416
U N I T 5
Circulatory Function
The impaired circulation increases sensitivity to cold.
The peripheral pulses are diminished or absent, and
there are changes in the color of the extremity. In mod-
erately advanced cases, the extremity becomes cyanotic
when the person assumes a dependent position, and the
digits may turn reddish-blue even when in a nondepen-
dent position. With lack of blood flow, the skin becomes
thin and shiny, hair growth slows, and nail growth is
impaired. Chronic ischemia causes thick, malformed
nails. If the disease continues to progress, tissues even-
tually ulcerate and gangrenous changes arise that may
necessitate amputation
2
(Fig. 18-8).
Diagnostic methods are similar to those for athero-
sclerotic disease of the lower extremities. It is essen-
tial that the person stop smoking cigarettes or using
tobacco. Even passive smoking and nicotine replace-
ment therapy should be eliminated. Other treatment
measures are of secondary importance and focus on
methods for producing vasodilation and preventing tis-
sue injury. Sympathectomy, or excision of a segment of
a sympathetic nerve, may be done to alleviate the vaso-
spastic manifestations of the disease. As the distal arte-
rial tree is occluded, revascularization is not possible. If
smoking cessation is not achieved, then the prognosis
is generally poor, with amputation of both lower and
upper extremities the eventual outcome.
Raynaud Phenomenon
Raynaud phenomenon is a functional disorder caused
by intense vasospasm of the arteries and arterioles in
the fingers and, less often, the toes.
1,2,21,22
The disorder is
divided into primary and secondary types.
Primary Raynaud phenomenon is seen in otherwise
healthy young women, and it often is precipitated by
exposure to cold or by strong emotions and usually is
limited to the fingers. The cause of vasospasm in pri-
mary Raynaud phenomenon is unknown or idiopathic.
Hyperreactivity of the sympathetic nervous system
has been suggested as a contributing cause. Secondary
Raynaud phenomenon is associated with previous vessel
injury, such as frostbite, occupational trauma associated
with the use of heavy vibrating tools, collagen diseases,
neurologic disorders, chronic arterial occlusive disor-
ders, and drugs, such as bleomycin. Another occupa-
tion-related cause is the exposure to alternating hot and
cold temperatures such as that experienced by butchers
and food preparers. Raynaud phenomenon often is the
first symptom of collagen diseases. It occurs in almost all
persons with scleroderma and can precede the diagnosis
of scleroderma by many years.
22
The ischemic phase of Raynaud phenomenon is
manifested by changes in skin color that progress from
pallor to cyanosis, a sensation of cold, and changes in
sensory perception, such as numbness and tingling. The
color changes usually are first noticed in the tips of the
fingers, later moving into one or more of the distal pha-
langes (Fig. 18-9). After the ischemic episode, there is
a period of hyperemia with intense redness, throbbing,
and paresthesias. The period of hyperemia is followed
by a return to normal color. Primary Raynaud phenom-
enon attacks typically involve all fingers in a symmetric
fashion and are associated with minimal pain, whereas
asymmetric finger involvement and intense pain suggest
secondary Raynaud phenomenon.
21,22
In severe progres-
sive cases, trophic skin changes may develop. The nails
may become brittle, and the skin over the tips of the
affected fingers may thicken. Ulceration and superficial
gangrene of the fingers, although infrequent, may occur.
The initial diagnosis is based on a history of vaso-
spastic attacks supported by other evidence of the disor-
der. Immersion of the hand in cold water may be used to
initiate an attack as an aid to diagnosis. Laser Doppler
flow velocimetry may be used to quantify digital blood
flow during changes in temperature. Primary Raynaud
phenomenon is differentiated from secondary Raynaud
FIGURE 18-8.
Buerger disease. Hand showing necrosis of
the tips of the fingers.Temporal arteritis. (From Gotlieb AI,
Lui A. Blood vessels. In: Rubin R, Strayer DS, eds. Rubin’s
Pathology: Clinicopathologic Foundations of Medicine, 6th ed.
Philadelphia, PA: Wolters Kluwer Health/Lippincott Williams &
Wilkins; 2012:446.)
FIGURE 18-9.
Raynaud phenomenon.The tips of the fingers
show marked pallor. (From Gotlieb AI, Lui A. Blood vessels. In:
Rubin R, Strayer DS, eds. Rubin’s Pathology: Clinicopathologic
Foundations of Medicine, 6th ed. Philadelphia, PA: Wolters
Kluwer Health/Lippincott Williams &Wilkins; 2012:463.)
