Raynaud Phenomenon Clinical Presentation
- Author: Heather Hansen-Dispenza, MD; Chief Editor: Herbert S Diamond, MD more...
History
Numbness and pain in the affected area or areas may be present.
Affected areas show at least two color changes: white (pallor), blue (cyanosis), and red (hyperemia). The color changes are usually in the order noted, but not always. These changes should be reversible but may, in severe cases, lead to local ischemia and ulceration.
Any history of associated symptoms should raise suspicion of an underlying disorder. History of other vasospastic symptoms such as migraines may be useful.
Obtain occupational history.
- Industrial exposure to polyvinyl chloride has been implicated.
- Any history of injury or frostbite may leave the involved limb vulnerable to vasospasm.
- Raynaud phenomenon may be associated with laboratory work, with exposure to organic solvents such as xylene, toluene, acetone, or chlorinated solvents.[18]
Syndromes associated with Raynaud phenomenon include the following:
- Autoimmune disorders
- Progressive systemic sclerosis (scleroderma) including the diffuse and limited (formerly called CREST syndrome)
- Systemic lupus erythematosus
- Mixed connective-tissue disease (and other overlap syndromes)
- Dermatomyositis and polymyositis
- Rheumatoid arthritis
- Sjögren syndrome
- Vasculitis
- Primary pulmonary hypertension
- Infectious syndromes
- Hepatitis B and C infections (especially associated with mixed or type 3 cryoglobulinemia)
- Mycoplasma infections (with cold agglutinins)
- Neoplastic syndromes
- Lymphoma
- Leukemia
- Myeloma
- Waldenström macroglobulinemia
- Polycythemia
- Monoclonal or type 1 cryoglobulinemia
- Lung adenocarcinoma
- Other paraneoplastic disorders
- Environmental associations
- Vibration injury
- Vinyl chloride exposure
- Frostbite
- Lead exposure
- Arsenic exposure
- Organic solvent exposure (eg, xylene, toluene, acetone, chlorinated solvents)[18]
- Metabolic/endocrine syndromes
- Acromegaly
- Myxedema
- Diabetes mellitus
- Pheochromocytoma
- Fabry disease
- Hematologic syndromes
- Paroxysmal nocturnal hemoglobinuria
- Polycythemia
- Cryofibrinogenemia
- Drug-related associations
- Oral contraceptives
- Ergot alkaloids
- Bromocriptine
- Beta-adrenergic blocking drugs
- Antineoplastics (eg, vinca alkaloids, bleomycin, cisplatin)
- Cyclosporine
- Alfa-interferon
Syndromes that may be confused with Raynaud phenomenon are as follows:
- Anatomic syndromes
- Miscellaneous circulatory syndromes
- Atherosclerosis
- Thromboangiitis obliterans
- Vasculitis
- Thromboembolic disease
- Vasospastic syndromes
- Livedo reticularis
- Acrocyanosis
- Chilblains
Causes
The cause of primary Raynaud phenomenon remains unknown.
Possible causes for secondary Raynaud can be divided into several broad categories, including the following:
- Occupational
- Hematologic
- Collagen-vascular (autoimmune)
- Medication-induced
- Miscellaneous syndromes such as Fabry disease, pheochromocytoma, lung adenocarcinoma, acromegaly, carpal tunnel syndrome, and myxedema
Although the following entities do not usually have the same inciting causes, nor do they encompass the usual color changes associated with Raynaud phenomenon, they can easily be mistaken for Raynaud phenomenon:
- Vasculitis
- Carpal tunnel syndrome
- Reflex sympathetic dystrophy
- Thromboembolic disease
- Thoracic outlet syndrome
Physical Examination
Carefully examine digits if either primary or secondary Raynaud is suspected.
- Observe for sclerodactyly, calcinosis, or digital ulcers.
- Examine nailfold capillaries under magnification from a dissecting microscope or ophthalmoscope to help diagnose underlying autoimmune disorders. Abnormalities often appear in patients with early scleroderma. The normally regular pattern of capillary loops is replaced with abnormally large loops, alternating with areas without any capillaries.
- A cold challenge test can trigger Raynaud phenomenon in the office setting but is not usually necessary to make the diagnosis.
- A sharp demarcation of the border between the affected and unaffected areas is required for diagnosis. Many patients do not have classic triphasic color changes.
Evaluate any signs or symptoms of other syndromes associated with secondary Raynaud.
- Bone pain may suggest a paraneoplastic syndrome associated with a hyperviscosity syndrome.
- The presence of nephritis, malar erythema, and arthritis suggests systemic lupus erythematosus.
Persistent cyanosis or necrotic distal tissue suggests an underlying disorder or permanent ischemia. Livedo reticularis suggests an autoimmune disorder or coagulation abnormality.
Carpal tunnel syndrome has been associated with an increased frequency of Raynaud phenomenon.
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