Erythema multiforme exudative: causes, symptoms, diagnosis, treatment

Erythema multiforme exudative is an acute, often relapsing disease of the skin and mucous membranes of infectious and allergic genesis, a polyethological disease predominantly of a toxic-allergic origin, most often developing sex through the influence of infections, especially viral infections, and the effects of medications. This disease was first described by Hebra in 1880.

The causes and pathogenesis of erythema multiforme exudative remain unclear. But the opinion of many scientists, the disease has a toxic-allergic origin. The disease is considered as a hyperactive reaction directed to keratinocytes. In the blood serum of patients, circulating immune complexes are detected and the deposition of the IgM and S3 complement components in the blood vessels of the dermis is noted. Trigger factors can be viral and bacterial infections, medicines. There is a connection with rickettsiosis. There are two forms of the disease: idiopathic with unknown etiology and secondary with an identified etiological factor.

Symptoms of erythema multiforme exudative. It is clinically manifested by small erythematous edematous spots, patchy-papular rashes with eccentric growth with the formation of two-contour elements due to a brighter peripheral and cyanotic central part. There may be ring-shaped, cockade shaped figures, bubbles, and in some cases bubbles with transparent or hemorrhagic contents, vegetation. Preferred location - extensor surfaces, especially the upper limbs. Often, rashes occur on the mucous membranes, which is more typical of the bullous form of exudative multiform erythema. The most severe clinical variant of the bullous form of the disease is Stevens-Johnson syndrome, which occurs with a high fever, joint pain. There may be signs of myocardial dystrophy and damage to other internal organs (hepatitis, bronchitis, etc.). Expressed the propensity of multiforme exudative erythema to relapse, especially in the spring and autumn period.

In clinical practice, two forms of multi-form exudative erythema are distinguished - idiopathic (classical) and symptomatic. With an idiopathic form, it is usually not possible to establish an etiological factor. With a symptomatic form, a certain precipitating factor is known.

Idiopathic (classical) form usually begins with prodromal phenomena (malaise, headaches, fever). After 2-3 days, symmetrically located restricted spots or flattened edematous papules of round or oval shape, 3-15 mm in size, pinkish-red or bright red, appear sharply on the periphery. Peripheral cushion acquires a cyanotic hue, the central part sinks. In the center of individual rashes new papular elements are formed with the same development cycle. On the surface of the elements or on unchanged skin, there are various-sized vesicles, blisters with serous or hemorrhagic contents, surrounded by a narrow inflammatory rim ("bird's eye symptom"). After a while, the bubbles drop and their bezel becomes cyanotic. In such areas, concentric figures are formed - herpes iris. Their dense cover is opened and erosions are formed, which quickly become covered with dirty-bloody crusts.

The preferred location of the elements is the extensor surfaces of the upper limbs, mainly the forearms and hands, but they can also be located on other areas - the face, neck, legs, ghoul foot.

Lesion of the mucosa and lips occurs in about 30% of patients. In the beginning there are edema and hyperemia, and in 1-2 days there are bubbles or bubbles. They quickly open, exposing the bleeding bright red colors of erosion, at the edges of which the remains of tires cover. Lips swell, their red border is covered with bloody and dirty crusts and more or less deep cracks. Due to severe soreness, it is very difficult to eat. The outcome in most cases is favorable, the disease usually lasts 15-20 days and disappears without a trace, rarely in places of rashes for a while there is a small pigmentation. Sometimes the process can transform into Stevens-Johnson syndrome. For the idiopathic form, the seasonality of the disease (in the spring and autumn months) and relapses are characteristic.

With a symptomatic form, rashes appear similar to classical exudative erythema. Unlike the classical type, the appearance of the disease is associated with the reception of a specific agent, there is no seasonality, the process is more common. In addition, the skin of the face and trunk is not less affected, the cyanotic hue of the rashes is not so pronounced, ring-shaped and iridescent rashes, etc., may be absent.

Drug multiforme exudative erythema is mostly of a fixed nature. Bubbles predominate from morphological elements, especially when the process is localized in the oral cavity and on the genital organs.

Depending on the clinical picture of the rash, spotted, papular, spotty-papular, vesicular, bullous or vesicle-bullous forms of exudative erythema are distinguished.

Histopathology of erythema multiforme exudative. The histopathological picture depends on the clinical nature of the rashes. At spotted-papular form in the epidermis spongios and intracellular edema are noted. In the dermis there are edema of the papillary layer and perivaskunny infiltrate. The infiltrate consists of lymphocytes and a certain amount of polymorphonuclear leukocytes, and sometimes eosinophils. In cases of bullous eruptions, the blisters are localized under the epidermis and they can sometimes be found intraepidermally only in old rashes. The phenomena of acantholysis are always absent. Sometimes extravases of erythrocytes without signs of vasculitis are visible.

Pathomorphology of erythema multiforme exudative. Characteristic changes in the epidermis and dermis, but in some cases the epidermis predominates, in others - the dermis. In this regard, there are three types of lesions: dermal, mixed dermo-epidermal and epidermal.

With the dermal type, infiltration of the dermis of varying intensity is observed, sometimes occupying almost all of its thickness. Infiltrates consist of lymphocytes, neutrophils and eosinophils, granulocytes. When the edema of the papillary layer of the dermis is expressed, bubbles can form which are covered with the epidermis together with the basal membrane.

The dermo-epidermal type is characterized by the presence of a mononuclear infiltrate, located not only perivascularly, but also near the dermo-epidermal junction. In the basal cells there is hydrophilic degeneration, in prickly ones - necrobiotic changes. In some areas, infiltrate cells penetrate the epidermis and, as a result of spongiosa, can form intraepidermal vesicles. Hydropic degeneration of basal cells in combination with pronounced edema of the papillary dermis can lead to the formation of subepidermal blisters. Quite often, extravases from erythrocytes are formed in this type.

With epilermaadium type in the dermis, only a weak infiltration is observed, mainly around the surface vessels. In the epidermis even in the early stages there are groups of epithelial cells with necrosis phenomena, which then undergo lysis, merge into a continuous homogeneous mass that separates to form a subepidermal bladder. This picture is similar to that in toxic epidermal necrolysis (Lyell's syndrome). Sometimes necrobiotic changes are noted in the superficial parts of the epidermis and, together with edema, lead to the formation of subthreshold blisters with further rejection of its upper parts. In these cases, it is difficult to differentiate exudative erythema multiforme from herpetiform dermatitis and bullous pemphigoid.

Histogenesis of erythema multiforme exudative. The main mechanism of the development of the disease, most likely, the immune. In patients with direct immunofluorescence microscopy, a high titer of intercellular circulating antibodies is detected, however, the results of direct immunofluorescence microscopy of the affected tissue are negative. These antibodies are able to fix complement, in contrast to antibodies in pemphigus. Scientists have found an increase in the number of lymphokines, a macrophage factor, which indicates a cellular immune response. In the cell infiltrate in the dermis, mainly T-lymphocytes-helpers (CD4 +) are detected, and in the epidermis - mainly cytotoxic T-lymphocytes (CD8 +). The immune complexes are also involved in pathogenesis, which is primarily manifested by damage to the walls of the blood vessels of the skin. Thus, it is proposed that a combined immune response develops, including a delayed type hypersensitivity (type IV) and an immunocomplex allergic reaction (type III). The association of the disease with the antigen HLA-DQB1 was revealed.

Differential diagnosis is performed with a fixed sulfanilamide erythema, disseminovapnuyu red lupus, erythema nodosum, bullous pemphigoid, pemphigus, urticaria, allergic vasculitis.

Treatment of erythema multiforme exudative. With spotty, papular and light bullous forms, symptomatic treatment is performed - hyposensitizing (calcium preparations, sodium thiosulfate), antihistamines and external-aniline dyes, corticosteroid preparations. In severe cases, corticosteroids are administered orally (50-60 mg / day) or as injections, in the presence of secondary infection - antibiotics, herpes infection - antiviral drugs (acyclovir).

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