Raynaud’s phenomenon is a paroxysmal vascular peripheral vascular disease. It is due to transient ischemia of the fingers, often triggered by cold and emotion. Frequent, it would affect 8% of women and 5% of men.
The diagnosis is clinical. The main difficulty is not to recognize the Raynaud’s phenomenon, but to establish the etiologic diagnosis.
Indeed, it may be unjust, isolated (primary Raynaud’s phenomenon) or secondary to a general cause (first, scleroderma in women).
DIAGNOSIS:
The approach is based primarily on the interrogation and clinical examination, supported by a few simple tests which capillaroscopy.
A unilateral Raynaud’s phenomenon towards a locoregional because while a bilateral phenomenon towards a general cause.
The three phases of Raynaud’s phenomenon:
Raynaud’s phenomenon is cut in three phases. Only the first phase (white) is constant and necessary for the diagnosis.
Phase syncope (white phase):
This phase corresponds to a screeching halt, but transient, finger arterial circulation.
The fingers are white, described as “dead”, painful, often with a loss of sensitivity.
The syncopal phase lasts from minutes to an hour.
Phase asphyxia (blue phase):
The blue phase corresponds to a slowdown in the venular flow. The fingers are blue and painful. It is inconstant and lasts from a few minutes to an hour.
Hyperemic phase (red phase):
The red phase corresponds to a reflex arterial vasodilation.
Differential diagnosis:
Raynaud’s phenomenon is not the only vascular peripheral vascular disease. The clinical characteristics of other symptoms can differentiate.
Acrocyanosis:
Unlike Raynaud’s syndrome, acrocyanosis is a permanent phenomenon more common in women than in men.
Permanent cyanosis of the extremities, favored by the cold, it is not painful. This is a vascular functional disorder.
Erythermalgia:
The erythermalgia opposed point by point in Raynaud’s phenomenon, since it is a peripheral vascular disease triggered by heat and effort (not cold), with red and warm extremities (not white and cold) due to excessive vasodilatation (not vasoconstrictor).
The erythermalgia is mainly primary or secondary to a myeloproliferative disorder or a vasodilator treatment.
Frostbite:
Frequently, frostbite caused by exposure to cold rather moderate and humid. Women are affected more often. There is often a family history. Purple papules stretch over several weeks on the dorsal surface of the fingers or toes. It should not be confused with vasculitis.
Chilblain:
It is a skin necrosis secondary to exposure to extreme cold. They affect the subjects debilitated defenses against the cold lessened (for example, the homeless) or young people exposed to extreme cold (as mountaineers). The affected region is livid, anesthetized. Bullous and necrotic lesions appear secondarily.
Examination:
The research examined:
– Family history of Raynaud’s phenomenon;
– Personal history: migraine, vascular problems, thyroid dysfunction, any neoplasia;
– Vascular risk factors;
– Drug intake: vasoconstrictor drugs especially beta blockers type migraine;
– Patient profession: including tile, jackhammer user;
– Leisure activities, especially karate.
Clinical examination:
Clinical examination should be general, with particular attention to the vascular examination (palpation of pulse, blood auscultation, etc.) looking for:
– Trophic disorders: especially pulp ulcerations, scarring;
– Skin lesions suggestive: including telangiectasia, erythema periungual.
Additional tests:
Some additional tests may be required to guide the etiologic diagnosis:
– Blood count (CBC) platelets, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), fibrinogen;
– Research antinuclear factors (FAN) with particularly centromere antibodies and anti ScL 70;
– Radiograph of the hands (calcinosis, acro-sclerosis);
– X-ray of the lungs (pulmonary fibrosis, cervical rib);
– Capillaroscopy (organic microangiopathy capillary rarefaction, macro-capillaries).
ETIOLOGIES:
No cause:
The most common cause is the lack of cause. Indeed, it is estimated that about 75% of the frequency of primary Raynaud’s phenomenon. The latter, also called Raynaud’s disease, are due to hypersensitivity vasoconstrictor receptors and a disturbed secretion of vasoactive mediators.
The characteristics of primary Raynaud’s phenomena are the following:
– Age of the subject: young woman;
– Personal or family history of migraines Raynaud phenomenon;
– Sometimes hypersensitivity to cold, frostbite;
– Trigger crises typically cold and emotion;
– Bilateral involvement;
– Absence of necrotic lesions;
– No other symptoms;
– Normal minimum etiological, especially no organic microangiopathy in capillaroscopy;
– Greater scalability than two years.
The primitive nature of Raynaud’s phenomenon is also enhanced as scalability than five years.
Secondary to connective tissue:
The most common cause is systemic sclerosis.
Raynaud’s phenomenon is then bilateral acquired in a woman over 35 years.
We must seek a mouth or dry eye syndrome, telangiectasia, sclerodactyly, pulpal ulcers, trophic disorders, joint pain, esophageal or lung symptoms. This is most often the limited cutaneous form of systemic scleroderma known as CREST (calcinosis subcutaneous [C], Raynaud syndrome [R], dysfunction of the esophagus [E], sclérodactylie [S], telangiectasia [T ]) associated with centromere antibodies and risk of pulmonary arterial hypertension (PAH).Capillaroscopy is essential here, looking for organic microangiopathy signs.
Other collagen may be involved: systemic lupus, rheumatoid arthritis, Sharp syndrome, dermatomyositis, Sjogren’s syndrome, etc.
Arterial causes:
Buerger’s disease:
In a young patient, heavy smoker, he must think of Buerger’s disease. The concept of intermittent claudication foot, recurrent superficial venous thrombosis, of distal trophic disorders is sought.
Treatment is based on the complete and final cessation, which is often very difficult to obtain and antiplatelet agents.The IV prostacyclin analogues (iloprost Ilomédine®) is useful when pushed in hospitalization.
Digital arteritis:
In a middle-aged man, smoking, other cardiovascular risk factors (diabetes, hypertension, Dysmetabolic syndrome, dyslipidemia), look for a digital arteritis. The radial or ulnar pulse can be abolished. There trophic disorders with dry skin, cyanotic, blasts on arterial routes and other manifestations of atherosclerosis.
The patient is told to the vascular surgeon after completion of a Doppler descrambling.
Takayasu disease:
Takayasu’s arteritis affects women between 20 and 30 years. Besides Raynaud’s syndrome, there may be a limp effort upper limb postural syndrome, a blood pressure asymmetry.
Imaging tests (CT angiography or MR angiography especially) show evocative images (stenosis, dilatation, particularly achievement of post-spinal portion of the subclavian).
There are no classical cardiovascular risk factor. An association with Crohn’s disease or a history of tuberculosis can be.
The patient is entrusted to the specialist.
Disease of vibrating tools:
In a man exposed for more than six months of strain, with the presence of paresthesia (carpal tunnel syndrome), think the disease vibrating machinery. There is often a need to know that smoking wean and outplacement is usually required.
Unilateral Raynaud’s phenomenon:
A unilateral Raynaud’s phenomenon towards a vascular anomaly of the ipsilateral limb.
The abolition of a pulse, breath sousclavier are sought. An arterial Doppler and CT angiography or MR angiography more than arteriography are to perform.
The anomalies of the subclavian artery can cause Raynaud’s phenomenon by distal embolism (costoclavicular parade, atherosclerosis, arthritis).
Subjects using the palm of the hand as a tool in leisure or work may constitute the ulnar artery aneurysm with distal emboli.
Toxic and medicine:
Besides tobacco, taking cocaine and cannabis is sought. Their arrest is paramount. Vasoconstrictor drugs (derived from ergot, triptans, beta blockers, amphetamines) may be involved. Also include bleomycin, interferon-α, bromocriptine, cyclosporine, vinblastine. Occupational exposure to vinyl chloride (autoclave people descaling) may cause Raynaud’s syndrome with abnormal capillaroscopy.
Other causes:
Cryoglobulinemia, cold agglutinin be sought in cases of clinical orientation.
Conventionally, various causes are cited in Raynaud’s phenomenon: hypothyroidism, acromegaly, myeloproliferative disorders, neoplasms, etc.
TREATMENT:
Treatment is based on stopping the aggravating factors first: stop smoking or taking toxic stop suspicious drug intake, protection against cold and moisture.
Calcium channel blockers (type Tildiem® 160 1 / d) may be prescribed at the beginning of winter.
If trophic disorders, including digital necrosis in scleroderma, in addition to fat dressings and local care, iloprost infusions may be performed on
five days in hospital.