contracture

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.

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.

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.

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.

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. 

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.

Thromboangiitis obliterans (Buerger’s 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.

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 Raynaud’s phenomona, it appears when the hand is used as a hammer and where the hamate bone compresses the ulnar artery.

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 Raynaud’s phenomona, it appears when the hand is used as a hammer  and where the hamate bone compresses the ulnar artery.

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 Raynaud’s phenomona, it appears when the hand is used as a hammer and where the hamate bone compresses the ulnar artery.

The fibrocontractive disorder of palmar fascia known as Dupuytren’s 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.

The fibrocontractive disorder of palmar fascia known as Dupuytren’s 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.