Porphyria

The existence of the substance porphyrin and the disorder of its metabolism were discovered more than 100 years ago. H. Guntcr (1901) called diseases occurring with the disorder of porphyrin metabolism "hemoporphyria", and J. Waldenstrom (1937) the term "porphyria".

Porphyrins (Greek porphyreis - dark red) are organic compounds belonging to the tetrapyrol group. In the human body, the dark part of hemoglobin is synthesized from porphyrins, and in plants - chlorophylls. Hemoglobin in the body consists of the protein globin and protein-free heme. In pure form, porphyrins are red crystals. 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, oxygen transportation and other important functions. Exogenous porphyrins enter the body together with meat and plant products, are absorbed through the blood, penetrate the liver and turn into coproporphyrins. The main part of coproporphyrins is excreted into the intestines with bile, and the rest enters the blood and is excreted through the kidneys with urine.

Endogenous free porphyrins are formed as a result of the breakdown of hemes and myoglobins. All porphyrins appear due to the chemical change of ethylporphyrin in the body. Porphyrin IX, being one of the main porphyrins in the body, combines with iron to form heme. In the bone marrow, 250-300 mg of porphyrin is synthesized per day, and it is used in the synthesis of heme. The largest amount of free porphyrins (50 mg) penetrates into erythrocytes. In a pathological state, the amount of porphyrins inside erythrocytes increases by 10-15 times. As a result of a violation of the functional state of the liver, the conversion of porphyrin into bile acid and its neutralization decreases. This leads to an increase in the content of porphyrins. Due to the destruction of hemoglobin in the intestine, porphyrins can also be formed. However, under the influence of bacteria, porphyrin is converted into deuterporphyrins IX (III) and lysoporphyrin IX (III). All compounds with a porphyrin ring absorb rays with a wavelength of 400 nm. All porphyrins fluoresce, emitting 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, as a result of which the body's sensitivity to sunlight increases. Due to the ability of porphyrins to cause spasm of blood vessels, abdominal pain, constipation, and oliguria are observed with this disease.

In the symptom complex of rickets, hypokalemia, hypotension, and depressive states, a decrease in the content of porphyrins is observed.

Depending on where the porphyrins are synthesized, erythropoietic and hepatic forms of porphyria are distinguished. Congenital Gunther's porphyria, erythropoietic protoporphyria, erythropoietic coproporphyria make up the group of erythropoietic porphyrias. The group of hepatic porphyrias includes acutely changing, or pyrroloporphyria (manifest, latent forms); variegated, or protocoprophyria (occurring with a rash on the skin, cutaneous, latent forms - without rashes), late cutaneous porphyria (urocaporphyria) and hereditary coproporphyrias.

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Causes of Porphyria

Ethylated gasoline, lead, poisoning with heavy metal salts, alcoholism, long-term use of estrogen, barbiturate, griseofulvin, severe forms of hepatitis, etc. play a major role in the etiology and pathogenesis of late cutaneous porphyria.

As a result of studies, patients with porphyria were found to have elevated iron levels in the blood serum and liver parenchyma, Kupffer cell siderosis, and liver damage of varying degrees.

The progress of lipid peroxidation has been proven to be involved in the pathogenesis of the disease. Under the influence of ultraviolet rays, the process of lipid peroxidation intensifies. As a result, there is inhibition of the activity of singlet and triplet oxygen, superoxide dismutase, catalase, peroxidase, glutathione reductase, a decrease in the content of alpha-tocopherol and sulfhydryl groups. Due to the intensification of the lipid peroxidation process, there is an increase in the amount of malonic dialdehyde and iron ions located in membranes. As a result, linides are destroyed. In the membrane of the erythrocyte in patients with late cutaneous porphyria, the content of the fraction of easily oxidized phospholipids decreases, and the content of difficultly oxidized phospholipids increases. To reduce the content of the increased amount of lysophosphatidylcholine, transmutase and phospholipase reactions 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 is destroyed, hydrolase enzymes come out and a pathological process (inflammation) develops. In the development of porphyrin disease, the significance of previous hepatitis A, B and C is great.

As scientific research conducted in recent years shows, late cutaneous porphyria has a hereditary nature, and this disease is most often found in people who are carriers of the HLA A3 and HLA B7 antigen. In the development of the disease, the deficiency of the enzyme uroporphyrinogen decorboxylase has an important pathogenetic significance.

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Symptoms of Porphyria

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

Clinical signs of dermatosis appear on exposed areas of the body (face, neck, hands) in the form of pigmentation, vesicles (or blisters), hypertrichosis, microcysts and rapid vulnerability of the skin.

The disease is characterized by the appearance of blisters on healthy or hyperpigmented skin that is exposed to sunlight or frequently damaged. The blisters are round or ovoid, few in number, 15-20 cm in diameter, and contain yellowish or serous fluid (if an infection occurs). The blisters do not tend to merge with each other, and there are no signs of inflammation on the surrounding skin. As a result of minor damage, the blisters quickly burst, leaving erosion or superficial ulcers in their place.

In late cutaneous porphyria, positive Nikolsky syndrome can be observed in 1/3 of patients. At the site of erosion or superficial ulcers, after 10-15 days, pigment spots, pink-bluish scars, and sometimes acne similar to milium appear. Sometimes pigmentation on the skin is the only clinical sign, but this pigmentation most often occurs together with other symptoms. Pigmentation can be dirty gray, reddish-brown or bronze. On the skin of patients with long-term late cutaneous porphyria, along with pigment spots, one can see spots similar to white spots in vitiligo or achromic spots of the pseudopoikilodermic type. Sometimes, as a result of minor damage or trauma (when removing a ring, wiping hands with a towel, etc.), erosions or escoriations appear on open areas of the body (most often the lateral surface of the hands). Hypertrichosis is visible on the temporal areas of the head, i.e. eyelashes and eyebrows grow quickly, their color darkens. During the period of clinical remission of the disease, these signs disappear. On the hands and the outer side of the fingers, face, ears of patients who have suffered from porphyria for a long time, one can find microcysts similar to milium. The color of such elements is whitish, the diameter is 2-3 cm, they are located in groups, in appearance they resemble whiteheads.

In late cutaneous porphyria, pathological changes may occur on the fingers. Hyperkeratosis is observed under the nails, they are deformed and destroyed (photoonycholysis).

A distinction is made between simple (benign) and dystrophic forms of late cutaneous porphyria.

In simple porphyria, blisters typical of porphyria are observed in the summer months, which do not last long. Erosions epithelialize in a short time. The disease recurs once a year and is mild. The appearance and general condition of patients hardly change.

In the 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 at the site of the ulcers, and milium-like cysts at the site of the erosion. Pathological foci are often complicated by secondary infection, nails fall out and are subject to destruction. Various changes can be detected in patients (hyperkeratosis on exposed areas of the body, hypertrichosis, scleroderma-like skin).

In addition to the above-described classical clinical manifestation of late cutaneous porphyria, atypical forms such as scleroderma-like, sclerovitiliginous, sclerolichinoid, porphyria-melanoderma, infiltrative-plaque porphyria or porphyria of the lupus erythematosus and erosive cheilitis type may also be encountered. Atypical forms account for 8-9% of the dermatosis structure.

Scleroderma-like form of dermatosis is common and is manifested by the following clinical signs:

• the rash occurs on areas of the body exposed to sunlight (face, neck, feet - sclerodactyly) and sometimes a mutilation process is observed;
• Along with foci of dyschromia (the sequence of appearance of hyperpigmented foci), one can observe thickened areas of skin, characteristic of scleroderma. Subsequently, skin atrophy develops;
• As with cutaneous scleroderma, the lesions are yellow-gray or pale yellowish in color;
• the disease recurs in the spring and summer months, with blisters appearing in the main lesions;
• skin fragility on exposed areas of the body and lack of it on closed areas of the body. This condition is not typical for scleroderma;
• increased synthesis of collagen fibers by fibroblasts under the influence of uroporphyrins, which explains the development of the scleroderma-like form of porphyria;
• 3% of patients have late cutaneous porphyria, similar in form to vitiligo. It is characterized by the appearance of large depigmented spots at the site of blisters. Sometimes, with the vitiliginous form of the disease, the skin hardens, thickens, and it is called the sclerovitiligiform form of late cutaneous porphyria. Scleroderma-like and vitiliginous forms develop during the period of severe dermatosis. The pathological process appears on the skin of the forehead, temporal part of the head. In the foci, non-pigmented and hyperpigmented spots are observed, subsequently, skin atrophy develops here.

A case of simultaneous occurrence of sclerovitiliginous and scleroderma-like forms of porphyria cutanea tarda in the same patient is described.

In the atypical form of dermatosis in the form of lupus erythematosus, the spots in the foci resemble a "butterfly", they disappear within a few days and do not leave behind atrophy. Around the erythematous foci on the face, an infiltrative pad is observed, and in the center - slowly developing skin atrophy. However, when examining the skin of patients with this form, signs characteristic of lupus erythematosus are not found. A case of simultaneous occurrence of lupus erythematosus and late cutaneous porphyria is described.

An atypical form of porphyria, occurring as erosive cheilitis, is observed in 10.7% of patients, with swelling of the lower border of the lip, peeling and the appearance of erosion.

In late cutaneous porphyria, various changes occur in the internal organs, nervous and cardiovascular systems. Patients complain of pain in the heart, palpitations (tachycardia), headaches, pain in the left side, increased or decreased blood pressure. Upon careful examination of patients, dilation of the heart borders, increased emphasis of the second tone over the aorta are observed, automatism, excitability, patency are impaired, and the contractile functional characteristics of the cardiac myocardium change. Dystrophic changes occurring in the heart occur due to the action of harmful substances formed as a result of a violation of the metabolism of porphyrins.

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

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

Porphyria cutanea tarda may occur with psoriasis, lupus erythematosus, scleroderma, other skin diseases, as well as with liver cancer, stomach cancer, pulmonary sarcoidosis, hemochromocytosis, and myeloma.

Histopathology

Under the epidermis, the presence of a hole or bubbles can be seen. The thinned epidermis forms the lid of the bubble, the papillary layer of the dermis is its bottom. In the spinous layer of the epidermis, smooth acanthosis, underdeveloped spongiosis, papillomatosis of the dermal papillae, damage to the vascular endothelium, degeneration of collagens, thinning of cellular fibers and their fragmentation are observed. In the fluid of the bubble, cellular elements are not detected, sometimes leukocytes can be found.

Differential diagnostics

Late cutaneous porphyria should be distinguished from bullous epidermolysis, vulgar pemphigus, Duhring's dermatitis herpetiformis, and pellagra.

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Congenital erythropoietic porphyria (Linter's disease)

The clinical signs of this disease were first described by H. Gunter in 1911. Congenital erythropoietic porphyria occurs in representatives of all nations, in all European and African countries, in Japan and the USA. The greatest prevalence of the disease was observed in India.

As scientific research shows, congenital porphyria occurs among brothers and sisters belonging to the same generation. Cases of porphyrin metabolism disorders in children born to patients with congenital erythropoietic porphyria have been described. Transmission of dermatosis by an autosomal recessive route has been revealed. Patients with congenital erythropoietic porphyria are homozygotes in relation to the pathological gene, and close relatives are heterozygotes.

Using fluorescent 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 in excess of the norm and after the destruction of erythrocytes, these substances enter the blood serum and then accumulate in the tissues. It has been proven that inside the erythrocytes, the so-called enzymes porphobilinogen deaminase (urocorphyrin I synthesis) and uroporphyrinogen isomerase (uroporphyrin III) control the synthesis of heme.

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

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 appear at the age of 3-4 years and older. The disease occurs in men and women equally. Red urine is the initial sign of the disease.

Dermatosis begins mainly in the spring and summer months. On open areas of the body exposed to sunlight, blisters appear, accompanied by itching. The blisters contain serous or serous-hemorrhagic fluid. Blisters can also occur under the influence of various mechanical factors. As a result of the addition of a secondary infection, blisters and erosions turn into ulcers and in their place (most often on the extensor part of the arms) scars form. As a result of a long and chronic course of the disease, deep tissues are involved in the pathological process and mutilation of the ears is observed. feet. Nails are subject to dystrophy, thicken, deform and fall out. X-ray of the bone and joint system reveals osteoporosis, complete or partial contracture of the ligaments. Changes in the patient's eyes are expressed by conjunctivitis, clouding of the cornea and pupils. The color of the rash depends on the accumulation of porphyrins on the enamel and dentin, the entire surface of the teeth can be pink, pink-yellow or dark red. Teeth exposed to ultraviolet rays have a dark red shine. Hypertrichosis is observed on the skin of the face! Eyebrows and eyelids.

Pink-red fluorescence may be seen in the gums and teeth of some healthy children. This fluorescence is caused by porphyrins secreted by bacteria living in the mouth.

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

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

In the daily urine of patients, compared to the norm, uroporphyrins increase several hundred times, amounting to 140-160 mg, and coproporphyrins - 30-52 mg. Such high indicators in urine, in contrast to the liver form of porphyrins, are characteristic only of congenital erythropoietic porphyria.

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

In the treatment of congenital erythropoietic porphyria, patients are recommended to avoid sun exposure, take beta-carotene preparations, and take antipyretic drugs. Sometimes splenectomy gives good results.

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Erythropoietic protoporphyria

Erythropoietic protoporphyria was first described in 1953-54 by W. Kosenow and L. Treids. The authors noted elevated protoporphyrin levels in feces, signs of photosensitivity in two sick infants, and fluorescence of red blood cells and called this disease protoporphyrinemic photodermatosis. After a complete study of the metabolism in this disease, in 1961 L. Magnus included it in the porphyria group. Erythropoietic porphyria is a hereditary disease and is inherited in an autosomal dominant manner.

Dermatosis is mainly found among the population of Europe and Asia and Europeans living on the African continent. In erythropoiesis porphyria, due to the deficiency of the enzyme ferrochelatase in erythrocytes and erythroblasts, the conversion of protoorphyrin into heme is weakened, and the content of this metabolite in erythrocytes and erythroblasts increases sharply. Patients are especially sensitive to rays with a wavelength of more than 400 nm. The liver plays an important role in the development of erythronoetic porphyria. As in phytroblasts, protoporphyrins are synthesized in the liver in a pathological direction and accumulate in liver cells, as a result of which poorly soluble protoporphyrins are retained and have a toxic effect on the liver. The porphyrins present in large quantities in the blood plasma then enter the dermis, photodynamic reactions develop, cells and cell organelles are affected, lysosomal and cytolytic enzymes are released from them, which damage tissues and cells. Thus, clinical signs characteristic of phythropoietic porphyria appear on the skin. The period from the onset of sun exposure to the development of clinical symptoms of the disease depends on the strength of the acting rays and the concentration of porphyrins in the excited tissue.

Biochemical examination of close relatives of patients with erythropoietic protoporphyria revealed dermatosis occurring in a latent form.

In diagnosing the latent form of erythropoietic protoporphyria, the relative coefficient of proto- and coproporphyrins in feces is of great importance.

Erythropoietic protoporphyria is most common in men and is characterized by a chronic, relapsing course.

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

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

The clinical signs of the disease are not limited to erythema and edema, subsequently purpura and blisters appear. In severe cases of the disease, deep excoriations appear, and the clinical picture resembles pellagroid dermatitis. Erythropoietic porphyria has no specific clinical signs and in its clinical course is very similar to such photodermatoses as urticaria caused by exposure to sunlight, prurigo-eczematous photodermatosis and Bazin's light pox.

In almost all patients, the skin surrounding the eyes, mouth, upper part of the nose and hands is rough, thickened, and the skin pattern is pronounced. In the spring and summer months, hyperkeratosis and cracks on the red border of the lips, limited light-brown spots and superficial atrophic oval scars can be seen in some patients.

In the erythrocytes of almost all patients, the content of protoporphyrins is sharply increased. Increased content of uroporphyrins is rarely observed. There is information about increased content of protoporphyrins in the blood serum, increased content of coproporphyrins in some patients and the impossibility of determining the content of uroporphyrins (or a small content). In diagnosing the disease, the ratio of proto- and coproporphyrins plays a major role.

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, hyaline-like substances are located, which are manifested by a positive Schick symptom.

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Erythropoietic coproporphyria

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

Erythropoietic porphyrias 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 the treatment begins to manifest itself after 1 and 3 days. Correction of pathological changes in the liver is a complex task. For this, it is advisable to consult a hepatologist, gastroenterologist, transfuse red blood cells, cholesterolamine, hematin and other hepatotropic drugs.

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Mixed porphyria

Mixed porphyria belongs to the group of congenital hepatic porphyrias and is transmitted in a dominant manner.

Causes and pathogenesis. The disease is based on the deficiency of the enzyme protoporphyrinogen oxidase, as a result of which protoporphyrinogen is unable to turn into protoporphyrin. During an attack, the content of aminolevulanic acid increases sharply. In mixed porphyria, there is information about a decrease in the activity of ferrodelatases, which are part of normoblasts.

Abdominal and neurological signs of the disease can be mainly caused by drugs (barbiturates, sulfonamides, analgesics, antipyretics, etc.), alcoholic beverages and other hepatotoxic agents. Viral hepatitis, pregnancy, low carbohydrate content in food have a certain significance in the occurrence of the disease.

Symptoms

The disease is mainly found among white people aged 20 to 30 years living in South Africa. The skin manifestations of the disease are very similar to porphyria cutanea tarda (photosensitivity on exposed skin, blisters, erosion, scars). In addition, mental disorders, dysfunction of the central and peripheral nervous system and abdominal pain are observed. The clinical signs described above do not always appear simultaneously. When examining 113 patients with mixed porphyria, 50% of them had acute attacks and skin rash, 3.4% had only skin rash and 15% had only attacks. According to some authors, mixed porphyria in England and Finland, compared to South Africa, is mild, skin rash is the initial sign of the disease.

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

Histopathology

Pathological changes on the skin are no different from late cutaneous porphyria.

Treatment

Symptomatic treatment measures are carried out. During an attack, glucose, adenosine monophosphate, riboxin are prescribed, and in severe cases of the disease - hematin. It is advisable to use antioxidants.

Hepatoerythropoietic porphyria

The causes and pathogenesis of hepatoerythropoietic porphyria are not fully understood. 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 protoporphyropogen decarboxylase is inhibited.

The content of protoporphyrins increases in erythrocytes and serum, uroporphyrin in urine, and coproporphyrin in feces.

Symptoms

The disease begins at birth or in early childhood. Hepato-erythropoietic porphyria includes such clinical signs of diseases as porphyria cutanea tarda, congenital erythropoietic porphyria.

Histopathology

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

Differential diagnosis

The disease should be distinguished from congenital elidermolysis, Bazin's photopox, which occurs from rays, and other forms of porphyria.

Treatment

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

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Hereditary coproporphyria

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

Symptoms

In terms of clinical manifestation, the disease, being close to mixed porphyria, is mild. Intestinal pain is more common. Neurological and psychological changes are less common. Due to the fact that coproporphyrins have a lower phototoxic capacity than uroporphyrinogen, they accumulate little on the skin. Skin changes occur in only 1/3 of patients. Blisters form at the site of injury, and the clinical picture resembles late cutaneous porphyria.

The patient's feces show a sharp increase in coproporphyrin III levels. Sometimes this substance can be detected in urine.

Histopathology

Pathologically, the changes in the skin do not differ from those of late cutaneous porphyria.

Treatment

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

Treatment of porphyria

There is no specific treatment for porphyria cutanea tarda. During the treatment, it is necessary to normalize the disturbed metabolism in the body, remove the increased amount of circulating porphyrins from the body. In therapeutic doses, it is advisable to use B vitamins (B1, B6, B12), nicotinic acid. Vitamins are recommended every other day, vitamins B1 and B6 cannot be administered simultaneously on the same 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 0.1 g), aevit (2-3 times 1 capsule), methionine (0.5-0.75 g per day), sirepar (intramuscularly 2-3 ml, 50-60 injections per 1 course), etc. are recommended.

In late cutaneous porphyria, there is no consensus on the use of antimalarial (antipyretic) drugs. Some dermatologists believe that it is impossible to use delagil or other antipyretic drugs in late cutaneous porphyria, since under the influence of these drugs, retinopathy, agranulocytosis, vomiting, toxic psychosis, hair depigmentation and other negative consequences often occur. Along with this, other scientists suggest using antipyretic drugs in small doses (125 mg of chloriquine 2 times a week for 8-18 months). According to the scientists, antipyretic drugs form soluble complexes with porphyrsts in water and they are easily excreted from the body along with urine. It is advisable to recommend antipyretic drugs after vitamin therapy, after 15-20 days.

To suppress lipid peroxidation, antioxidant drugs, beta-carotene, alpha-tocopherol (100 mg once a day) are prescribed.

In severe cases of the disease, prednisolone is prescribed for 2 weeks at 5 mg 2 times a day, as well as B vitamins, ascorbic acid, calcium chloride (10% solution, one tablespoon 3 times a day).

To protect against sun rays, it is recommended to use sunscreens. The following medications are prohibited for patients: sulfonamides, griseofulvin, barbiturates (barbital, thiopental, phenobarbital, etc.).

A dietary diet is recommended. The diet should not include fatty meat (lamb or pork), fried fish, or rich soups.

Erythropoietic porphyrias are characterized by the following features:

• the disease usually begins in childhood;
• the cause of the disease is genetic (hereditary) enzyme deficiency;
• before the development of clinical symptoms of the disease, provoking factors have no effect;
• porphyrin metabolism disorder occurs in bone marrow erythroblasts;
• During fluorescence microscopy of erythrocytes and erythroblasts found in peripheral blood, red fluorescence (glow) characteristic of porphyrins can be observed;
• Red blood cells in the peripheral blood always contain a high content of porphyrins.

A distinction is made between congenital erythropoietic porphyria, erythropoietic proto- and coproporphyria.