Listovidny puzyrchatka (eksfoliativny puzyrchatka) — a clinical form of a puzyrchatka of which formation of flat not intense bubbles, repeated emergence of bubbles under crusts on the place of already resolved elements and sharply expressed stratifying of epidermis is characteristic. The course of a disease chronic, long-term, about tendency to generalization and defeat of extensive sites of skin. Exact diagnosis and a differentiation of a disease from other forms of a puzyrchatka is performed during the histologic research. At a listovidny puzyrchatka therapy by immunosupressivny medicines (kortikosteroidny hormones, cytostatics), local treatment is carried out.
The listovidny puzyrchatka received the name for the account of the characteristic layered crusts covering the erosion formed on the place of bubbles. In structure of a dermatosis incidence of a listovidny puzyrchatka does not exceed 1,5%. The Listovidny puzyrchatka is the most often found form of a puzyrchatka at children. At adults it prevails over a vegetiruyushchy puzyrchatka. As well as other types of a puzyrchatka, the listovidny puzyrchatka has the autoimmune mechanism of development with education autoantitet to cells of epidermis. Without adequate therapy patients can die from accession of a secondary infection, a kakheksiya.
Symptoms of a listovidny puzyrchatka
The Listovidny puzyrchatka begins with emergence of the reddened sites of skin with the flattened flabby bubbles located on them. In some cases the beginning of a disease proceeds as a vulgar puzyrchatka, dermatitis of Dyuringa or reminds eritematozny changes at eczema, a toksikodermiya, seboreyny dermatitis. Sometimes bubbles are not expressed, and flaking of epidermis decides as the site on an uneven surface.
Bubbles have a thin tire and are quickly broken off. Bright red erosion with plentiful separated which dries up with formation of crusts are formed. New portions of separated form new layers of crusts. The elements, characteristic of a listovidny puzyrchatka, covered with layered crusts and reminding puff pastry are as a result formed.
The main distinctive feature of a listovidny form of a puzyrchatka is weakening of communication of granular and horn layers of epidermis which is shown even on externally healthy sites of an integument. It causes sharply positive symptom of Nikolsky — peeling of epidermis on considerable distance at a pandiculation for the remains of a tire of the become torn bubble. For the same reason even insignificant superficial damage of not changed sites of skin leads to stratifying of epidermis with formation of bubbles.
Erosion heal slowly. Often under crusts new bubbles are formed and this process can constantly continue that is also clinical feature of a listovidny puzyrchatka. When progressing a disease the located bubbles separate nearby are inclined to merge with formation of extensive sites of defeat.
The general condition of patients with a listovidny puzyrchatka at the beginning of a disease can be normal. In process of increase in the area of affected areas of skin it worsens. Even the insignificant movements begin to hurt the patient because of what the mentality suffers and there are sleep disorders. Rises in temperature are noted, patients lose appetite and grow thin, the kakheksiya develops. At distribution of process on head skin, there is a hair loss. Nails grow dull, are thickened and can drop out. Damage of a mucous oral cavity is observed extremely seldom.
Diagnostics of a listovidny puzyrchatka
Often the diagnosis of a listovidny puzyrchatka can be made on the basis of the clinical features of a disease described above. In dabs prints from a surface of erosion akantolitichesky cages, that is cages, characteristic of all types of a puzyrchatka, with the broken intercellular communications are found. The histologic research finds existence of cracks or bubbles between a horn and granular layer of epidermis, a rupture of communications between cages (). In the old centers the degeneration of cages of a granular layer of skin is noted and .
From additional methods of diagnostics apply the immunological researches confirming the autoimmune mechanism of development of a disease. However reactions of a direct and indirect immunofluoristsention (REEF) do not allow to distinguish a listovidny puzyrchatka from vulgar precisely.
The Listovidny puzyrchatka is differentiated from other forms of a puzyrchatka, and also from dermatitis of Dyuringa, an eritrodermiya, a syndrome Layell and other diseases.
Treatment and forecast of a listovidny puzyrchatka
Treatment is carried out under observation of the dermatologist generally glucocorticosteroids: dexamethasone, Prednisolonum, methylprednisolonum, , etc. In hard cases therapy is supplemented with immunosupressor (, a methotrexate). The positive effect at a combination of kortikosteroidny medicines with synthetic antimalarial means is noted (, , etc.). Bathtubs with anti-inflammatory, disinfectants or the knitting additives are locally recommended (for example, oak bark broth).
The forecast is adverse. Against the background of treatment the disease can proceed a long time. But as a result the patient perishes from a kakheksiya, the joined infections or complications of continuous glucocorticoid therapy.