Livedo is a pathology of the vessels of the skin and subcutaneous tissue, which develops as a result of vasomotor disorders, leads to the development of a transient or persistent spotted reddish–blue or purple reticulated cyanotic pattern. It can be an independent disease or a significant symptom of a number of endocrine, neurological, infectious, autoimmune pathological processes. The diagnosis is established on the basis of general examination data, cold tests. Treatment includes physiotherapy, acupuncture, prescription of medications, protection of affected areas from exposure to low temperatures.
95.8 Other skin-restricted vasculitis
Livedo (translated from Latin. – “bruise”) – a mesh pattern of the skin due to local circulatory disorders. For the first time, this term for vascular pathology was used by the Austrian doctor, the founder of dermatology as a science, Franz Gebra in 1845. The synonymous name “skin asphyxia” is also found in the literature. As an independent pathology, livedo is diagnosed mainly in young and middle-aged female patients in areas of the trunk and limbs poorly protected by clothing from external influences. As a symptom of other diseases, livedo is registered in patients of both sexes and all ages, including children. Most often, the consequences of vasomotor disorders are observed in patients with antiphospholipid syndrome (in 20% of cases).
Skin asphyxia is a polyethological disease. For its development, a combined effect of several damaging factors is necessary. Angioedema is recognized as the leading factor – a disorder of the nervous regulation of vascular tone of the microcirculatory bed of the skin, sometimes subcutaneous tissue. Also , in the development of the pathological process , it may be important:
- Circulatory disorders. In the development of livedo, a general reduced venous outflow through the external veins of the extremities can play a role, which provokes the development or strengthening of the skin pattern in the cold season. The probability of developing pathology is higher in patients with a general decrease in blood flow rate in heart failure, arterial hypertension. Local microcirculation disorder, leading to the formation of morphological changes in the vessels of the microcirculatory bed, can be triggered by tissue compression, lack of movement (“standing” or “sedentary” work), fat, air and other embolism.
- Temperature effects. Both cold and intensive warming of certain areas of the body, for example, the use of a heating pad or diathermy procedures, can disrupt normal microcirculation in tissues. Prolonged or repeated episodes of exposure to low or high temperatures are important.
- Intoxication and side effects of drugs. Toxic substances, in particular, alcohol, can change the tone of blood vessels, the activity of nerve centers. Chronic alcohol poisoning leads to the formation of persistent manifestations of skin asphyxia. A number of medications, such as amantadine or dapsone, can provoke a change in capillary tone.
- Hormonal problems. Of paramount importance are disorders of the thyroid and genital glands: hyper- and hypothyroidism, menstrual cycle disorders. There are cases of marbling of the skin and other diseases of the endocrine system. Correction of hormone levels, as a rule, leads to the resolution of skin manifestations of vascular pathology.
- Infections. Livedo is a characteristic skin manifestation of a number of infectious diseases, such as tuberculosis, secondary, tertiary, congenital syphilis, dysentery, malaria. In foreign literature there are separate reports of the development of reticular asphyxia of the skin in leprosy.
- Autoimmune and other problems. The appearance of a vascular reticular or tree-like cyanotic pattern may be the first manifestation of cryoglobulinemia, Sneddon syndrome, cholesterol embolism syndrome, antifisfolipid syndrome and other pathology.
The development of skin manifestations is based on spastic-atonic capillary neurosis. A local violation of the regulation of the tone of small vessels leads to a spasm of arterial capillaries, as a result of which the venous ones overflow with blood. The long-term existence of an obstacle to normal microcirculation gradually leads to morphological changes. Protein masses accumulate in the walls of the venous knee of capillaries, which causes a significant thickening of the endothelium, narrowing of the lumen up to complete obliteration of blood vessels. Degenerative processes are progressing in the surrounding tissues: perivascular infiltration and fibrosis. Slowing of blood flow provokes the formation of multiple blood clots in the microvascular bed.
The degree of severity of morphological changes is largely determined by the form of livedo. The idiopathic form of reticular livedo is characterized by a minimal degree of fibrosis and thickening of the endothelium. Racemose livedo is characterized by a significant and persistent violation of microcirculation, which often leads to the formation of necrosis sites, the formation of ulcers. In the genesis of the ribbon-like livedo, an inflammatory reaction is also important, which is manifested by endophlebitis of the veins.
The most complete and easy to understand is the Livedo classification, built on the basis of morphological manifestations of pathology. It was created at the beginning of the XX century and has not lost its relevance to the present time. The classification takes into account the clinical forms of skin asphyxia and the conditions of its occurrence:
- Reticular (reticular) livedo. Transient or persistent deterioration of blood circulation in areas of the body exposed to temperature influences. At the same time, “marble skin” is a consequence of frequent local hypothermia, and “pigmented livedo” is the result of intense local heat exposure. In the second case, in addition to cyanotic, pigmented spots also appear.
- Racemose livedo. Persistent local circulatory disorder as a manifestation of general circulatory insufficiency, intoxication, a number of infectious and autoimmune diseases. The lesion of the entire skin develops several months after skull injuries simultaneously with neurological disorders and mental disorders.
- Ribbon-like livedo. The initial stage of Bazin’s indurative erythema, one of the forms of focal tuberculosis of the skin. Develops against the background of tuberculous lesions of internal organs. It is registered mainly in women. Predisposing factors for the development of skin lesions are local changes in microcirculation due to hypothermia, hypodynamia.
The reticular form is characterized by the appearance of pale or bluish single or multiple spots and loops of various sizes on the skin of the arms, legs, and rarely the lateral surfaces of the trunk. The spots form a grid of alternating cyanotic and normal areas on the affected areas.
There are physiological and idiopathic forms of mesh asphyxia. Physiological livedo is a variant of the norm, appears on the skin of the lower extremities in healthy people when cooling and disappears when warming. The idiopathic form develops in young patients in open areas of the body against the background of neurohumoral disorders (dysmenorrhea, hypothyroidism). Spots are unstable, they appear stronger when cooled, become less contrasting when warmed and pressed.
Marbling of the skin in idiopathic form may be the only manifestation of angioedema, but is more often combined with acrocyanosis, chills, keratinization disorder, hyperhidrosis. As the only manifestation, pale or cyanotic spots can exist for a number of years and then disappear on their own. In the presence of other symptoms of angioedema, improvement occurs only as a result of repeated courses of treatment.
The favorite localization of racemose livedo is the extensor surfaces of the extremities, buttocks. Clinically, it is manifested by tree-branching spots of bluish or burgundy-red color of various diameters. The spots do not peel off, do not disappear when pressed and warmed, acquire a more saturated color with local cooling. Cases of the development of racemous asphyxia in the immediate vicinity of the limb gangrene zone, the focus of ischemic tissue necrosis, and Martorella ulcers are described.
Ribbon – like livedo
The manifestations of Livedo lenticularis are located mainly on the shins. The reticulated marbling of the skin is formed by spots of bluish or burgundy color. Cyanotic spots do not exist for long, gradually being replaced by dense nodes with indistinct borders characteristic of indurative erythema.
A characteristic outcome of racemous livedo is the formation of necrosis foci in places of complete cessation of microcirculation. The development of complications occurs several years after the onset of the disease against the background of deterioration of the general condition of the patient.
The gradual closure of the capillary lumen leads to oxygen starvation of tissues, their necrotization and rejection with the formation of ulcers. This can be avoided with the full treatment of the underlying disease, regular courses of local therapy of vascular disorders. It is necessary to start treatment when the first signs of dystrophy appear, since the ulcers that have opened pose a threat of infection of tissues, complicate patient care, heal poorly in conditions of lack of oxygen and nutrients.
It is not difficult to diagnose skin asphyxia. To do this, a dermatologist needs a general examination and questioning of the patient. It is much more difficult to diagnose the underlying disease that caused the development of vascular disorders, to conduct differential diagnosis of livedo and other dermatological diseases. To make a correct diagnosis , you can be assigned:
- Temperature tests. The reaction of vascular spots to heat and cold allows us to determine the form of skin asphyxia, the degree of prescription of the existence of trophic disorders, their reversibility. Based on the data obtained, the dermatologist determines the treatment plan and predicts its effectiveness.
- Laboratory and instrumental studies. They are prescribed for the underlying disease, if the diagnosis was not made earlier. For the purpose of diagnosis, the patient can be referred to a specialist of a certain profile: an endocrinologist, a rheumatologist. Additional tests may also be needed if the skin manifestations of asphyxia need to be differentiated with roseoli in secondary syphilis (Wasserman reaction, PCR), toxidermy (basophil degranulation reaction, lymphocyte blast transformation).
Therapy of vascular pathology of the skin is carried out for a long time, repeated courses. A pronounced therapeutic effect is given by a combination of medications with physiotherapy procedures and reflexology. Livedo treatment is carried out according to a specific plan:
- Protection from the cold. The patient is recommended to choose clothes in such a way as not to expose the affected areas to excessive cooling. In the case of an idiopathic form of reticular livedo, this alone may be enough for vascular changes to reverse development.
- Medications. Mainly systemic drugs are used. These are vitamins C, PP, calcium, ascorutin and other means designed to strengthen the vascular wall. According to studies, the effect can be obtained from the use of the immunosuppressant azathioprine. Complex treatment of the underlying disease is mandatory.
- Physiotherapy. The priority is thermal procedures (baths, ozokeritotherapy), exposure to suberythemic doses of UV radiation, plasmapheresis, massage. Physiotherapy is prescribed for mesh and racemose livedo in cases where there are no contraindications to treatment for the underlying disease.
Prognosis and prevention
Methods of prevention of vascular skin disorders have not been developed. The prognosis is determined by the nature and severity of the underlying disease, the form of angioedema. The idiopathic form of mesh asphyxia is rather a cosmetic defect. Racemose livedo requires the attention of a doctor due to the high probability of ulceration. The occurrence of characteristic vascular loops and spots against the background of general health or after endovascular procedures requires a comprehensive examination of the patient due to the high probability of a serious pathology occurring covertly, without pronounced clinical manifestations.