Content
Dühring's disease is considered a chronic skin disease. In another way, the disease is called dermatitis herpetiformis, which affects the area of \u200b\u200bthe dermis and epidermis, forming a characteristic rash. The recurrence of the pathology occurs at any age, accompanied by the appearance of erythematous spots, polymorphic vesicles with liquid like herpes and severe itching.
What is Dühring's dermatitis
Bullous, polymorphic or herpes dermatitis is otherwise referred to as Dühring's disease. The term refers to a chronic skin disease with polymorphic rashes. It is rare, only 1% of people, sometimes affects the mucous membranes (in 10% of cases). Herpetiform disease got its name from the name of the professor who first described its symptoms and causes at the end of the 19th century.
Causes of dermatitis
Until now, doctors do not know exactly what causes herpetic dermatitis. Modern research has established that a possible factor provoking Duhring's syndrome is an autoimmune reaction, disruption of the gastrointestinal tract. The reasons are reduced immunity, increased sensitivity to gluten from cereals and halogens. Endocrine changes (pregnancy, menopause) also affect the occurrence of herpetiform disease.
It has been proven that women suffer from dermatitis herpetiformis less frequently than men. The disease can affect adults 25-55 years old; diseases of the child and the elderly are extremely rare. The causes of herpetiform syndrome can be toxemia, vaccination, nervous, physical overwork and lymphogranulomatosis. Scientists have found that patients are sensitive to iodine, which gave an opinion about the allergic reaction of the body.
Forms of dermatitis
Depending on the predominant type of rash manifested on the skin, the following forms of herpetiform disease are distinguished:
- herpetic vesicular dermatitis- the rash is represented by vesicles with serous transparent contents;
- papular- the rash resembles acne with a red head, sometimes there may be pustules with a pronounced top;
- bullous herpetiformis- rashes are similar to vesicles, but larger in size;
- urticariform- rashes resemble a nettle burn in the type of spots.
Depending on the nature of the course of dermatitis, the following forms of it are found in clinical medicine:
- acute herpetiformis- characterized by a sudden onset of symptoms, a serious condition of the patient, fever, sleep disturbances, changes in blood counts;
- chronic herpetiformis- characterized by a change in periods of remission and exacerbation, remissions are long - up to a year.
Symptoms of Dühring's dermatitis
Dühring's dermatosis herpetiformis is characterized by a polyetiological syndrome - it develops against the background of other diseases (disturbances in the functionality of the small intestine or the formation of allergic reactions). The patient begins to feel itching, burning, tingling on the skin, bouts of scratching. Fever, deterioration of health may begin, and after a few hours, vesicular or papular spots of large diameter with cavities inside are poured out.
Rash herpetiformis is a combination of red bloodshot spots and inflated blisters. Edema occurs, papules, vesicular and urticaria-like "burns" are visible. Rashes are symmetrical - they can be found on the surfaces of the limbs involved in extension, buttocks, shoulders, lower back. Frequent spots on the face and scalp. If the face is affected by dermatitis, the mucous membrane and epithelium of the mouth also suffer. At first they swell, turn red, then they are covered with blisters and vesicular blisters.
The rashes are similar to herpes, which gave the name herpetiform disease. Three days later, the blisters open, form bright red erosions with jagged edges and mild pain. The ulcers stay in the mouth for about a crescent, are localized on the palate and cheeks, leave crusts. After the healing of the elements and the subsidence of symptoms, a remission occurs, after some time it returns again with a herpetiform exacerbation.
In the event of a complication of dermatitis, the patient experiences body aches, fever, pain in the joints and muscles. The appearance of these symptoms can provoke being in the cold wind, hypothermia, which is another factor in the similarity of the disease with herpes. The manifestation of herpetiform signs on the genitals and palms is extremely rare, while itching on the genitals may not be.
Diagnostics
Based on typical manifestations, the diagnosis of dermatitis herpetiformis is carried out. Dühring is diagnosed after examining the patient by the location of the blisters on the mucous membrane and face. Distinctive reactions of herpetiform disease are:
- negative symptom of Nikolsky;
- disease cycles;
- polymorphism of rashes;
- the absence of a certain type of cell (acantholytic);
- histological test according to Yadasson.
Eosinophilia is present in the peripheral blood of a patient with dermatitis, dermatologists study the contents of the blisters for serosity plus skin sensitivity to iodine. Children in the diagnosis of dermatitis may lack eosinophilia and iodine sensitivity. Analyzes show the presence of certain antibodies in the basement membrane of the affected herpetiform skin areas or in internal inflammatory foci.
The following forms of herpetiform disease are distinguished depending on the degree of differentiation of the blisters:
- true acantholytic pemphigus;
- nonacantholytic dermatitis;
- multiform exudative erythema;
- bullous toxicoderma.
Treatment of Dühring's dermatitis
The treatment of Dühring's herpetiform dermatitis should be complex. Use sulfonic drugs, vitamins, antihistamines, corticosteroids. In severe persistent disease, antibiotics are prescribed. To maintain the patient's condition, a diet is normally prescribed, which should prohibit gluten, as a prophylaxis of herpetiform infection, traditional medicine is used.
Medicines
Treatment of Dühring's dermatitis, depending on the form, is carried out with the following drugs:
- mild herpetiform type- taking sulfonic drugs (Dapson, DDS, Avlosulfon), Diucifon. Along with them, vitamins C, P, B, antihistamines are taken.
- Severe herpetiform type- oral corticosteroids are prescribed (Prednisolone, Dexamethasone solution), and in case of intolerance - sulfapyridines. To relieve itching in dermatitis, antihistamine medications are used in the form of ointments and tablets, and complex vitamins are used to support immunity. Experts put a positive prognosis for the cure of herpetiform disease.
ethnoscience
It is possible to treat Duhring's dermatitis herpetiformis with herbal medicine methods that have anti-inflammatory and antihistamine effects. Of the herbal components of decoctions, compresses or infusions, use:
- calendula;
- juniper;
- knotweed;
- mistletoe;
- licorice;
- sea buckthorn;
- aralia;
- leuzea.
Diet for Dühring's dermatitis
An important factor in complex therapy is the diet for Dühring's dermatitis. It limits the intake of products with rye and wheat. Detailed recommendations on the types of food, diet of a patient with herpetiform dermatitis are presented in the table:
|
Product group |
Can eat |
Forbidden |
|
Bread, cereals |
Corn flour, rice flour, starch |
Wheat, barley, rye flour |
|
Meat, fish, poultry without fat, oven-cooked, milk, dairy products except yogurt |
Sausages, smoked meats, dumplings, pasties, meat pies |
|
|
Any, except cereals, legumes, cabbage |
Legumes, cabbage, cereals |
|
|
Rice, corn, buckwheat |
Semolina, barley, pasta, noodles |
|
|
Vegetables, broths, puree |
With peas, lentils, beans, vermicelli |
|
|
No restrictions |
||
|
Mineral water without gas 1.5-2 liters per day, tea, coffee |
Kvass, coffee, chicory, beer |
Video: Dühring's dermatitis
Photo of dermatitis herpetiformis
Attention! The information provided in the article is for informational purposes only. The materials of the article do not call for self-treatment. Only a qualified doctor can make a diagnosis and give recommendations for treatment, based on the individual characteristics of a particular patient.
Did you find an error in the text? Select it, press Ctrl + Enter and we'll fix it!Dühring's disease, or bullous dermatitis herpetiformis, is a dermatosis that is characterized by a primary polymorphic rash (true polymorphism), severe itching, burning sensation, paresthesias, and a chronic course with periodic relapses and remissions.
Disease prevalence
The available data of various authors are ambiguous. The number of patients per 100 thousand population ranges from 11.3 to 75.5. Moreover, in accordance with the studies of some authors, men are somewhat more likely to get sick, according to others - women. Dühring's dermatitis herpetiformis occurs mainly in the northern European countries among the adult population of predominantly middle age (30-40 years), somewhat less often among older people. In children, this pathology is quite rare, but among them, about 10% are infants.
Causes and mechanism of development of pathology
The disease has been known for more than 130 years, but its causes and pathogenesis have not yet been fully established. In 1966 and in subsequent years, reports began to appear of the identification of celiac disease among many patients with dermatitis herpetiformis. Celiac disease, or gluten-sensitive enteropathy, is damage to the villi of the epithelium of the small intestine by the protein gluten or similar hordein, avenin, etc., contained in the gluten of cereal plants - barley, wheat, rye, oats. This pathology of the intestine is often accompanied by symptoms of malabsorption.
genetic theory
According to the results of family studies in 4-7% of patients with Duhring's disease, the closest relatives with the same disease were identified and a significantly higher percentage - with the presence of relatives suffering from celiac disease. In addition, monozygotic twins were identified, one of which suffers from gluten-sensitive enteropathy, and the second - from dermatitis herpetiformis.
All these and many other facts formed the basis for the assumption of the genetic nature and similarity between these two pathological conditions. Confirmation is the presence in 90% of such patients in the sixth chromosome of various forms of the same gene, which determine the variant of the development of the disease (Dühring's dermatitis or celiac disease).
Autoimmune theory of the pathogenesis of dermatitis herpetiformis
Serological examination of the affected areas of the skin using the method of direct immunofluorescence in most patients revealed fixed deposits of immunoglobulin "A" (IgA) at the junction of the epidermis with the dermis or in the papillary layer of the skin. IgA is an antibody and is located in the form of granules in the area and inside the dermal papillae, located near the basement membrane.
These antibodies are directed against the structural components of the papillae of the dermis. Some patients have gluten-IgA complexes, which also circulate in the blood. According to various data, in 30-100% of patients with dermatitis herpetiformis without gastrointestinal symptoms in the mucous membrane of the small intestine, partial or complete atrophy of the villi of the epithelium of the mucous membrane of the small intestine is found, similar to celiac disease.
In this regard, the most widespread is the autoimmune theory of the pathogenesis of the disease, according to which the mechanism for the development of pathology lies in autoimmune tissue damage.
Provoking factors
Thus, it is assumed that the genetic cause of the disease is realized through an autoimmune mechanism in the presence of provoking factors - background diseases and disorders in the body. The main ones are:
- The use of a relatively significant amount of flour products and dishes with pearl barley, semolina and barley groats, starch, which contain gluten and similar protein components, as well as increased sensitivity to iodine, iodine preparations and seafood (contain iodine). In this regard, the diet for Dühring's dermatitis is the basis of pathogenetic therapy.
- Allergic reactions to vaccines and/or drugs (less common).
- Autoimmune diseases of the connective tissue.
- Acute or chronic thyroiditis.
- Diabetes mellitus type I.
- Malignant tumors.
- Blood diseases (lymphogranulomatosis).
- Physiological changes in the endocrine system (during puberty, pregnancy, menopause).
- Viral infections, helminthic invasions and intoxication of the body.
- Irradiation with ultraviolet rays and stress conditions.
Symptoms of Dühring's dermatitis
The nature of the flow
The disease begins gradually, can last for several weeks and even months and is accompanied by moderate itching and burning of the skin, which are precursors. Acquiring a chronic course, dermatitis herpetiformis is interrupted by periodic remissions, the duration of which ranges from 3 months to 1 year. Relapses can last for years.
The onset is manifested by a moderate increase in temperature and subjective sensations in the form of general malaise, loss of appetite, burning and tingling of the skin.
Description of the rash
Typical for skin rashes in this pathology is true polymorphism, due to a combination of different primary (erythema, nodules, papules, blisters) elements with further addition of false polymorphism in the form of a combination of secondary elements (erosions, crusts and excoriations). The appearance of a rash is always accompanied by a burning sensation and intense itching.
Erythematous spots have a fairly clear rounded outline and, as a rule, small sizes. If puffiness develops, they rise somewhat above healthy skin, and the surface becomes smooth and rich pink-red.
Then, gradually, due to the "sweating" of the liquid part of the blood through the walls of the dilated vessels, an urticarial (nodular) rash forms against the background of spots. All this looks like hives.
Urticarial elements in Dühring's dermatitis tend to grow in a peripheral direction and merge with each other, resulting in the formation of large bluish-pink foci with clear boundaries that have bizarre, scalloped or (less often) regular rounded outlines. The surface of the foci is covered with individual vesicles (vesicles), serous and bloody crusts and excoriations (traces of scratching). The foci themselves tend to merge with the formation of rings with a diameter of up to 20-30 mm, arcs, figures with bizarre outlines and garlands.
Vesicles are small (no more than 2-3 mm). They can appear both on spots and against the background of a visually healthy skin surface. The vesicles are covered with a dense tire and contain serous fluid. If they are grouped, they resemble a rash with herpes simplex. As a result of their drying, a light crust is formed. But more often, their contents gradually become cloudy and as a result of the addition of a secondary infection (especially when scratching), a pustule with purulent contents is formed.
After the opening of the vesicles, erosive surfaces are exposed that are not prone to fusion, which are covered with a bloody crust and quickly epithelialize. If rashes appear in the form of blisters (bulls), then they are characterized by the same clinical and evolutionary development as for vesicles. Their difference lies only in a different size, reaching a diameter of 5-20 mm in blisters. In the contents of vesicles and blisters, a large number of eosinophils are often found from the first days or somewhat later.
The rashes are mainly symmetrical in nature and are localized on the scalp, on the skin of the face and back of the neck, on the arms and legs (extensor surface), in the area of the shoulder, elbow and knee joints, on the skin of the lumbosacral and gluteal regions. Uncharacteristic for dermatitis herpetiformis is the defeat of the mucous membranes, but according to some authors, it can be detected in 50% of patients. In exceptional cases, erythema, vesicles or bullae on the oral mucosa subsequently transform into erosion.
Depending on the predominance of the primary elements of the rash, the following main forms of dermatosis are conditionally distinguished:
- papular;
- urticoid;
- vesicular;
- pustular.
After the rash resolves, peeling occurs in its place, gradually disappearing areas of hyperpigmentation or depigmentation.
Diagnostics
The diagnosis is established on the basis of:
- medical history data - the presence of close relatives with Dühring's dermatitis or celiac disease, the development of the disease or its exacerbation after taking gluten- or iodine-containing products or drugs;
- the nature of the rash - true and false polymorphism, the specifics of localization, symmetry, a tendency to merge and group elements;
- severe itching and burning that accompanies the rash;
- the presence of eosinophilia in the blood and / or fluid of the blisters; at the same time, its absence does not exclude pathology;
- a positive Yadasson test, which consists in applying a 50% potassium iodide ointment on the skin of the forearm in the form of a compress for 1 day;
- histological data - the presence of blisters under the epidermis with significant accumulations of eosinophils and neutrophils at the top of the dermal papillae, dilated dermal blood vessels; the latter are surrounded by infiltrates consisting of clusters of eosinophils and fragments of destroyed nuclei ("nuclear dust") with neutrophilic leukocytes;
- detection in the area of the basement membrane of IgA (when conducting a direct immunofluorescent reaction).
Features of the clinical course in children
In most children, Dühring's dermatitis develops after an infectious disease. The onset of the disease, as a rule, is acute with a temperature rising to 39 ° during the week, and severe symptoms of general intoxication - lethargy or, conversely, agitation, lethargy and lack of appetite.
On the head, neck, torso (back, anterior surface of the chest, abdomen, buttocks), on the skin of the extremities (with the exception of the palmar and plantar surfaces), edematous foci appear symmetrically, against which other types of rash quickly appear. Especially often rashes are localized in the area of the external genital organs and in large skin folds.
Bullous dermatitis herpetiformis is characterized by larger elements than in adults, their more frequent suppuration and localization on the mucous membranes of the oral cavity, as well as a rarer content of an increased number of eosinophils in the contents of the blisters. In children, there is a predominance of common forms of localization of the rash with the development of polyadenitis (an increase in multiple lymph nodes), but a lesser tendency to group and merge elements. How to treat the disease?
Treatment of dermatitis herpetiformis Duhring
The complex method of treatment includes the recommendation of a diet that provides for the restriction or exclusion (during periods of relapse) of the above foods, as well as cabbage, legumes, bean pods, bread kvass, beer, salt, seafood, sausages, chocolate, ice cream. Recommended dishes with rice, buckwheat, corn, the use of an increased amount of fruit, from drinks - black and green tea, natural coffee, mineral water with a low content of iodine and bromine.
Treatment of Dühring's dermatitis with folk remedies includes mainly external agents that help reduce itching and inflammation - baths with infusion of St. , oils and ointments containing extracts of the same plants.
Of the drugs, the most effective are drugs of the sulfone series (dapsone, diaphenylsulfone, DDS, aulosulfone, sulfapyridine, diucifon, etc.) according to a certain scheme as drug monotherapy. In cases of prolonged or severe course, they are combined with glucocorticosteroid agents.
RCHD (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical Protocols of the Ministry of Health of the Republic of Kazakhstan - 2017
Dermatitis herpetiformis (L13.0)
Dermatovenereology
general information
Short description
Approved
Joint Commission on the quality of medical services
Ministry of Health of the Republic of Kazakhstan
dated June 29, 2017
Protocol #24
Dermatitis herpetiformis(dermatitis herpetiformis Duhring
; Duhring's disease) inflammatory skin disease associated with celiac disease and characterized by polymorphic itchy rashes, chronic relapsing course, granular deposition of IgA in the papillae of the dermis.
INTRODUCTION
ICD-10 code(s):
Protocol development date: 2017
Abbreviations used in the protocol:
| DG | - | dermatitis herpetiformis |
| mutual fund | - | direct immunofluorescence |
| ELISA | - | linked immunosorbent assay |
| GKS | - | glucocorticosteroids |
| RCT | - | randomized controlled trial |
| PHC | - | primary health care |
| Ig | - | immunoglobulin |
| i/v | - | intravenously |
| i/m | - | intramuscularly |
| gr | - | gram |
| mg | - | milligram |
| ml | - | milliliter |
| rr | - | solution |
| tab | - | tablet |
Protocol Users: general practitioner, therapist, pediatricians, dermatovenereologist.
Evidence level scale:
| BUT | High-quality meta-analysis, systematic review of RCTs, or large RCTs with a very low probability (++) of bias whose results can be generalized to an appropriate population. |
| AT | High-quality (++) systematic review of cohort or case-control studies or High-quality (++) cohort or case-control studies with very low risk of bias or RCTs with low (+) risk of bias, the results of which can be generalized to the appropriate population . |
| FROM |
Cohort or case-control or controlled trial without randomization with low risk of bias (+). Results that can be generalized to an appropriate population or RCTs with very low or low risk of bias (++ or +) that cannot be directly generalized to an appropriate population. |
| D | Description of a case series or uncontrolled study or expert opinion. |
| GPP | Best Clinical Practice. |
Classification
Classification:
There is no generally accepted classification.
Depending on the predominant type of rash, a number of clinical forms of HD are distinguished:
bullous;
· herpes-like;
· urticaria-like;
trichophytoid;
· strophulose;
eczematoid.
Note: if herpetic vesicular dermatitis develops under the influence of carcinogenic tumors, then it is often called paraoncological dermatosis.
Diagnostics
METHODS, APPROACHES AND PROCEDURES FOR DIAGNOSIS AND TREATMENT
Diagnostic criteria
Complaints and anamnesis:
Complaints about:
· skin rashes;
Itching of varying intensity (pronounced, moderate);
burning sensation.
From the anamnesis:
skin diseases in the family in the past and present;
The onset of the disease and its course (duration, number of exacerbations);
The effectiveness of previous therapy and other factors.
Physical examination:
General status:
Inspection and assessment of the general properties of the skin;
examination of the tonsils, auricles, nose, eyes;
Assessment of the state of peripheral lymph nodes, lungs, circulatory system, digestive tract, kidneys, liver (palpation, percussion and auscultation).
Local status:
The nature of the skin lesion (by morphology - exudative, by the course of the skin process - chronic);
· localization;
Rash elements (erythema, papule, blisters, blisters);
dermographism.
Main subjective and objective symptoms:
Chronic, relapsing course
polymorphism of rashes (spots, blisters, papules, blisters);
tendency to group
The symmetry of the rashes;
Predominant damage to the extensor surfaces of the upper and lower extremities, scalp, knees and elbows, sacrum and buttocks;
The characteristic primary morphological element of the rash is a bubble of various sizes, usually with a dense cover, with serous or turbid, sometimes hemorrhagic contents, which occurs against a hyperemic edematous background. After opening, erosion is formed, which quickly epithelializes, leaving hyperpigmentation;
Nikolsky's negative symptom.
Llaboratory studies (LE-B):
To make a diagnosis, the following laboratory tests are required.
Cytological examination: determination of the number of eosinophils in the contents of the blisters (eosinophilia);
· Histological examination of a skin biopsy (obtained from a fresh lesion containing a cavity - bladder): identification of the subepidermal location of the cavity and determination of the content of fibrin, neutrophilic and eosinophilic leukocytes of the skin in it;
PIF: detection of granular deposits of class A immunoglobulin in the papillary dermis in a skin biopsy;
ELISA: determination of the content of IgA antibodies to tissue transglutaminase and the content of IgA antibodies to endomysium in the blood serum.
Instrumental Research: No.
Indications for expert advice:
consultation of a gastroenterologist - in the presence of diseases of the gastrointestinal tract;
consultation of a neuropathologist - in the presence of diseases of the nervous system;
consultation of a therapist - in the presence of concomitant diseases;
consultation of an oncologist - in case of suspected oncological diseases.
Diagnostic algorithm:(scheme)
Diagnostic Algorithm
| Diagnostic indicators | Evaluation of the indicator and conclusion | ||
| 1 |
a) Polymorphic rash (spots; blisters, papules, blisters); a-1) blisters of various sizes, usually with a dense cover, with serous or cloudy, sometimes hemorrhagic contents; a-2) erosions quickly epithelialize, leaving hyperpigmentation; b) a tendency to group; symmetry of the rashes; c) characteristic localization (primary lesion of the extensor surfaces of the upper and lower extremities, scalp, knees and elbows, sacrum and buttocks); d) severe itching; e) Nikolsky's symptom is negative; |
There are all listed clinical signs and symptoms (a (1.2), b, c, d, e). Conduct a study of indicators No. 2 and / or 3. |
There are some listed clinical signs and symptoms (two or three out of five (a, b, c, d, e). Conduct a study of indicators No. 2,3,4,5. |
| 2 | Determination of the amount in the contents of the blisters (eosinophilia) |
The result is positive. If all of the listed clinical symptoms and signs are present, the diagnosis of HD is considered confirmed. |
The result is negative. Conduct a study of indicators No. 3,4,5. |
| 3 | Identification of the subepidermal location of the cavity and determination of the content of fibrin, neutrophilic and eosinophilic leukocytes in it by histological examination of a skin biopsy obtained from a fresh lesion containing a cavity (bladder) | The result is positive. If all of the listed clinical symptoms and signs are present, the diagnosis of HD is considered confirmed. |
The result is negative. Conduct a study of indicators No. 2,4,5. |
| 4 | Detection of granular deposits of immunoglobulin class A in the papillary dermis in a skin biopsy using the PIF method |
The result is positive. Compare with the results of indicators 2,3,5. |
The result is negative. Compare with the results of indicators 2,3,5. The diagnosis of HD is made after comparison with all diagnostic indicators |
| 5 | Determination of the content of IgA antibodies to tissue transglutaminase and the content of IgA antibodies to endomysium in blood serum by ELISA |
The result is positive. Compare with the results of indicators 2,3,4. |
The result is negative. Compare with the results of indicators 2,3,4. The diagnosis of HD is made after comparison with all diagnostic indicators |
Differential Diagnosis
Differential diagnosis and rationale for additional studies:
Differential diagnosis of HD is carried out with pemphigus vulgaris, Lever's bullous pemphigoid, bullous form of toxicoderma, linear IgA-dermatosis, as well as with atopic dermatitis, scabies, papular urticaria in children.
| Diagnosis | Duhring's dermatitis herpetiformis | Pemphigus vulgaris | Lever's bullous pemphigoid | Erythema multiforme exudative | Subcorneal pustular dermatosis |
| Rationale for differential diagnosis |
- severe itching; - polymorphic rash (spots; blisters, papules, blisters); - propensity to group; - symmetry of rashes; - predominant damage to the extensor surfaces of the upper and lower extremities, scalp, knees and elbows, sacrum and buttocks; - a characteristic morphological element is blisters of various sizes, usually with a dense cover, with serous or cloudy, sometimes hemorrhagic contents. - erosion quickly epithelializes, leaving hyperpigmentation; Nikolsky's symptom is negative. |
- monomorphic rash; - a characteristic morphological element is intraepidermal blisters with a thin, flaccid lid, which quickly open; - the first rashes most often appear on the mucous membranes of the mouth, nose, pharynx and / or the red border of the lips; - pain when eating, talking, swallowing saliva. Characteristic signs are hypersalivation and a specific smell from the mouth; - bubbles and erosion tend to merge and grow peripherally; - Nikolsky's symptom is positive; - acantholytic cells are found in smears of prints. |
- no intense itching; - subepidermal blisters tense with a dense cover; - no tendency to group; - more often localized in the area of folds; - PIF method - deposition of Ig G in the area of the basement membrane. |
- blisters form on the mucous membranes, which open with the formation of painful erosions; - along the periphery of the spots and / or edematous papules, an edematous roller is formed, and the center of the element, gradually sinking, acquires a cyanotic hue (a symptom of a "target", or "iris", or "bull's eye"); - rashes tend to merge, forming garlands, arcs; - the appearance of a rash is accompanied by a deterioration in the general condition: malaise, headache, fever. |
- superficial pustules - conflicts with a flabby tire; - histologically, blisters are determined directly under the stratum corneum. |
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Chronic recurrent skin lesions, manifested by a polymorphic rash in the form of erythematous spots, blisters, papules, blisters and accompanied by severe itching and burning. The disease got its name due to the fact that the elements of the rash with Dühring's dermatitis are grouped in the same way as rashes with herpes. Diagnosis is carried out using histological examination, analysis of the contents of the bubbles and direct immunofluorescence reaction. In the treatment of Dühring's dermatitis herpetiformis, the sulfonic group of drugs and corticosteroids are effective.
General information
Duhring's dermatitis herpetiformis occurs at any age, but most often it develops in 30-40 years. Men are more susceptible to this disease than women. In some cases, Duhring's dermatitis herpetiformis is a skin reaction to a malignant tumor of the internal organs in the body, that is, it acts as a paraoncological dermatosis.
Causes
The causes and mechanism of development of Dühring's dermatitis herpetiformis are unknown. Many patients have an intolerance to the gluten protein found in cereals. In favor of the autoimmune component in the development of the disease is the detection of IgA antibodies at the border of the dermis and epidermis - in the area of the basement membrane. It is assumed that increased iodine sensitivity, heredity, ascariasis, inflammatory processes of the gastrointestinal tract (gastritis, peptic ulcer), viral diseases (ARVI, herpes infection, etc.) play a certain role in the occurrence of Dühring's herpetiform dermatitis.
Symptoms
Usually, Duhring's dermatitis herpetiformis has an acute onset with the appearance of foci of a polymorphic rash. Rashes may be preceded by a moderate rise in body temperature, general weakness, itching and tingling. Rash elements can occur on any part of the skin, except for the soles and palms. But their most common location is the extensor surfaces of the arms and legs, the area of the shoulder blades, shoulders, lower back and buttocks. On the palms, petechiae and ecchymosis can occur - large (more than 3 mm) spots of intradermal hemorrhages. Rashes are accompanied by severe discomfort: burning sensation, intense itching and paresthesia. The defeat of the mucous membranes with Dühring's herpetiform dermatitis, as a rule, is absent. In rare cases, blisters may appear in the oral cavity, quickly turning into erosion.
The true polymorphism of the rash in Dühring's dermatitis herpetiformis is associated with the simultaneous appearance on the skin of various sizes of erythematous spots, blisters, papules and blisters. Over time, a false one joins the true polymorphism: erosions and crusts associated with the transformation of rashes are formed, as well as excoriations caused by strong scratching of the skin. When the elements of the rash heal, foci of hypo- and hyperpigmentation remain on the skin, sometimes scars.
Erythematous spots in Dühring's dermatitis herpetiformis have a clear contour and a rounded shape. Their smooth surface is often covered with scratches, bloody and serous crusts. Over time, they are saturated with effusion from dilated vessels and turn into blister-like (urticaria-like) formations. The latter grow along the periphery and merge, transforming into pink-bluish foci covered with crusts, scratches and vesicles. Along with this, erythematous spots may turn into juicy pink-red papules. In addition, papules and urticaria-like lesions may occur without the erythematous patch stage.
Bubble elements of the rash with Duhring's dermatitis herpetiformis can be small in size - vesicles and more than 2 cm in diameter - bullous rashes. They are filled with a clear liquid, the turbidity of which indicates the addition of an infection. The blisters break open and dry out to form a crust. Due to combing, the crust is removed and erosion remains in place of the bubble.
Depending on the predominance of one or another type of rash over the others, the following types of Dühring's herpetiform dermatitis are distinguished: papular, vesicular, bullous and urticaria-like. Atypical variants of the disease are possible: trichophytoid, eczematoid, strophuloid, etc.
Acute periods of Dühring's dermatitis herpetiformis are combined with rather long remissions (from several months to a year or more). Exacerbations often occur with a deterioration in the general condition of the patient, a rise in temperature, and sleep disturbances.
Diagnostics
If Dühring's dermatitis herpetiformis is suspected, an iodine test of Yadasson is performed. A compress with an ointment containing 50% potassium iodide is applied to a healthy area of \u200b\u200bthe skin. The compress is removed after 24 hours. The detection of redness, vesicles or papules in its place speaks in favor of Duhring's dermatitis herpetiformis. If the test is negative, then it is repeated. To do this, after 48 hours, the same compress is applied to the area of pigmentation left after the former rashes. The Yadasson test can be performed with an oral iodine preparation. But such a study is fraught with a sharp exacerbation of the disease.
With Dühring's dermatitis herpetiformis, an increased content of eosinophils is detected in a clinical blood test. Cytological examination of the contents of the blisters also reveals a large number of eosinophils. However, these data, like the Yadasson test, are not mandatory or strictly specific for the disease.
The most reliable way to diagnose Duhring's dermatitis herpetiformis is histological examination of the affected skin areas. It reveals cavities located under the epidermis, accumulations of eosinophils, neutrophils and the remains of their destroyed nuclei. The direct immunofluorescence reaction (RIF) detects deposits of IgA. dermatologist on the tops of the dermal papillae. A diet is prescribed that excludes cereals and iodine-containing foods (sea fish, seafood, lettuce, etc.). Drug therapy is carried out with drugs of the sulfonic group: diaphenylsulfone, sulfasalazine, solusulfone and others. These drugs are usually administered orally in cycles of 5-6 days, with breaks of 1-3 days. In cases of ineffectiveness of sulfonic therapy, treatment is carried out with medium doses of corticosteroids (prednisolone, dexamethasone, etc.). Antihistamines are used to relieve itching: loratadine, cetirizine, desloratadine.
Local treatment of Duhring's dermatitis herpetiformis includes warm baths with a solution of potassium permanganate, opening the blisters and treating them with brilliant green or fucarcin, applying corticosteroid ointments or aerosols, and using 5% dermatol ointment.
Duhring's disease is a type of skin disease. To date, doctors have not been able to accurately determine the cause that provokes the development of this disease.
The main feature of Duhring's disease is that it has a long course, in which a rash appears on the surface of the skin in the form of vesicles and blisters, which are grouped into rings or garlands. Such a rash can be quite painful and will cause an unpleasant feeling of discomfort, accompanied by severe itching and burning.
Most often, people aged 15 to 60 suffer from Duhring's disease, while the peak of the disease occurs precisely in the age period from 30 to 40 years. Unlike women, men are significantly more affected
Symptoms
Most often, this disease is manifested by a slight increase in temperature, there is a feeling of weakness throughout the body. In the affected area, a slight tingling of the skin is felt and severe itching develops, accompanied by a burning sensation. Literally a few days later, a rash appears on the surface of the skin, which, by external signs, strongly resembles herpes.
Most often, such a rash begins to appear on the flexion parts of the body - for example, on the elbow, shoulder, legs, buttocks or lower back. In this case, the rash will never appear on the surface of the foot or palms. However, subcutaneous hemorrhages called petechiae may appear on the palms. In the most rare cases, a rash forms on the mucous membranes of the mouth, but this possibility should not be ruled out.
In the presence of Dühring's dermatitis, a diverse (polymorphic) rash can appear, which is formed in the form of red spots covered with a painful crust and causing severe itching or papules, the formation of transparent vesicles is also possible.
Inside the blisters there is a transparent or slightly cloudy content, which may contain a slight admixture of blood. Literally after 3 or 4 days, the bubbles can open, after which red erosion begins to form in their place. After healing, scars or hyperpigmented areas appear at the site of erosion.
Quite often, this disease is accompanied by such an unpleasant symptom as profuse fatty stools, which can have an unpleasant grayish tint. That is why, if you feel worse or the first signs of Duhring's disease appear, you need to seek help from an experienced doctor who, after examining the patient, will prescribe treatment.
In the future, Duhring's disease will be chronic, that is, constantly accompanied by recurrent seizures that manifest themselves throughout life. The period of remission will be quite short, during which dermatitis can go into the stage of a torpid course. In this case, a violation of the general condition of the patient occurs - immunity is significantly reduced, as a result of which there is a risk of developing various secondary diseases that greatly complicate treatment.
Diagnostics
In the event that there is a suspicion of the presence of Dühring's dermatitis herpetiformis, the doctor may conduct an iodine test of Yadasson. When conducting such an analysis, a special compress is applied to the affected area of the skin, in which an ointment is used that includes 50% potassium iodine in its composition. Such a compress must be worn for 24 hours, after which it is removed. If redness remains on the skin, then there is Duhring's disease.
But there are times when the result is negative, then a re-analysis may be prescribed. In this case, a second compress is applied to the area of pigmentation that remains after the rash, but not earlier than after 48 hours. It should be borne in mind that such an analysis as a result can lead to a sharp exacerbation of the course of the disease.
During the diagnosis of Dühring's dermatitis, the entire clinical picture of the course of the disease should be taken into account - these are subepidermal vesicles that have an edematous hyperemic base, may be accompanied by quite severe itching and an unpleasant burning sensation. Not only in the contents of these bubbles, but also in the blood, pronounced eosinophilia is observed. The patient begins to show hypersensitivity to iodine.
Modern laboratory methods can also be used to diagnose this disease. In the presence of Dühring's dermatitis, subepidermal vesicles appear, which in some cases may also be intraepidermal. In the event that there is an accumulation in the internal contents of fibrin vesicles and the presence of eosinophilic granulocytes, there is a risk of developing microabscesses.
Prevention
It is worth remembering that Duhring's disease is one of the chronic diseases that can be accompanied by relapses throughout a person's life. However, despite this, for most patients there is a very favorable prognosis, if you follow a few fairly simple recommendations for the prevention of this disease.
To prevent the possible development of relapse, it is necessary to pay special attention to your own diet. You should completely exclude from your diet products made from wheat, barley, oats, rye, and of course, you should abandon those products that include iodine in their composition - for example, sea fish, seaweed and much more. People suffering from Duhring's disease are strictly prohibited from using any medications that contain iodine.
This disease can proceed for several years, while periodically interrupting for small periods of stable remissions, the duration of which can vary from several weeks to months. There are cases when children who have entered puberty have a complete recovery.
As already mentioned above, it is necessary to strictly observe a simple diet, in which all products containing iodine are completely excluded.
Patients should constantly be at the dermatologist's dispensary observation, while undergoing periodic examinations, during which there is a risk of detecting the presence of oncological diseases.
Treatment
It is worth preparing for the fact that the treatment of Duhring's disease will take quite a long time. Before the doctor prescribes a course of treatment, the patient will have to be examined for the presence of diseases of the gastrointestinal tract, as well as oncological pathologies.
The most effective are the treatment methods during which derivatives of the sulfone series are used - these are diucifone, and diaphenylsulfone, as well as others. The drugs should be used in cycles of 5 days, during which short breaks of 1 or 2 days are taken (this is determined only by the attending physician).
During treatment, it is necessary to carry out a clear control of blood and urine tests, as there is a risk of side effects, which may include nausea, severe vomiting, aranulocytosis, and more. Comprehensive treatment should include the use of antioxidants, as well as unitiol.
Discussion and reviews (3)
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Larisa |
When my son was 10 years old, he developed Dühring's disease. Then we lived in the Crimea. No matter how many doctors we went to, no one could help us. Even the doctors couldn't make a diagnosis. Launched to such an extent that we could lose a child. He had a high temperature, the bubbles on his legs were bursting. And we were all ascribed some kind of red bastard. And then my husband's brother told us that we would urgently take him to Moscow. There he was admitted to a clinic, now I don’t remember which one. They immediately made a diagnosis, made some kind of white ointment, smeared it from head to toe, we stayed there for a month and things went to recovery. Now my son is 46 years old. After that incident, it never happened again. God bless! And when we returned from Moscow, our doctors were surprised. Doctors in the provinces do not know much. |
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