Porphyria skin

Porphyria skin (late) - a chronic disease caused by the violation of porphyrin metabolism and result in increased sensitivity of the skin to ultraviolet rays. In occurrence porphyria skin is of great importance disruption of the liver resulting infection, chronic intoxication leaded gasoline, alcohol abuse.
Ill more often than men in middle age.
In the open areas of the body (face, neck, arms) after sun exposure bubbles with serosanguineous content on slightly damaged basis. Their thin tire easily compromised, exposing erosive surface, covering the crusts; after resolution of the lesions remain atrophic skin. In feces and urine enhanced content of porphyrin; urine color red or beer. The aggravation of porphyria skin comes in the spring-summer period.
Treatment. Prescribed drugs that reduce the sensitivity of the skin to ultraviolet rays,- chloroquine, delagil, resochin - analogues hingamina 0.25 g 2 times a day cycles for 10 days on a three-day break; injections of vitamins B12 and B6; nicotinic acid, methionine; naruzhno - solution of aniline dyes.
Prevention. The patients should be under medical supervision. Prohibit the use of alcohol in the spring appoint a preventive course of treatment of the above mentioned drugs, sunscreen ointments (with 5-10% of the salol, 3-10% quinine), cream "Ray" and other

Porphyria cutanea tarda (late porphyria skin) is a chronic disease characterized by the appearance of the skin under the influence of ultraviolet irradiation of bubbles. Late porphyria skin usually develops in individuals with increased number of porphyrins (see), which increases sensitivity is the main cause of the disease. In causing the disease great role is played by the various violations of the liver, especially under the influence of alcohol, drugs arsenic, bismuth, leaded petrol. A number of authors have noted the appearance of porphyria skin in patients who have dealt with dichlorphenamide and trichlorophenolate, the persons in contact with hexachlorbenzene. In the liver are not enough processes decarboxylation and dehydrogenation of uroporphyrin in protoporfirina.
Porphyria skin occurs mainly in spring and summer, much less in autumn and winter; occurs more frequently in men aged 40-60 years. Localized rash mainly on the exposed parts of the body, the hands, the bottom third of the forearms, face, sometimes on the neck, chest, and back. First, there is itching, then redness and swelling, almost simultaneously bubbles and blisters with serous, serosanguineous, sero-purulent content. The result itching and injuries on the spot bubbles formed erosion, then serous and serous bloody brown; further on these places atrophy, appear prosapogenin elements, pigmentation, mainly in the temporal-Malar region, reminiscent of the color of the skin at Addison disease; pigmentation on mucous membranes rare; in the zygomatic areas can develop hypertrichosis.
Often in the lesions observed symptom of St. Nicholas (detachment of the epidermis after a little pressure). Should take into account the General condition of patients who, in addition to the symptoms of hepatic dysfunction may be different abdominal and nerve syndromes, especially polyneuritis with pathological reflexes, atrophy of muscles, rarely detected diabetes. Histologically - thorn in the layer intercellular edema, many pigment (melanin), mainly in the basal layer; subepidermal - bubbles. Differential diagnosis - light the smallpox (see Hydroa vacciniforme), pellagra (see), degenerative form of bullosa, a limited type of the disease during the (see during illness).
Treatment. Vitamins Niacin, Riboflavin, vitamin B12, ascorbic acid with glucose intravenously, methionine (better than fresh cheese). To protect from sunlight - inside antimalarial drugs (delagil, chloroquin-diphosphate), sodium salt para-aminobenzoic acid. You should avoid any photosensitizing tools, techniques barbiturates and sulfanilamidov. Sunscreen means - ointment with 10-15% of para-aminobenzoic acid, with 5-10% salol or quinine, cream "Ray"; wearing protective red and yellow veils, hats with a wide brim.