If someone would like to know exactly what pigment xeroderma looks like, it is worth revisiting the mystical thriller “The Others” by director A. Amenabar, where the children of the main character suffer from intolerance to sunlight and live in a house with tightly drawn curtains. Xeroderma pigmentosa actually occurs due to increased sensitivity of the skin to the rays of the sun, starts as skin inflammation, and can eventually lead to skin cancer. Today, there are enough treatments that help to significantly slow down the development of xeroderma pigmentosum and prevent its transition to a malignant tumor.
Xeroderma pigmentosa is a hereditary disease. The problem is that the enzymes that protect skin cells from damage from ultraviolet rays and eliminate the defects that have developed are either inactive or absent in some people. As a result, with each irradiation with solar ultraviolet, more and more damaged DNA molecules become in the cells, and there is nothing to restore them. The defects accumulate and over time can lead to the development of skin cancer.
Clinical manifestations of xeroderma pigmentosa and diagnosis
The development of xeroderma pigmentosa goes through three stages.
Stage 1 most often manifests itself in childhood - in the summer, after prolonged exposure to the sun, the skin in open areas - the face and neck, forearms and hands - becomes inflamed and flaky. The inflammatory reaction is persistent, then the skin becomes covered with spots like freckles or lentigo. With each new irradiation, the spots become larger and darker.
Stage 2 usually becomes pronounced after a few years - the skin becomes variegated due to uneven pigmentation, visible spider veins, the appearance of atrophied areas. A little later, warty growths, ulcers and cracks appear on the skin. Without proper treatment, cartilage and connective tissues suffer, which leads to deformation of the auricles, nasal passages, and mouth. Some patients have eye lesions - blepharitis, eyelash loss, corneal clouding, photophobia.
Stage 3 is characterized by the development of tumors (keratomas, basaliomas, fibromas, melanomas) in foci affected by xeroderma pigmentosa. Particularly dangerous are foci of warty growths that metastasize to internal organs.
Diagnosis of xeroderma pigmentosa is based on the data of a cytological study and the relationship of its results with a history of solar exposure, external manifestations of the disease in open areas of the skin.
Treatment of xeroderma pigmentosum and prevention of complications
At the first and second stages of pigment xeroderma, the treatment is carried out by a dermatologist. A good effect is the use of antimalarial drugs, which protect cellular DNA from destruction, make the skin less sensitive to sunlight. Therapy must be supplemented with the intake of vitamins A, group B, PP. If necessary, antihistamines and desensitizers are recommended.
Corticosteroid ointments are applied topically on damaged areas of the skin, areas with warty growths are treated with cytostatics, and if possible, growths are surgically removed to avoid malignancy. At the third stage of xeroderma pigmentosa, an oncologist performs observation and treatment.
To slow down the progression of the disease and its transition to a malignant stage, patients are advised to protect open body plots from sunlight - use sunscreen, wear tight clothing, hats, gloves, umbrellas.
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