Erythromelalgia (erythromelalgia) is also known as erythrothermal limb pain; erythromelalgia. It is a vascular dysfunctional disease that occurs in young people, most of whom are women. Patients with this disease often complain of burning and stinging pain at the ends of the extremities, especially the palms and soles of the feet, local congestion, redness, sweating and heat. The slightest pressure or friction by a foreign body makes the pain even more embarrassing. The pain tends to be constant and vague, intensifying in bursts. When the ambient temperature is a little higher, or when the extremities are congested with blood due to frequent activities, the pain in the extremities intensifies. Therefore, the patient prefers to be barefoot, fears walking, and prefers to lie flat with both feet raised. When sleeping in winter, the limbs are often forced to be exposed outside the nest, or the hands and feet are often soaked in cold water to relieve pain. Over time, the skin and nails become thick and rough, accompanied by mild puffiness. In some cases, the skin and nails become thickened and rough, with mild puffiness. Some of them become necrotic and even disabling.
Overview.
Erythromelalgia (erythromelalgia) is a rare, paroxysmal vasodilatory peripheral autonomic disorder of unknown etiology most often complicated by thrombocythemia and true erythrocytosis. The disease was first reported by Mitchell (1878), and in 1938 Smith and Allen introduced the concept of erythermalgia, which is often confused with erythromelalgia and is characterized by elevated skin temperatures on the extremities, flushing and swelling, and severe burning pain, especially on the soles of the feet and toes, which increases when the ambient temperature increases.
Etiology.
Erythrodermic limb pain is a primary vascular disease caused by platelet increase platelet-mediated vascular inflammation and thrombotic response Erythrodermic limb pain is not associated with platelet increase but both involve autonomic innervated vessels and produce similar clinical symptoms. Idiopathic erythrodysesthesia is an autosomal dominant disorder secondary to erythrodysesthesia caused by drug intoxication, etc.
Pathogenesis.
The pathogenesis of this disorder is unknown. It may be due to central nervous system and autonomic nervous system disorders, which cause dysfunction of peripheral vasomotor function and extreme dilatation of small arteries in the extremities, resulting in local blood flow disorders and local congestion. The clinical symptoms occur when intravascular tension increases to compress or stimulate the adjacent nerve endings, and the disease is treated with 5-hydroxytryptamine antagonists with good results. It has been suggested that this disease is a disorder of prostaglandin metabolism and that the increased ability of prostaglandin to be synthesized in the lesioned tissues of patients may be related to the effectiveness of aspirin therapy. Capillary blood flow studies have shown an increased response of these tiny vessels to temperature, resulting in increased intracapillary pressure and marked dilation.
Primary erythermalgia occurs mostly in children or adolescents, with a family history and a reported gene locus at 2q31-32. Secondary erythermalgia is often combined with primary hypertension, diabetes mellitus multiple sclerosis infectious mononucleosis, systemic lupus erythematosus, and a few are side effects of medications. Secondary erythrodermic limb pain often occurs in true erythrocytosis, hypertension, diabetes mellitus, lupus erythematosus, gout, rheumatoid arthritis, neuritis, etc.
Clinical manifestations.
1, mostly seen in young and middle-aged people the main symptoms are mostly seen in the extremities, especially the feet are commonly manifested toes or soles red, swollen, hot, painful pain is paroxysmal, can last for several minutes hours or days as intense burning pain, obvious at night and more episodes higher temperature environment, prolonged standing and walking or feet drooping easy to make the symptoms aggravated patients are reluctant to wear socks or gloves for fear of increased pain, when sleeping like to put both feet on the quilt The pain can be relieved by walking on cold ground, dipping the foot in cold water or elevating the affected limb.
2, examination can be seen in the affected limb skin color red skin temperature increased vasodilatation, mild swelling and sweating due to the small intracutaneous arteries and capillaries significantly dilated light pressure on the skin of the limb can make the red temporarily fade, the affected limb dorsalis pedis artery pulsation is normal with slight acupressure edema without motor, reflex and sensory impairment
Complications.
A few patients develop nutritional disorders in late stages, thickening and ulceration of the skin and nails of the extremities, and occasionally gangrene of the skin secondary to erythrodysesthesia mostly combined with clinical manifestations of primary hypertension, diabetes mellitus, multiple sclerosis, infectious mononucleosis systemic lupus erythematosus and other diseases
Diagnosis.
Pay attention to the four major symptoms of paroxysmal redness, swelling, heat and pain in the extremities followed by questioning of the medical history with the pain intensifying when heated and relieved by local cold compresses, then the diagnosis of most cases is not difficult. There is a clear family history of idiopathic red hot limb pain. The ability of small doses or single doses of aspirin to specifically and rapidly reduce or eliminate the painful symptoms of platelet hyperplastic erythromelalgia can be used as a characteristic diagnostic criterion.
Differential diagnosis.
1, Raynaud’s disease is mostly seen in young women cold is the main trigger performance pale cyanosis and flushing local skin temperature low warmth can reduce
2, thrombo-occlusive vasculitis is almost always male, reduced blood flow leads to intermittent claudication, pale or cyanotic skin, diminished dorsalis pedis artery pulsation, etc.
3, calf erythroderma cold as the onset of erythema predominantly in the lower legs, no obvious pain should also be distinguished from erythrocytosis, diabetic peripheral neuritis, etc. In addition, spinal consumption, subacute spinal cord joint degeneration spinal cord cavitation, etc. can be found in the extremities of sensory abnormalities but they have no other objective signs and symptoms of sensory impairment in addition to mild pallor of the skin during attacks and other features.
Examination.
Laboratory tests.
1, often with thrombocytosis and erythrocytosis
2, blood biochemical tests and cerebrospinal fluid routine tests are mostly non-specific but secondary red fever limb pain is associated with the primary disease
3, drug and toxicological tests have differential diagnostic significance
Other auxiliary examinations.
1, the majority of cranial limb imaging is normal results.
2, genetic testing has confirmed the diagnosis of primary erythermalgia (erythermalgia) or differential diagnosis significance
Related tests.
Cerebrospinal fluid, platelet therapy, should first determine whether it is primary erythermal limb pain or secondary erythermal limb pain. Secondary is erythema limbicum caused by other systemic diseases, mostly seen in hematologic diseases such as erythrocytosis and thrombocytosis, autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus, and also in diseases such as multiple sclerosis, spinal cord disease, diabetes mellitus, and AIDS. If it is secondary, the cause should be identified and the primary disease should be treated actively. Concurrent treatment also includes
Western medicine
1.General treatment
Bed rest, avoid prolonged standing, elevate the affected limb during the acute period. Apply cold compresses locally to reduce pain. After the acute period, strengthen the limb activity exercise and avoid any stimulation that causes local vasodilation.
2.Drug treatment
(1) Platelet hyperplastic erythema limb pain can be treated with small doses of aspirin 50~100mg/d orally.
(2) β-blocking drugs such as propranolol 20～40mg/d orally, 3 times/d
(3) 0.15% procaine 500-1000ml intravenous drip 1 time / d for 5 days as a course of treatment, adrenocorticotropic hormone short-term shock therapy may also control the symptoms.
(4) Application of 5-hydroxytryptamine antagonists, such as dimethyl ergometrine (Methysergide) 2mg / time 3 times / d; or phenothiazine (Pizotifen), 0.5mg / time 1 to 3 times / d can often be taken to obtain complete relief. The affected limb is closed with 10ml of a mixture of 1% lidocaine and 0.25% bupivacaine (Bupivacaine), plus 10ml of saline diluted for the upper ankle ring and acupuncture point injection in severe cases or its liquid for the sacral epidural local seal also has a certain effect.
(5) Local herbal topical application; aspirin can be taken in small doses for patients with hematologic disorders secondary to thrombocytosis; 5-hydroxytryptamine reuptake inhibitors, patients sensitive to this class of drugs, should be applied from small doses; prostaglandins relieve symptoms by improving circulation; tricyclic antidepressants, calcium channel antagonists, beta-blockers, gabapentin, clonazepam, etc. are also therapeutic for this disease. The effect.
3.Special treatment
Idiopathic red and hot limb pain can be treated with local nerve block effectively by choosing supra-ankle ring closure sacral epidural closure or lumbar sympathetic ganglion block secondary red and hot limb pain patients should eliminate or intervene related etiology
4.Closure therapy
5.Physical therapy: ultrasound or ultrashort wave therapy can be used.
Prognosis and prevention
Prognosis: The prognosis of this disease is generally good secondary red fever limb pain is mainly treated for the primary disease can be relieved by treatment but may recur.
Prevention: There are no good measures to prevent the birth of children with genetic backgrounds, including avoidance of consanguineous marriage, implementation of genetic counseling, carrier genetic testing and prenatal diagnosis and selective abortion.
Prevention of secondary red fever limb pain is the main focus of prevention of the original disease
Health tips
1. During the cold season, pay attention to limb insulation and keep shoes and socks dry; when riding, standing or walking for a long time, it is advisable to change the posture in time and get out of the car regularly to prevent or reduce attacks or alleviate symptoms.
2. Symptomatic treatment is the main treatment. Local cold compresses or cold water immersion of the affected limb can be given to alleviate the symptoms during seizures; elevate the affected limb and avoid adverse stimulation such as overheating or stroking.
3.Oral rifampin, chlorpromazine, rifampin, etc. may improve the symptoms.
4.Intersacral nerve block and lumbar sympathetic nerve block have better efficacy.
5.The disease often has remission, relapse, and may have a chronic course. Most of them have good prognosis and can recover naturally.