Gangrenous piodermiya – the secondary dermatosis with destruction of skin and necrosis of the subject fabrics accompanying system pathologies. It is clinically shown by a rash of bulls with serous, purulent or hemorrhagic contents which are opened, forming the pink ulcers extending on the periphery of the size of a palm with torn edges and piogenny separated. Emergence of ulcers is followed by prodromy. Diagnose a gangrenous piodermiya on the basis of system pathology in the anamnesis and typical skin symptomatology – piogenny ulcers. For an exception of other dermatosis carry out a complex of laboratory researches. Perform treatment of primary pathology, in parallel appoint corticosteroids and antibiotics inside, outwardly apply turbulent bathtubs, damp antiseptic bandages.
Gangrenous piodermiya – the heavy progressing skin necrosis arising against the background of system pathology and which is characterized by formation of deep ulcers with it is purulent - necrotic separated. The gangrenous piodermiya is a rare disease, is annually diagnosed for 1-3 of 100 000 people. Most often skin pathology accompanies inflammatory diseases of a gastrointestinal tract (30%), a patergiya (30%), arthritises, tumors, leukoses. From 25% to 50% of cases of a gangrenous piodermiya have idiopathic character. The first descriptions of a gangrenous fagedenichesky ulcer belong to the French dermatologist Louis Brock investigating this pathology at the beginning of the XX century. Modern idea of a nosology was entered into dermatology by the American experts A. Bransting, V. Hokerman and P. O'Leyri in 1930.
The gangrenous piodermiya has also other names: geometrical fagedenizm, ulcer dermatitis, serpinginiruyushchy and gangrenous and ulcer vegetiruyushchy piodermiya. The essence of names of pathology is connected with the main clinical symptom of a disease – progressing is purulent - a necrotic ulcer. The Fagedenichesky form of a disease meets only in the tropical and subtropical countries of both hemispheres where climate crude and hot. Other forms are not endemic. Pathological process arises at any age, the peak of incidence falls on 20-40 years, children are ill seldom. Insignificant prevalence of female patients is noted. Relevance of a problem is connected with serious trophic violations which significantly reduce quality of life of patients.
Reasons of a gangrenous piodermiya
The etiology and pathogenesis of pathological process are definitely not established. In some cases hereditary predisposition matters. The gangrenous piodermiya as simptomokompleks is a part of the rare genetically determined PAPA syndromes (purulent arthritis, a gangrenous piodermiya, gangrenous acnes), PASH (the gangrenous piodermiya, an acne, purulent gidradenit) and PASS (purulent arthritis, a gangrenous piodermiya, an acne, purulent gidradenit). Mutations of a gene of PSTPI1/CD2BP1 located in a chromosome 15q are the reason of the listed syndromes. Pathologies are inherited on the autosomno-prepotent mechanism.
There is also immune theory of development of a gangrenous piodermiya. According to this theory pathological process arises against the background of the changed sensitivity terms to endogenous and exogenous agents. It is long the existing chronic somatic diseases cause changes of cellular and humoral immunity due to violation of neuroendocrine regulation, exchange processes, traffic of fabrics. Aberrantny immune reaction in which T-lymphocytes and tsitokina are involved begins. Cages of the immune T-helpery system develop antibodies – the reagina creating a skin sensitization basis. At the same time T-supressory try to suppress these reactions, the non-standard immune answer in the form of necrotic destruction of connecting fabric as a result develops.
Process of formation of an ulcer at a gangrenous piodermiya takes place three stages. At a stage of immune reactions there is an accumulation of antibodies, specific to a certain type of an anti-gene. At the same time immune lymphocytes and antibodies of a class E of blood pass into skin tissues, sensibilizing it. At the second (patokhimichesky) stage of a disease repeated hit of an alien anti-gene causes activation of proteolytic enzymes of cages and fabrics with violation of their functions. Epidermalny cages begin to produce biologically active agents provoking an inflammation. At the third (pathophysiological) stage there is a specific response of skin to introduction of a pathological anti-gene in the form of degranulation of cages, destruction of vessels, violation traffic and local destruction of fabric. Depth of destruction depends on quantity of a pathogenic anti-gene and degree of stability of immune system.
Classification of a gangrenous piodermiya
In modern dermatology it is accepted to distinguish several kinds of pathological process. Allocate:
1. A classical (ulcer) gangrenous piodermiya which is characterized by deep ulcers of a violet shade with the edges hanging over a surface of defect of skin. Pathology is combined with system processes in intestines, joints, pulmonary fabric. Enter into this group piostomatit, piogenny gangrenous damage of genitals, periorbitalny fabrics, mucous top airways.
2. The atypical gangrenous piodermiya consisting of a bullous and pustulous version. This form of a disease accompanies blastny leukoses or miyeloproliferativny diseases, is characterized by puzyrny or gnoychikovy rashes on an edematous border on the periphery of an ulcer with localization on a face and extremities.
3. The post-traumatic gangrenous piodermiya arising against the background of a patergiya.
4. The Fagedenichesky gangrenous piodermiya, endemic for tropics and subtropics which distinctive feature is plurality and strong accustoming to drinking with the subject fabrics.
5. The malignant superficial granulematozny gangrenous piodermiya accompanying system pathologies and malignant tumors.
Symptoms of a gangrenous piodermiya
Changes on a surface debut terms furunkulopodobny elements with transparent or hemorrhagic contents against the background of hyperaemia. The surface of skin in places of rashes at a gangrenous piodermiya infiltrirutsya, then nekrotizirutsya with formation of the ulcer of the polycyclic form reaching the sizes of a palm of the adult increasing deep into and in breadth. At peak of the development the center of defeat represents a continuous ulcer surface with valikoobrazny edges and frills from the epidermis remains. The ulcer of lividny color with raised edges and a wide strip of hyperaemia on perimeter is filled with pus, blood clots, the remains of epidermis and bacteria, exhales a stench. The bottom of defect of skin hilly, is covered with bright granulations. An outcome of a gangrenous piodermiya – a reepitelization from edges of defect in a hem.
The subjective state is broken a little, formation of an ulcer is followed by morbidity, in the period of aggravations there are prodromalny phenomena, artralgiya, mialgiya join. The gangrenous piodermiya begins sharply, but hronizirutsya quickly enough, possesses a wavy current with a frequent recurrence. In 50% localization of pathological process is the shin, as a rule, the center single. Fagedenichesky ulcers more often multiple, arise mainly on genitals. Distinctive feature of this form of a gangrenous piodermiya is bystry formation of the ulcer covered with a difteroidny raid of gray color and densely soldered to the subject fabrics. The roller around defect of skin has the pointed edges and extremely dense consistence. Formation of defect of fabrics is followed by polyneuritis and a lymphangitis.
Diagnostics and treatment of a gangrenous piodermiya
The clinical diagnosis is made by the dermatologist on the basis of the anamnesis, specific symptomatology and consultations of narrow experts as the gangrenous piodermiya is a cross-disciplinary disease. The histology of the center of defeat is not indicative, but in difficult cases is useful. Thrombosis of vessels, granulematozny infiltrates, destruction islands in epidermis and a term comes to light. Carry in addition out an electrophoresis of serumal protein, exclude the mikotichesky nature of an ulcer, take blood on RW. Differentiate a gangrenous piodermiya with ulcer tuberculosis of skin, tertiary syphilis, deep dermatomikoza, a vegetiruyushchy ulcer piodermiya, an anthrax, granulematozy Wegener, pannikulity, amyobiazy, bromodermy, gangrene, a vegetiruyushchy puzyrchatky, mikobakterialny and klostridialny infection, trophic violations, pustulous psoriasis.
Therapy of a gangrenous piodermiya is begun with correction of somatic pathology at profile experts as skin pathological process without it badly gives in to therapy. The purpose of dermatological treatment is removal of inflammatory manifestations, painful feelings, restoration of integuments. For this purpose conduct courses of hormonal therapy and antibiotic treatment. In hard cases connect cytostatics, immunosupressor, potassium iodide, intravenously enter immunoglobulin. The plasma exchange is shown. Outwardly at a gangrenous piodermiya clear a surface of defect of skin by means of vibration and turbulent bathtubs, antiseptic solutions. Apply the damp drying bandages with silver solutions, hormonal medicines. Taking into account a patergiya try to avoid surgical manipulations. The forecast of a gangrenous piodermiya is defined by primary pathology which caused an ulcer.