Hydroa Vacciniforme

HYDROA VACCINIFORME (from Greek hydor - water), synonym hydroa aestivale, puerorum, a relatively rare disease, first described in 1862 by Bazin and characterized by extreme sensitivity of the skin to ultraviolet rays of the solar spectrum. The etiology is unclear; most authors see the cause of H. v. in metabolic disorders, expressed by the significant hematoporphyrinuria often found in hydroa vacciniforme cases (see). Often having a familial character, the disease almost always begins in the first years of life and localizes on open parts of the body. After b. or m. prolonged exposure to the sun, dense nodes, the size of a lentil to a forest nut and larger, appear on the face and hands; the central parts of which soon transform into tense, sometimes hemorrhagic blisters and then into pustules; after the fall of necrotic crusts, smallpox-like scars remain. In mild cases, blisters form only in spring and summer (hydroa aestivale); in severe cases, eruptions appear year-round, also on closed parts of the body; in some, rare cases, the process can cause significant disfigurement. In the final stage, the skin, mainly of the face and fingers of the hands, is sharply pigmented, covered with telangiectasias and is dense, as in scleroderma. Eruptions are always accompanied by severe itching. By 20-30 years of age, spontaneous healing usually occurs. Histologically, acute inflammatory phenomena leading to deep necrosis are noted in the skin. Treatment of affected areas is purely symptomatic (ichthyol, l-sinovar ointments, etc.); the main treatment is preventive, consisting in appropriate protection of healthy skin parts from sunlight (yellow and red veils, powders with red clay, ointments with coumarin).