- See: vascular malformations of the upper extremity
- Discussion:
- first described by Maurice Raynaud in 1862;
- involves vasoconstriction of the cutaneous arteriovenous shunts, digital arteries, and precapillary arterioles;
- pallor of the digits with or w/o cyanosis on exposure to cold;
- Raynaud's disease (primary form):
- definition: when the phenomenon occurs w/o another demonstrable or associated disease;
- absence of organic disease (causing the symptoms)
- more common than Raynaud's phenomenon;
- mainly occurs in young women (median age of onset is 14 years);
- bilateral and symmetric involvement
- absence of clinical occlussion of peripheral arteries
- gangrene or trophic changes are absent or are limited to distal digital skin;
- normal nail-fold capillaries;
- negative test for antinuclear antibody;
- normal erythrocyte sedimentation rate.
- Raynaud's phenomenon (secondary form):
- definition: when the phenomenon occurs with another inciting disorder;
- most commonly seen in females in their late 20s to late 30s;
- some of these patients will demonstrate others signs of vascular pathology such as migraines or pulmonary hypertension;
- patients may note that symptoms are triggered by a cold room or by strong emotions or anxiety;
- lab data demonstrates auto-antibodies;
- antinuclear antibodies
- contributing disorders:
- scleroderma patients with scleroderma often first present with Raynaud's phenomenon;
- typically associated with anticentromere antibody
- look for ANA titer > 1000
- ref: Images in Clinical Medicine. Cold Hands Associated with Scleroderma
- other inflammatory arthritic conditions: RA SLE, dermatomyositis;
- neurologic disorders
- arterior occlusive disorders
- blood dyscrasias
- Exam:
- usually all digits will be affected during vascular episodes;
- triple color changes:
- digital pallor, episodic cyanosis, hyperemia;
- pallor results from arterial insufficiency, where as cyanosis results from deoxygenation of blood which remains in the finger;
- reactive hyperemia occurs after the vascular episode is over;
- ref: Images in Clinical Medicine. Primary Raynaud's Phenomenon
- character and quality of right and left radial pulses (irregular heart beat and look for extinguished pulse with hyperabduction and E.
rotation of shoulder);
- assess patency of radial and ulnar digital arteries with Allen's test, and then assess patency of the arteries to the digits using
a digital Allen's test;
- Lab data:
- laboratory findings will typically be negative in primary Raynaud's disease where as in Raynaud's phenomena, positive findings
are not uncommon;
- sed rate:
- an increased sed rate may point to a collagen vascular disease;
- thyroid panel;
- these patients will often have a subtle hypothyroidism;
- cryoglobulins:
- many patients with significant amounts of cryoglobulins are asymptomatic; other patients develop purpura, Raynaud's
phenomena, cyanosis, and tissue necrosis when exposed to cold;
- pts w/ mixed cryoglobulinemia frequently have vasculitis, glomerulonephritis, lymphoproliferative disorders, or chronic
infection, (esp w/ hepatitis B virus);
- protein electrophoresis;
- measurement of C3 and C4 compliment levels;
- arterial ultrasound or arteriography in selected cases;
- Radiographs:
- Images in Clinical Medicine: Band Acro-osteolysis
- Management:
- patients with the primary form of the disease are more often successfully treated than patients w/ the secondary form;
- patients with scleroderma will be more resistant to treatment than patients w/o scleroderma;
- adress environmental conditions:
- protection from the cold;
- stop smoking;
- vibrating machinery and repetitive conditions (carpal tunnel syndrome);
- harmful medications:
- estrogen, cocaine, clonidine, sympathomimetic agents, serotonin-receptor agonists;
- beneficial medications:
- ASA;
- alpha blocking agents (dibenzyline), and myovascular relaxants (nicotinic acid, cyclospasmol, and NTG oitment)
- catecholamine and or serotonin depletors (reserpine)
- thyroxine;
- calcium channel blockers:
- nifedipine (either short or long acting) may be more effective in preventing attacks than the other types of calcium
channel blockers;
- surgical:
- digital and or cervical sympathectomy;
- sympathectomy at the level of the hand should include the radial and ulnar arteries as well as the superficial palmar arch;
- cold stress testing may help indicate which patients will have a favorable response to sympathethectomy;
- references: Adventitial stripping of the radial and ulnar arteries in Raynaud's disease
References
Treating Raynaud's phenomenon and its underlying causes. Wigley FM. J Musculoskel Med. 1998:53.
The microcirculatory effects of peripheral sympathectomy.
Clinical practice. Raynaud's Phenomenon.
Images in clinical medicine. Cold hands associated with scleroderma.