Syndrome It is twisted
Contents:
Description:
Syndrome It is twisted - rare (or seldom diagnosed) the disease from group of a neutrophylic dermatosis which is characterized by a recurrent current, painful, bright red color papules and the inflamed plaques with mastoidal eminences, fever, an arthralgia and a neutrophylic leukocytosis.
The syndrome Suite arises in most cases at patients with general and limfoproliferativny diseases and malignant tumors. It is most often about postinfectious acute reactions, and in 20% of cases - about paraneoplaziya. Women (80-90% of cases) are ill more often, and most of patients has age of 30 years and are more senior (on average than 45-50 years). From associated diseases acute respiratory infections, hemoblastoses are noted. Distinguish two forms of a disease: an idiopathic syndrome It is twisted also paraneoplastic, connected with a neoplasm.
Symptoms of the Syndrome It is twisted:
For several weeks (usually for 2-4 weeks) to rashes patients have an acute respiratory disease, intestinal infection, quinsy or flu. Patients note deterioration in the general state, weakness, a headache, an ache in joints, muscle pains, fever with body temperature to 39-40 °C. On this background there are skin rashes which are located most often on a face, a neck, hands and legs; skin of a trunk is surprised seldom. Also generalized damage of skin is possible. Rashes are presented in the form of papules or nodes of red or violet color which merge among themselves and form plaques, with a diameter of 3-5 cm, irregular shape, with a clear boundary. Owing to a strong cutaneous dropsy rash can remind vesicles and bubbles, but to the touch they dense (so-called pseudo-vesicles). Sites of a necrosis of epidermis, the becoming wet erosion and crusts are noted.
At the expressed leukocytosis the disease is shown by the real bubbles, and then the centers of defeat remind a gangrenous pyoderma. Rashes cause pain or morbidity when pressing. Elements single or multiple, are located randomly and asymmetrically. For some time skin in the center of plaques takes a normal form, and they become similar to rings or arches. Disturbance of the general state is possible: fever, headache, arthralgia, indisposition. Some patients have a conjunctivitis, an episcleritis. Skin damages can develop on site microtraumas or pricks. This phenomenon is called a pathergy and is noted also at a gangrenous pyoderma and Bekhchet's disease. Without treatment the state worsens within several days or weeks then rashes begin to heal slowly. Duration of a disease varies from 3 to 9 weeks. A recurrence is observed at a half of patients, at the same time rash usually arises on the same place.
The reasons of the Syndrome It is twisted:
The etiology and pathogeny of a syndrome Suite are unknown. It is supposed that the caused reaction to viral, bacterial or tumoral antigens, however without existence of the classical, mediated by cell-bound immune complexes komplementzavisimy reaction is the cornerstone of a disease immunological. Development of a disease after reception of medicines is noted. It is possible that reaction of supersensitivity like Artyus to antigen of the postponed infection is the cornerstone of a disease. After stimulation allergen often forms cell-bound immune complexes which after accumulation in fabrics cause direct activation of inflammatory cells and release of mediators and cytokines. Level интерлейкина - would increase, and the maintenance of G-CSF (a colony stimulating factor of granulocytes) in an acute phase of a disease increases by 300 times. In certain cases the disease is caused iyersiniozy. Some authors on similarity of a clinical current consider a disease of one of options of a mnogoformny exudative erythema. The syndrome Suite can develop against the background of myeloproliferative diseases, acute or a myelosis, other malignant new growths (urogenital and gastrointestinal paths), nonspecific ulcer colitis, a high-quality monoclonal gammapathy, autoimmune diseases.
Treatment of the Syndrome It is twisted:
Choice drugs at an idiopathic syndrome Suite are corticosteroids. Use of system steroids at a paraneoplastic form of a disease is inefficient. Sometimes good results are yielded by dapsone and potassium iodide. Antibiotics are effective only when the disease was provoked iyersiniozy. Locally appoint the fatty shaken-up suspensions or creams with corticosteroids (целестодерм).
