Duhring's herpetiform dermatitis

Herpetiform dermatitis of Dühring (synonyms: Dühring's disease, pemphigoid herpes, etc.) belongs to the group of herpetiform dermatoses.

This group of diseases includes a variety of etiology and pathogenesis, but similar in the clinical and morphological manifestations of rashes of dermatoses, which are characterized by herpetiform grouping of the rash. In addition to the herpetiform dermatitis Dühring, this group also includes herpes of pregnant women and subcorneal pustules.

The name was given to the disease by the Philadelphia dermatologist Duhring in 1884. Currently, the disease is not rare, occurs in people of any age group. Men are sick more often than women.

[1], [2], [3], [4], [5], [6], [7]

Causes and pathogenesis of herpetiform dermatitis Dühring

The causes and pathogenesis of the disease to the present time remain not fully understood. It is believed that the herpetiform dermatitis of Dühring is a polysystemic disease of the autoimmune nature. The polysystemic nature of the disease is confirmed by the fact that the signs of enteropathy are due to an increased sensitivity to gluten, in particular to gluten, found in cereal proteins. In connection with this, the appointment of a gluten-free diet for therapeutic purposes leads to both clinical improvement and normalization of the mucosa of the small intestine. Detection of IgA antibodies in the papillary layer of the dermis or along the basement membrane of circulating immune complexes in the serum indicates the autoimmune nature of the dermatosis. Some dermatologists believe that hereditary predisposition, increased sensitivity to iodine, a decrease in antioxidant activity, in particular SH-groups, etc., are of great importance in the development of dermatosis. In a number of cases, Dühring's disease is considered as a paraneoplastic process.

Most authors refer to Duhring's herpetiform dermatitis for autoimmune diseases with the presence of IgA antibodies against the structural components of dermal papillae near the basal membrane. V.V. Serov (1982) considers herpetiform dermatitis an immunocomplex disease caused by various exogenous antigens. Indirectly the immune nature of herpetiform dermatitis confirms its combination with other autoimmune processes. Indicate the role of gluten enteropathy in the development of the disease. Depending on the nature of the deposition of IgA (granular or fibrillar) on the tips of the papillae of the dermis or linear along the basal membrane, two variants of this dermatosis are distinguished. Granular deposits predominate, occurring in 85-95% of cases. According to S. Jablonska and T. Chorzelsky (1979), the granular type of IgA deposition is characteristic of patients suffering from gluten enteropathy at the same time.

[8], [9], [10], [11], [12], [13]

Histopathology of herpetiform dermatitis Dühring

Under the epidermis, a bubble is formed, which is formed as a result of the separation of the epidermis from the dermis under the influence of the edema in the upper part of the skin itself. The epidermis above the bladder is not changed. Bubbles are rounded and contain a significant number of eosinophils. Detect IgA in the dermo-epidermal zone or in the papillary layer of the dermis.

Pathomorphology of herpetiform dermatitis Dühring

A typical picture of herpetiform dermatitis is observed in the erythematous elements of the rash in the early stages of the process, which is expressed in the accumulation of neutrophilic granulocytes with an admixture of eosinophils in the region of the tips of the papillae of the dermis, with the increase in the number of microabscesses. In the latter, in addition to neutrophilic and eosinophilic granulocytes, fibrin accumulates; The tissue of the papillae in these areas undergoes necrosis. Interpapillary outgrowths of the epidermis remain attached to the dermis, as a result of which the bubbles are multi-chambered. A few days later, the connection of the epidermal outgrowths to the dermis is broken, the blisters are enlarged, becoming single-chambered and clinically pronounced. Very often, on the periphery of a single-chambered bladder, one can see histologically the papillary microabscesses characteristic for this disease. Sometimes, in long-term foci, in connection with the regeneration of the epidermis, gradually covering the bottom of the blisters, they rise higher, becoming intra-epidermal and may be found in prickly and horny layers. In the subepidermal sections of the dermis, a moderate inflammatory infiltrate from neutrophilic and eosinophilic granulocytes is seen, among them many destroyed nuclei forming the so-called nuclear dust. In the lower parts of the dermis - perivascular infiltrates, consisting of mononuclear cells with an admixture of neutrophilic granulocytes. The above signs in bioptates are not always detected. So, according to V. Connor and co-authors. (1972), papillary abscesses occur in 50%, subepidermal blisters - in 61%, "nuclear dust" in the upper parts of the dermis - in 77% of cases.

Histogenesis

The mechanism of bubble formation is unclear. Direct immunofluorescence test in this disease reveals deposits of IgA in the dermoepidermal junction of the unaffected skin and in the foci of foci at the beginning of the process, mainly at the apex of the papillae of the dermis, and also inside them. In some cases, deposition of IgG is observed, rarely IgM. Antithyroid antibodies, antibodies against parietal cells of the stomach and IgA-nephropathy have also been detected. In recent years, the importance of antibodies against gliadin has been studied. Reticulin and smooth muscle endomisis. The specificity of IgA for gliadin has been shown, but their frequency with herpetiform dermatitis is low, therefore, it has no diagnostic value. The sensitivity and specificity of antireticulin antibodies and antibodies against Enlomizium have been established. In most patients, the production of antibodies (IgA) provokes an ingested gluten antigen, contained in gluten meal and cereal foods; it also causes disease-specific enteropathy. The association of the disease with some antigens of the HLA system is observed: HLA-B8, DR3, etc. The HLA-B8 / D3 haplotype occurs in patients with herpetiform dermatitis of Dühring several times more often than in the control.

25-35% of patients with herpetiform dermatitis of Dühring detect circulating immune complexes, which gives grounds to attribute this disease to immunocomplex.

Symptoms of Durings herpetiform dermatitis

The majority of patients are middle-aged and elderly, rarely children.

Clinical manifestations of the disease are polymorphic, there are erythema-edematous (urticaro-like), papular, papulevezikuleznye, vesicular and less often (mainly in the elderly) bullous eruptions, accompanied by burning and itching. The rash is often located symmetrically on the skin of the extremities, mainly in the area of large joints, shoulders, buttocks. Typical is the tendency toward grouping, typically the development of hyperpigmentation in places of regressed rash. The atypical (eczematoid, trichophytoid, strobuloid, etc.), mixed (having signs of herpetiform dermatitis of Dühring and pemphigoid), clinical variants are also possible, and also petechial-ecchymosis purpura with localization on the skin of the palms. In atypical cases, as well as the development of the process in elderly people, paraneoplasia should be excluded. Symptom Nikolsky negative, increased sensitivity to iodine preparations. In the blood and the contents of the blisters, many eosinophils are found. The course of the disease is long, cyclical, with remissions and paroxysmal exacerbations. Mucous membranes are less affected than with pemphigus, mainly with IgA-linear bullous dermatitis, which is regarded as a similar process with classical herpetiform dermatitis of Dühring. The peculiarity of cases with the linear arrangement of IgA is the presence of clinical and morphological signs of herpetiform dermatitis Dühring and bullous pemphigoid. In children, similar manifestations are designated as a juvenile form of lgA-linear dermatosis, which, according to M. Meurer et al. (1984) is probably identical to the previously described benign bullous dermatosis in children.

Before the onset of rash, some patients have prodromal signs (general malaise, fever, tingling of the skin). The disease is characterized by true polymorphism and is represented by erythematous spots, urtikaropodobnymi papules, vesicles, blisters and pustules. Depending on the predominance of elements in the lesions, there are vesicular, erythematous, bullous and pustular types of clinical course of herpetiform dermatitis Dühring. But sometimes the rash is monomorphic.

Dermatosis is characterized by the appearance of rashes on the erythematous background, but sometimes on the clinically unchanged skin. Elements of the rash (spots, urtikaropodobnye papules, vesicles, blisters and pustules) differ from similar rashes in other dermatoses. Rounded erythematous spots have a small amount, a smooth surface, clear boundaries. Urtikaropodobnye elements and papules have fanciful and scalloped outlines with clear boundaries of pink and red coloration. On the surface of spots, urtikaropodobnyh elements and papules are visible excoriations. Hemorrhagic crusts and scales. Small vesicles (0.2-0.5 cm in diameter) appear on the edematous erythematous base and have a pronounced tendency to herpetiform arrangement (the second characteristic feature), a strained tire and transparent contents, which eventually becomes turbid and can become purulent. There is a vesicular form of dermatosis. Bubble sizes from 0.5 to 2 cm and more. The bubble cover is thick and thick, so they do not burst so quickly. They usually appear on the erythematous, slightly edematous background, but can develop on the externally unchanged skin. The contents of the blisters are usually transparent, rarely - hemorrhagic, and when infected - purulent. Often there is a combination of bullous and vesicular forms of the disease. Opening up, the bubbles form erosion with a wetting surface, on the periphery of which are visible scraps of tires of bubbles and bubbles. Bubbles usually have no tendency to peripheral growth. On the surface of erosion, crusts are formed, under which epithelization rapidly occurs, leaving areas of hyperpigmentation. Nikolsky's symptom is negative.

The third characteristic feature of During's herpetiform dermatitis is the presence of intense itching and burning, especially at the onset of the disease.

The disease proceeds with attacks, i.e., recurs at various intervals of time. Sometimes, in the most severe cases, rashes last for a long time without disappearing even under the influence of the therapy. The primary location of the rash is the extensor surfaces of the extremities, the scapula area, the buttocks, the sacrum, but the process can also capture any part of the body.

The defeat of the mucous membranes is not typical. In rare cases, there are vesicle-bullous elements. At the same time, surface erosions of an irregular shape are visible, along the circumference of which there are scraps of bubble covers.

For Dühring's disease, a cutaneous and internal test with potassium iodide (Yaddason's test) is of great diagnostic value. Eosinophilia is found in the blood and fluid. Acantholytic cells are always absent.

Herpetiform dermatitis in pregnant women (herpes gestationis, herpes of pregnancy) usually begins 3-4 months of pregnancy, but sometimes after childbirth. On the skin of the trunk and extremities against the background of erythematous-urticarial spots appear small cone-shaped vesicle or pustular elements. Usually there are generalized itching and widespread erythematous-vesicular rashes, accompanied by more or less pronounced common phenomena. Vesicles merge with each other, they are opened, their contents are poured into the crusts. Sometimes there may be bubbles with a dense coating. Mucous membranes are rarely affected. Relapse of the disease is noted during the next pregnancy.

In clinical practice, there is rarely a localized herpetiform dermatitis or herpetiform dermatitis of the Cottini type. The skin-pathological process is located in the area of elbows and knees, sometimes in the sacred area.

Diagnosis of herpetiform dermatitis Dühring

The disease should be distinguished from the bullous variety of multiforme exudative erythema, bullous pemphigoid, various forms of acantholytic pemphigus, bullous toxemia, vesicle variety of centripetal erythema Daria et al.

[14], [15], [16]

Treatment of herpetiform dermatitis Dühring

First, you must follow a diet: to exclude from the diet products that are rich in gluten. Treatment depends on the severity of the disease. Assign inside the preparations diamino-diphenylsulfone (dapsone, diucifon) to 0,05-0,1 g 2 times a day for 5-6 days with a three-day interval. In severe cases, glucocorticosteroids are recommended. The dose depends on the condition of the patient and the clinical picture of dermatosis (on average, 40-60 mg / day). Aniline dyes, corticosteroid ointments are used externally.