Porphyria

The existence of porphyrin substance and the violation of its metabolism were discovered more than 100 years ago. N. Guntcr (1901) called diseases that occur with a disruption of porphyrin metabolism, "hemoporphyria", and J. Waldenstrom (1937) by the term "porphyria".

Porphyrins (Greek porphyreis - dark red) are organic compounds belonging to the group of tetrapyrenes. In the human body, the dark part of hemoglobin is synthesized from the porphyrins, and chlorophyll in the plants. Hemoglobin in the body consists of protein globin and protein-free heme. In its pure form, porphyrins are crystals of red color. This color depends on the double bond in the pyrrole ring and the methyl group. In the body porphyrins perform the functions of biological oxidation, transportation of oxygen and other important functions. Exogenous porphyrins, along with meat and plant products, enter the body, are absorbed through the blood, penetrate the liver and are transformed into coproporphyrins. The main part of coproporphyrins, together with bile, is excreted into the intestine, and the rest enters the bloodstream and through the kidneys is excreted outward along with the urine.

Endogenous free porphyrins are formed as a result of the decomposition of the heme and myoglobins. All porphyrins appear due to a chemical change in ethylporphyrin in the body. Porphyrin IX, being one of the main porphyrins in the body, connecting with iron, forms heme. In the bone marrow, 250-300 mg of porphyrin is synthesized overnight, and it is used in heme synthesis. The greatest amount of free porphyrins (50 mg) penetrates into the erythrocytes. In the pathological state, the amount of porphyrins inside the erythrocytes increases by 10-15 times. As a result of a violation of the functional state of the liver, the conversion of porphyrin to bile acid and detoxification is reduced. This leads to an increase in the content of porphyrins. Due to the destruction of hemoglobin in the intestine can also form porphyrins. However, under the influence of bacteria, porphyrin is converted into deuterporphyrins IX (III) and lysoporphyrin IX (III). The weight of the compound having the porphyrin ring absorbs rays with a wavelength of 400 nm. All porphyrins fluoresce, giving off red rays.

Under the influence of solar insolation, erythrocytes can undergo hemolysis and porphyrins are formed. This process occurs with an increase in the content of histamine, which increases the sensitivity of the body to the sun's rays. Because of the ability of porphyrins to cause spasm of blood vessels in this disease, there are abdominal pains, constipation, oliguria.

With a symptomatic complex of rickets, hypokalemia, hypotension, depressive states, a decrease in the content of porphyrins is observed.

Depending on where the synthesis of porphyrins occurs, the erythropoietic and hepatic forms of porphyria are distinguished. Congenital porphyria of Gunther, erythropoietic protoporphyria, erythropoietic coproporphyria constitute a group of erythropoietic porphyria. The group of hepatic porphyria includes acute, or pyrroloporphyria (manifest, latent forms); varygatnaya, or protoprofiriya (flowing with a rash on the skin, dermal, latent forms - without rashes), late cutaneous porphyria (urokaporfiriya) and hereditary coproporphyria.

[1], [2]

Causes of porphyria

In the etiology and pathogenesis of late cutaneous porphyria, an important role is played by ethylated gasoline, lead, heavy metal poisoning, alcoholism, long-term use of esterogen, barbiturate, griseofulvin, severe hepatitis, etc.

As a result of studies in patients with porphyria, elevated levels of iron in the blood serum and liver parenchyma, sidereous Kupffer cells and liver damage of various degrees were revealed.

In the pathogenesis of the disease, participation in the progress of peroxidation of lipids has been demonstrated. Under the influence of ultraviolet rays, the process of peroxide oxidation of lipids is enhanced. As a result, oppression of singlet and triplet oxygen activity, superoxide dismutase, catalase, percosidase, glutathione reductase, decrease in the content of alpha-tocopherol and sulfhydryl groups is noted. Owing to the intensification of the lipoperoxidation process, an increase in the amount of malonic dialdehyde and iron ions located in the membranes is noted. As a result, the lonidae undergo destruction. In the erythrocyte membrane in patients with late cutaneous porphyria, the content of the fraction of easily oxidized phospholipids decreases, and the content of hardly oxidizable phospholipids increases. To reduce the content of increased amounts of lysophosphatidylcholine, the reactions of transmutase and phospholipase are involved. The course of these reactions in the body is confirmed by an increase in the content of phospholipases A and C. As a result, the shape of the cell membrane changes, and sometimes the cell collapses, the hydrolase enzymes go outside and the pathological process (inflammation) develops. In the emergence of porphyrin disease, the importance of transferred hepatitis A, B and C.

As shown by scientific research carried out in recent years, late cutaneous porphyria has a hereditary nature, and this disease is most often found in people who carry antigen HLA A3 and HLA B7. In the onset of the disease, the deficiency of the enzyme uroporphyrinogen dekorboksilazy has an important pathogenetic significance.

[3], [4], [5], [6], [7], [8]

Symptoms of porphyria

Porphyria is most often found in men, as they are more likely to smoke and drink alcohol than women. The disease is characterized by the formation of traumatic or actinic blisters in the skin, an excessive increase in the content of uroporphyrins in the urine as compared with the norm, a slight increase in the content of coproporphyrins, our liver in various kinds of functional and organic changes. The disease mainly begins in the spring and summer months, when increased solar radiation.

Clinical signs of dermatosis are manifested in the open areas of the body (face, neck, hands) in the form of pigmentation, vesicles (or blisters), hypertrichosis, microcyst and rapid skin damage.

The disease is characterized by the precipitation of blisters on healthy or hyperpigmented skin, exposed to sunlight or often getting damaged. Bubbles are round or ovoid, small in diameter 15-20 cm, inside they contain yellowish or serous fluid (when infection is attached). Bubbles are not inclined to merge with each other, on the skin around there are no signs of inflammation. As a result of slight damage, the blisters quickly burst, erosion or superficial ulcers appear in their place.

With late cutaneous porphyria, one can observe a positive syndrome of Nikolsky in 1/3 of patients. In the place of erosion or superficial ulcers after 10-15 days there are pigment spots, pink-bluish scars, and sometimes acne resembling a milium. Sometimes pigmentation on the skin is the only clinical sign, but this pigmentation often occurs along with other symptoms. Pigmentation is dirty gray, reddish-brown or bronze. On the skin of patients with prolonged late cutaneous porphyria, along with pigmented spots, spots similar to white spots in vitiligo or achromic spots of pseudopyokylodermic type can be found. Sometimes, as a result of slight damage or trauma (when removing the ring, wiping the hands with a towel, etc.), erosions or escorts appear on the open parts of the body (most often the lateral surface of the hands). In the temporal areas of the head, hypertrichosis is seen, that is, eyelashes and eyebrows grow rapidly, their color darkens. During the period of clinical remission of the disease, these signs disappear. On the hands and on the outside of the fingers, face, and the ears of the patients, for a long time suffering from porphyria, one can find microcysts, similar to the milium. The color of such elements is whitish, 2-3 cm in diameter, they are arranged in groups, resembling whiteheads in appearance.

With late cutaneous porphyria on the fingers can be pathological changes. Under the nails, hyperkeratosis is observed, they are deformed and destroyed (photoionicholysis).

There are simple (benign) and dystrophic forms of late cutaneous porphyria.

With simple porphyria, bubbles characteristic of porphyria are noted in the summer months, which persist for a short time. Erosions are epithelized in a short time. The disease recurs once every year, it proceeds easily. Appearance and general condition of patients almost do not change.

In dystrophic form, the disease continues until late autumn and the blisters persist for a long time. The upper layers of the dermis are affected, deep erosion and ulcers appear. Subsequently, atrophic scars appear on the site of ulcers, and on the site of erosion are milymatic cysts. Often pathological foci are complicated by a secondary infection, nails fall out and undergo destruction. In patients, various changes can be detected (hyperkeratosis in open areas of the body, hypertrichosis, scleroderma-like skin).

In addition to the above described classical clinical manifestation of late cutaneous porphyria, there can also occur atypical forms such as scleroderma, sclerovitilinosis, sclerolychinoid, porphyria-melanoderma, infiltrative-plaque porphyria, or porphyria such as lupus erythematosus and erosive cheilitis. In the structure of dermatosis, atypical forms are 8-9%.

The sclerodermatoid form of dermatosis occurs frequently and is manifested by the following clinical signs:

• the rash occurs in areas of the body exposed to sunlight (face, neck, foot - sclerodactyly) and sometimes there is a process of mutation;
• along with foci of dyschromia (the sequence of the appearance of hyperpigmented foci), one can observe the condensed areas of skin characteristic of scleroderma. In the future, skin atrophy develops;
• as with skin scleroderma, the foci are of a yellow-gray or pale yellowish color;
• the disease recurs in the spring-summer months, in the main foci there are bubbles;
• the vulnerability of the skin in the open areas of the body and the absence of skin in the closed parts of the body. This condition is not characteristic for scleroderma;
• increased synthesis of collagen fibers of fibroblasts under the influence of uroporphyrins, which explains the development of the scleroderm-like form of porphyria;
• in 3% of patients, there is a late skin porphyria, similar in shape to vitiligo. This is characterized by the appearance of large depigmeptic spots in the place of the blisters. Sometimes, in the vitiligo form of the disease, the skin hardens, thickens, and is called the sclerovitiligous form of late cutaneous porphyria. Scleroderm-like and vitiliginous forms develop during the period of severe dermatosis. The pathological process appears on the skin of the forehead, the temporal part of the head. In the foci there are pigment-free and hyperpigmented spots, in the following, skin atrophy develops here.

A case of the simultaneous appearance of sclerovitilinous and scleroderm-like forms of late cutaneous porphyria in the same patient is described.

In the atypical form of dermatosis in the form of red lupus, the spots in the outbreaks resemble a "butterfly", within a few days they disappear and after themselves do not leave atrophy. Around the erythematous foci on the face there is an infiltrative pad, and in the center - slowly developing atrophy of the skin. However, when examining the skin of patients with this form, no signs characteristic of lupus erythematosus are found. A case of simultaneous appearance of lupus erythematosus and late cutaneous porphyria is described.

Atypical form of porphyria, which proceeds in the form of erosive cheilitis, is observed in 10.7% of patients, with swelling of the lower border of the lip, peeling and erosion.

With late skin porphyria in the internal organs, in the nervous and cardiovascular systems, various changes occur. Patients complain of pain in the heart, palpitation (tachycardia), headaches, pain in the left side, rising or lowering of blood pressure. With a careful examination of the patients, the enlargement of the heart borders is observed, the accent of the 2nd tone over the aorta is increased, the automatism, excitability, patency are violated, the contractile functional myocardial heart functions change. Dystrophic changes taking place in the heart, occur due to the action of harmful substances formed due to metabolic disorders of porphyrins.

In the eyes of all patients, specific changes (varying of blood vessels, conjunctivitis, pigmentation of the sclera and disc of the optic nerve, appearance of blisters in the cornea, disseminated choroiditis and other dystrophies) are revealed in varying degrees of development.

With late cutaneous porphyria, changes in the liver (specific porphyrin hepatitis) are secondary as a result of the action of pathological metabolites of porphyrin on the liver parenchyma. According to some scientists, in the initial period, develops precursorosis, and in the future - cirrhosis of the liver. Pathological changes in the liver indicate a metabolic disorder in patients. Violation of protein metabolism is noted in the form of a decrease in albumins and albumin-globulin coefficient, an increase in gamma globulins. When palpation of the right hypochondrium and epigastric region, the consistency of the liver is hard, the liver is enlarged, painful, the pigmentation of the skin becomes worse, the small capillaries in the chest are dilated.

Late skin porphyria can appear along with psoriasis, lupus erythematosus, scleroderma, other skin diseases, as well as liver, stomach, lung sarcoidosis, hemochromatosis and myeloma.

Histopathology

Under the epidermis, one can see the presence of a hole or bubbles. The dilated epidermis forms the lid of the bladder, the papillary layer of the dermis is its bottom. In the spine-shaped layer of the epidermis, there are even acanthosis, underdeveloped spongiosis, papillomatosis of the papilla of the dermis, damage to the vascular endothelium, degeneration of collagens, thinning of cellular fibers and their fragmentation. In the fluid of the bladder, cellular elements are not detected, sometimes it is possible to meet leukocytes.

Differential diagnostics

Late skin porphyria should be distinguished from bullous epidermolysis, vulgar pemphigus, herpetiform dermatitis Dühring, pellagra.

[9], [10], [11]

Congenital erythropoietic porphyria (Lynter's disease)

The clinical signs of this disease in 1911 were first described by H. Gunter. Congenital erythropoietic porphyria is found in representatives of all nations, in all European and African countries, in Japan and the United States. In India, the most widespread disease.

As research shows, congenital porphyria is found among brothers and sisters who belong to the same generation. Cases of porphyrin metabolism in children born from patients with congenital erythropoietic porphyria are described. Transmission of dermatosis by autosomal recessive pathway was revealed. Patients with congenital erythropoietic porphyria in relation to the pathological gene are homozygotes, and close relatives are heterozygotes.

When using the methods of luminescent microscopy and radioisotopes, the presence of normal and pathological erythroblasts in the bone marrow of patients with congenital erythropoietic porphyria was revealed. Inside the pathological erythroblasts, porphyrins are produced more than normal and after the destruction of red blood cells these substances enter the blood serum, and then accumulate in the tissues. It has been proved that inside the erythrocytes the so-called enzymes porphobilinogen-deaminase (urocorpyrin I synthesis) and uroporphyrinogen isomerase (uroporphyrin III) control the heme synthesis.

Due to hereditary deficiency (deficiency) of the enzyme uroporphyrinogen III cosyntase located in the pathological erythroblasts of the patient, the heme biosynthesis is disrupted and the content of uroporphyrinogen I increases in the patient's body.

Congenital erythropoietic porphyria develops with the birth of a child or in the first year of his life. Sometimes the initial signs of the disease can occur at the age of 3-4 years and older. The disease occurs in men and women alike. Red color of urine is the initial sign of the disease.

Dermatosis begins mainly in the spring and summer months. In open areas of the body, where the sun's rays fall, bubbles appear, accompanied by itching. Bubbles contain serous or serous-hemorrhagic fluid. Bubbles can also occur under the influence of various mechanical factors. As a result of secondary infection, blisters and erosions become ulcers and in their place (most often on the extensor part of the hands) scars form. As a result of a long and chronic course of the disease, deep tissue is involved in the pathological process and ear mutilation is observed. Stop. Nails are exposed to dystrophy, thicken, deform and fall out. Radiography of the osteoarticular system reveals osteoporosis, complete or partial contracture of ligaments. Change in the patient's eyes is expressed by conjunctivitis, clouding of the cornea and eye pupils. The color of the rash depends on the accumulation of porphyrins on the enamel and dentine, the whole surface of the teeth can be pink, pink-yellow or dark red. Teeth exposed to ultraviolet rays have a dark red luster. On the face skin hypertrichosis is observed! Eyebrows and eyelids.

You can observe pink-red fluorescence in the gums and teeth in some healthy children. This fluorescence is caused by the action of porphyrins, released by bacteria that live in the mouth.

Congenital erythropoietic porphyria is characterized by an enlarged spleen, which can weigh 1.5 kg. At the same time, there are observed poikilocytosis, anisocytosis, spherocytosis, thrombocytopenia, and others.

Previously, the prognosis of congenital erythropoietic porphyria was unfavorable, patients under the age of 30 died from various intercurrent diseases and hemolytic anemia. Currently, the prognosis of the disease is favorable, however, the patients do not fully recover.

In the daily urine of patients, compared with the norm, uroporphyrins increase several hundred fold, amounting to 140-160 mg, and coproporphyrins - 30-52 mg. Such high indices in urine, unlike the hepatic form of porphyrins, are peculiar only to congenital erythropoietic porphyria.

Histopathologically, the number of melanocytes increases in the basal layer of the epidermis, and the number of fibers in the dermis decreases, proliferation of fibroblasts is observed, infiltration consisting of lymphocytes is detected around the blood vessels, fat and sweat glands. In the basal layer of the epidermis and the papillary layer, the location of the porphyrins, the walls of the superficial blood vessels and the positive symptom of Schick and diastase, the resistant glucocopolysaccharide and immunoglobulins are revealed.

In the treatment of congenital erythropoietic porphyria, patients are recommended protection from sunlight, beta-carotene preparations, antiplatelet agents. Sometimes splenectomy gives a good result.

[12]

Erythropoietic protoporphyria

Erythropoietic protoporphyria was first described in 1953-54. W. Kosenow and L. Treids. The authors drew attention to the increased content of protoporphyrins in feces, the signs of photosensitization in two infants and the fluorescence of erythrocytes in the blood, and called this disease protoporphyrinemic photodermatosis. After a complete study of the metabolism in this disease in 1961, L. Magnus introduced him into the porphyria group. Erythropoietic porphyria is a hereditary disease and is inherited by autosomal dominant type.

Dermatosis, in general, is found among the population of Europe and Asia and Europeans living on the African continent. In erythropoiesis porphyria, the ferrochelatase enzyme deficiency in erythrocytes and erythroblasts weakens the transformation of protoiorphyrin into heme, and the content of this metabolite in erythrocytes and erythroblasts increases sharply. Patients are especially sensitive to rays of wavelength more than 400 nm. The liver plays an important role in the development of erythropoietic porphyria. As in the phytoblasts, in the liver, lrotoporphyrins are synthesized in the pathological direction and accumulate in the liver cells, as a result poorly soluble protoporphyrins are delayed and exert a toxic effect on the liver. The large porphyrins in the blood plasma then enter the dermis, photodynamic reactions develop, the cells and cell organelles are affected, lysosomal and cytolytic enzymes are released from them, which damage tissues and cells. Thus, the skin shows clinical signs characteristic of phytophrotic porphyria. The time from the onset of sunlight to the development of clinical symptoms of the disease depends on the strength of the active rays and the concentration of porphyrins in the tissue being excited.

A biochemical examination of close relatives of patients with erythropoietic protoporphyria revealed dermatosis, which proceeds in a latent form.

When the latent form of erythropoietic protoporphyria is diagnosed, the relative coefficient of protopro and co-proporphyrins in feces is of great importance.

Erythropoietic protoporphyria most often occurs in men and is characterized by a chronic recurrent course.

Unlike other porphyria, patients with erythropoietic protoporphyria are very sensitive to sunlight. Even from weak rays penetrating through the window glass, after 2-3 hours on the skin diffuse edema and erythema are observed.

The pathological process proceeds with such subjective symptoms as itching, pain, tingling. Bubbles appear on the skin.

Clinical signs of the disease are not limited only to erythema and edema, subsequently there are purpura, blisters. In severe disease, deep excoriation appears, and the clinical picture resembles pellagroide dermatitis. Erythropoietic porphyria does not have specific clinical signs and is very similar in clinical course to such photodermatoses as urticaria caused by sunlight, prurigo-eczematous photodermatosis, and Bazin's smallpox.

Almost all patients have skin that surrounds the eyes, mouth, upper part of the nose and hands is rough, thickened, the skin pattern is expressed. In spring and summer months, in some patients, hyperkeratosis and cracks on the red border of the lips, limited light brown spots and superficial atrophic scars of oval form can be found.

In the erythrocytes of almost all patients, the content of protoporphyrin is sharply increased. An increase in uroporphyrins is rare. There is evidence of an increased content of protoporphyrins in the blood serum, an increase in the content of co-proporphyrins in some patients, and inability to determine the content of uroporphyrins (or small amounts). In the diagnosis of the disease, a large role is played by the ratio of proto- and co-proporphyrins.

Histopathologically in the acute period of the disease, changes in the skin are characterized by signs of acute inflammation. In the upper layers of the dermis around the vessels are located hyaline-like substances, which manifest a positive symptom of Schick.

[13]

Erythropoietic coproporphyria

Erythropoietic coproporphyria is less common and inherited by autosomal dominant type. The disease is based on an increase in the content of coproporphyrins in erythrocytes. Dermatosis is characterized by the manifestation of signs of photosensitization, and due to the similarity of the clinical picture of the disease with erythropoietic protoporphyria, it is very difficult to distinguish them from each other.

Erythropoietic porphyria should be distinguished from other forms of porphyria, skin atrophy.

In the treatment of erythropoietic proto- and coproporphyria, it is recommended to take 60-180 mg of beta-carotene every day on sunny days (or months). The effectiveness of treatment begins to appear after 1 and 3 days. Correction of pathological changes in the liver is a difficult task. For this purpose it is advisable to consult a hepatologist, gastroenterologist, transfusion of erythrocyte mass, cholesterolamine, hematin and other hepatotropic drugs.

[14], [15], [16], [17], [18]

Mixed porphyria

Mixed porphyria belongs to the group of congenital hepatic porphyria, transmitted by the dominant type.

Causes and pathogenesis. At the heart of the disease is the lack of enzyme protoporphyrinogen oxidase, as a result of which protoporphyrinogen is unable to turn into protoporphyrin. During an attack, the content of aminolevulanic acid rises sharply. With mixed porphyria, there is evidence of a decrease in the activity of ferrodelatases that are part of normoblasts.

Abdominal and neurological signs of the disease can mainly be caused by drugs (barbiturates, sulfonamides, analgesics, antipyretic agents, etc.), alcoholic beverages and other hepatotoxic agents. Viral hepatitis, pregnancy, a small amount of carbohydrates consumed in food, have a certain significance in the onset of the disease.

Symptoms

The disease is mainly found among white people from 20 to 30 years old, living in South Africa. Cutaneous manifestations of the disease are very similar to late cutaneous porphyria (photosensitivity on the exposed part of the skin, blisters, erosion, scars). Besides; Moreover, there are mental disorders, a violation of the functional activity of the central and peripheral nervous system and abdominal pain. The clinical signs described above do not always manifest simultaneously. When examining 113 patients with mixed porphyria, 50% of them had acute attacks and a rash on the skin, 3.4% had only a rash on the skin and 15% had only seizures. According to some authors, mixed porphyria in England and Finland is easy in comparison with South Africa, a rash in the skin is the initial sign of the disease.

In feces the content of proto- and coproporphyria is sharply increased. During an attack in the urine, porphobilinogen, aminolevulin and X-porphyrins are detected.

Histopathology

Pathomorphological changes on the skin do not differ from late cutaneous porphyria.

Treatment

Conduct symptomatic treatment. When the attack is prescribed, glucose, adenosine monophosphate, riboxin, and when severe; the course of the disease - hematin. It is advisable to use antioxidants.

Hepatoerythropoietic porphyria

The causes and pathogenesis of hepatoerythropoietic porphyria have not been fully studied. There is evidence that the gene is the only one that causes hepatoerythropoietic porphyria and a mixed form of late cutaneous porphyria, the activity of protoporphyripogen-decarboxylase is inhibited.

In erythrocyte and serum, the maintenance of protoporphyrins increases, in urine - uroporphyrin, and in feces - kopproporphyrins.

Symptoms

The disease begins with the moment of birth or from an early age. Hepato-erythropoietic porphyria includes such clinical signs of diseases as late skin porphyria, congenital erythropoietic porphyria.

Histopathology

Under the epidermis there is a bubble, in the dermis - hemogenization of collagen fibers, thickening of the walls of the vessels, and around them - the accumulation of hyaline.

Differential diagnosis

The disease should be distinguished from congenital elidermolysis, Bazin's smallpox, arising from the rays, and other forms of porphyria.

Treatment

The drugs used in the treatment of congenital erythropoietic porphyria are used.

[19], [20], [21], [22], [23], [24]

Hereditary coproporphyria

Causes and pathogenesis of hereditary coproporphyria: occurs due to a lack of enzyme coproporphyrinogenase.

Symptoms

According to the clinical manifestation, the disease, being close to the mixed porphyria, proceeds easily. Pain in the intestine is more common. Neurological and psychological changes are less common. In view of the fact that coproporphyrins, in comparison with uroporphyrinogen, have a lower phototoxic ability, they accumulate little on the skin. Changes in the skin occur in only 1/3 of the patients. Bubbles are formed at the site of the injury, and the clinic resembles the late cutaneous porphyria.

In the feces of the patient sharply increased the content of coproporphyrin III. Sometimes this substance can be found in the urine.

Histopathology

Pathomorphological changes on the skin do not differ from the late cutaneous porphyria.

Treatment

The same measures are applied as in the treatment of mixed porphyria.

Treatment of porphyria

Specific treatment of late cutaneous porphyria is absent. During the period of medical procedures, it is necessary to normalize the disturbed metabolism in the body, remove an increased number of circulating porphyrins from the body. In therapeutic doses, it is advisable to use vitamins of group B (B1, B6, B12), nicotinic acid. Vitamins are recommended every other day, vitamins B1 and B6 can not be administered simultaneously in one day. Along with this, folic acid (0.01 g 3 times a day), riboflavin (3 times 0,005 g), ascorbic acid (3 times a day for 0.1 g), Aevit (2-3 times 1 capsule ), methionine (0.5-0.75 g per day), sireppar (intramuscularly 2-3 ml, 50-60 injections per 1 course), etc.

With late cutaneous porphyria there is no consensus on the use of antimalarial (antiplatelet) drugs. Some dermatologists consider it impossible to use delagil or other antiplatelet drugs in late cutaneous porphyria, as these drugs often cause retinopathy, agranulocytosis, vomiting, toxic psychosis, depigmentation of hair and other negative consequences. Along with this, other scientists suggest using antiplatelet drugs in small doses (125 mg of chloroquine twice a week for 8-18 months). According to scientists, anti-fever drugs form soluble complexes with porphyries in water and they are easily excreted from the body along with urine. It is advisable to recommend anti-fever drugs after taking vitamin therapy, after 15-20 days.

In order to inhibit lipid peroxidation, antioxidant drugs, beta-carotene, alpha-tocopherol (100 mg once a day) are prescribed.

In severe cases during 2 weeks prednisolone 5 mg twice a day, as well as B vitamins, ascorbic acid, calcium chloride (10% solution in a tablespoon 3 times a day) are prescribed.

To protect from sunlight it is recommended to use photoprotective agents. Patients are forbidden to prescribe the following drugs: sulfonamides, griseofulvin, barbiturates (barbitals, thiopental, phenobarbital, etc.).

Dietary meals are recommended. In the diet should not be fatty meat (lamb or pork), fried fish, rich soups.

Erythropoietic porphyria is characterized by the following features:

• the disease mainly begins in childhood;
• the cause of the disease is genetic (hereditary) fermentopathy;
• before the development of the clinic, the provoking factors have no effect;
• the disturbance of porphyrin metabolism occurs in the erythroblasts of the bone marrow;
• with luminescent microscopy of erythrocytes and erythroblasts present in the peripheral blood, one can observe the red fluorescence (luminescence) characteristic of porphyrins;
• in erythrocytes in peripheral blood, the content of porphyrins is always high.

There are congenital erythropoietic porphyria, erythropoietic proto- and coproporphyria.