Severe course of acantholytic pemphigus in the practice of a pediatrician, pediatric dermatologist, allergist, pulmonologist. Meaning of acantholytic cells in medical terms

True or acantholytic pemphigus- bullous dermatosis, characterized by the formation of intraepithelial blisters on unchanged skin and/or mucous membranes as a result of acantholysis.

The disease has a long history chronic course with remissions varying degrees severity and varying duration.

There are 4 clinical forms acantholytic pemphigus:

vulgar,
vegetative,
leaf-shaped
seborrheic (erythematous).

What provokes Pemphigus vera (acantholytic):

Etiology unknown. There are viral and autoimmune theories of this disease. The leading role has now been proven autoimmune processes in the pathogenesis of true (acantholytic) pemphigus.

Pemphigus vulgaris, or vulgar, occurs much more often than other forms. Mostly men and women from 40 to 60 years old are affected; in childhood and adolescence it is very rare.

Symptoms of true pemphigus (acantholytic):

Most often, the oral mucosa is affected by ordinary (vulgar) pemphigus (in 75% of patients), so it is of greatest interest to dentists. Pemphigus vulgaris almost always begins with damage to the mucous membrane of the mouth or larynx, and then spreads to the skin. Even if the disease begins with skin lesions, lesions of the oral mucosa are almost always detected later.

Damage to the mucous membrane of the mouth and lips with pemphigus is characterized by the formation of single blisters with serous or hemorrhagic contents, having a very thin covering. Due to constant maceration in the oral cavity, the blisters open very quickly, so they are rarely seen. Along the periphery of the erosion, fragments of bubble tires are often found. In place of the blisters, painful erosions of a round, oval or elongated crack-like shape are formed, which do not heal for a long time. Erosions of bright red color are located against the background of unchanged or slightly inflamed mucous membrane. Their sizes in pemphigus vary - from a small abrasion to large surfaces of congestive red color. As a rule, there is no plaque on the surface of erosions, or there may be a thin layer of easily removable fibrinous plaque. Sometimes, instead of bubbles, white (greasy) films are formed, after which, when rejected, an erosive surface is exposed. With the progressive course of the disease due to the appearance of new blisters and pronounced acantholysis, the number of erosions and their size increase. When erosions merge, extensive lesions are formed, covering almost the entire mucous membrane of the mouth. Hypersalivation is possible. Most often, erosions are localized on the mucous membrane of the cheeks (especially in the retromolar region), the lower surface of the tongue, palate and the floor of the mouth. Sometimes lesions occur on the mucous membrane of the alveolar processes, transitional fold, lower and upper lips. In these cases, erosions epithelialize very slowly, even while taking large doses of corticosteroids.

In the absence of treatment, new erosions appear, which, merging with each other, form extensive erosive surfaces without a tendency to heal. The pain is quite severe, most intense when eating and talking. Erosions quickly become infected, especially in an unsanitized oral cavity. The addition of coccal, fungal flora and fusospirochetosis aggravates the patient’s condition, causing a specific foul odor from the mouth. Salivation intensifies. Saliva macerates the corners of the mouth, painful cracks appear. On the red border of the lips, in the corners of the mouth, blisters and erosions covered with hemorrhagic crusts are also possible. Sometimes hoarseness occurs, indicating damage to the larynx.

Blisters on the skin form mainly in places of friction with clothing (stomach, back, inguinal folds and etc.). After opening the blisters, very painful erosions remain on the skin. Any touch of clothing, linen or bandages to them causes sharp pain and forces the patient to remain motionless for hours.

Nikolsky's symptom in pemphigus is usually positive. There are three varieties of it:

if you grab the lid of the bladder or the upper layer of the epithelium at the edge of the erosion with tweezers and pull, then 37 Zak. 5491. Yu. M. Maksimovsky describes the detachment of the epithelial film on the apparently unchanged healthy mucous membrane and skin. The thin film of epithelium is very fragile and is easily cut with tweezers;
rubbing the unchanged mucous membrane or skin between the affected areas leads to the rapid formation of blisters or erosions;
if you rub areas located far from the affected area, the upper layers of the epithelium there will also peel off. The second and especially the third types of Nikolsky’s symptom indicate an increase in the intensity of acantholysis.

With pemphigus, in addition to the skin and mucous membrane of the mouth, other mucous membranes (intestines, stomach, esophagus, pharynx), as well as internal organs and the central nervous system can be affected.

Pemphigus is characterized by an undulating course, periods of exacerbation are followed by periods of remission, which rarely occur spontaneously, usually after treatment. In the absence of timely and necessary treatment the disease is steadily progressing. Rapid generalization of rashes on the skin and oral mucosa is possible, the general condition of patients worsens, weakness, malaise, loss of appetite, fever up to 38-39 ° C, and diarrhea appear; swelling lower limbs. Accession secondary infection accompanied by cachexia and intoxication. Without treatment the process ends fatal several months after the onset of the disease.

However, due to widespread use corticosteroids, such cases are now rare. Corticosteroid therapy interrupts the progressive course of pemphigus, and a stage of remission occurs.

Dühring's dermatitis herpetiformis characterized by a subepithelial arrangement of blisters. The blisters are small, tense, located on a swollen, hyperemic background, tend to merge, their formation is accompanied by burning and itching. Unlike pemphigus, rashes with Dühring's dermatitis are very rarely localized on the mucous membranes. Nikolsky's sign is negative, acantholytic cells are absent. The blisters contain a significant content of eosinophils, and there is also eosinophilia in the blood. In patients with Dühring's dermatitis herpetiformis, sensitivity to iodine is often increased.

In some cases, with pemphigoid, lichen planus and other blistering diseases with a subepithelial location of the blisters, around them or erosions, the upper layer of the adjacent epithelium can quite easily peel off. At the same time, the bladder cover is thick and difficult to rupture. This symptom is called false symptom Nikolsky, or a symptom of perifocal subepithelial detachment.

Benign non-acantholytic pemphigus of the oral mucosa differs from ordinary pemphigus by the localization of blisters only on the oral mucosa, subepithelial location, dense covering of blisters, often with hemorrhagic contents, and the absence of acantholytic cells.

Diagnosis of true pemphigus (acantholytic):

Diagnosis put on the basis clinical manifestations, positive Nikolsky symptom, results cytological examination and direct immunofluorescence reaction.

Cytological examination of smear impressions or scrapings from the bottom of erosions is mandatory for the diagnosis of pemphigus. The presence of acantholytic cells in them confirms the diagnosis of acantholytic pemphigus. Acantholytic cells, or Tzanck cells, which are modified cells of the spinous layer, have a round outline and are smaller in size than normal cells of the spinous layer. The nucleus is large relative to the entire cell, its diameter is y - y or more than the diameter of the cell, colored dark blue, often has from 1 to 6 light nucleoli or more. The cytoplasm of cells is heterogeneously colored: light blue around the nucleus and dark blue along the periphery. Acantholytic cells are characterized by polymorphism in color, size of cells and nuclei. There are giant multinucleated cells - “monsters”. At the height of the disease, the number of acantholytic and multinucleated cells increases sharply. They merge into a continuous conglomerate of polymorphic cells. During remission and during treatment with corticosteroids, the number of acantholytic cells decreases.

The cytological picture of pemphigus vegetans does not differ from that of pemphigus vulgaris. In seborrheic pemphigus, multinucleated cells, as a rule, are not found; acantholytic cells are found in smaller numbers, they are monomorphic.

Pathohistological studies. It has been established that the main morphological changes in acantholytic pemphigus are acantholysis and edema, resulting in the formation of intraepithelial blisters. The connections between the cells of the spinous layer are disrupted - the phenomenon of acantholysis, as a result of which the intercellular bridges melt, gaps and then bubbles form between the cells. The bottom of such blisters, as well as subsequently the surface of erosions, is lined predominantly with acantholytic cells.

Differential diagnosis

Acantholytic (true) pemphigus must be differentiated from other bullous lesions of the oral mucosa:

exudative erythema multiforme;
pemphigoid;
drug allergies;
bullous form of red lichen planus;
herpeti for a lot of Dühring's dermatitis;
benign non-acantholytic pemphigus of the oral mucosa only.

Differential diagnosis of acantholytic pemphigus with other diseases accompanied by the formation of blisters is based mainly on the location of the blisters in relation to the epithelium.

Thus, with exudative erythema multiforme, the blisters are surrounded by a zone of erythema along the periphery, located subepithelial, Nikolsky’s symptom is negative. In addition, exudative erythema multiforme is characterized by an acute onset, seasonal relapses, severe inflammation of the oral mucosa, and a short course.

With bullous pemphigoid, the blisters are located under the epithelium, their cover is thick, so their existence is longer. Bullous pemphigoid most often affects people over 60 years of age. Nikolsky's sign is negative, no acantholytic cells are detected.

Anamnesis (information about taking medications) and results help to distinguish acantholigic pemphigus from allergic drug stomatitis allergy tests. After discontinuation of the allergen drug, stomatitis quickly disappears. Bubbles in drug stomatitis are located under the epithelium, Nikolsky's symptom is negative, there are no acantholytic cells.

In the bullous form of lichen planus, the blisters are subepithelial, there is no acantholysis. There are multiple papules around the blisters or other areas of the oral mucosa, typical of lichen planus.

Treatment of Pemphigus vera (acantholytic):

Treatment of pemphigus currently includes corticosteroids, which are the mainstay of treatment for this disease. All other drugs, including cytostatics, are used to eliminate complications associated with taking glucocorticoids. The success of treatment with glucocorticoids depends on the timing of their use and dosages. The more correctly the doses of corticosteroid drugs are selected and the earlier their use is started, the greater the opportunity to achieve stable and long-term remission of the disease. Treatment of patients with acantholytic pemphigus should be carried out only in a specialized hospital.

For the treatment of patients with pemphigus, prednisolone, methylprednisolone (Metypred, Urbazone), dexamethasone (Dexazone), triamcinolone (Polcortolone, Kenacort) are prescribed in loading doses, which depend on the patient’s condition. Prednisolone is prescribed at 60-80 (up to 100) mg/day, triamcinolone at 40-80 mg/day, dexamethasone at 8-10 mg/day. So high, so called loading doses patients take it until the formation of new blisters stops and erosions are almost completely epithelialized, which is on average 10-15 days. Then slowly decrease daily dose prednisolone initially at 5 mg every 5 days, later these periods are extended to 7-10 days. When the daily dose reaches 20-30 mg, it is reduced very carefully. Subsequently, the daily dose is reduced until the minimum, so-called individual maintenance daily dose, which is administered permanently, is determined. For prednisolone it is usually 2.5-5 mg, for dexamethasone - 0.5-1 mg, every 4-5 days.

Long-term use of corticosteroid drugs causes side effects (increased blood pressure and glucose levels in urine, osteoporosis bone tissue, increased thrombus formation, etc.). In this regard, to reduce complications from corticosteroid therapy, dietary restriction is recommended. table salt, water. The diet should be predominantly protein with limited fat and carbohydrates. Potassium preparations are prescribed orally (potassium chloride, asparkam), ascorbic acid, B vitamins, calcium supplements, thyrocalcitonin.

Along with glucocorticoids, immunosuppressants are used in the treatment of patients with acantholytic pemphigus.

For the treatment of patients with acantholytic pemphigus use plasmapheresis and hemosorption, which help reduce side effect glucocorticoids and cytostatics, and also allow you to reduce their dose.

Local treatment, mainly aimed at preventing secondary infection of erosions and ulcers and accelerating their epithelization, includes painkillers in the form of oral baths; antiseptic drugs in non-irritating concentrations; applications to the oral mucosa or lubrication with corticosteroid ointments. After each meal and before applying corticosteroid-containing ointments, rinsing with warm, weak solutions of potassium permanganate, 0.25% chloramine, 0.02% chlorhexidine, etc. is necessary. Important To ensure rapid epithelization of erosions on the mucous membrane, careful sanitation of the oral cavity is necessary. If the red border of the lips is affected, applications and lubrication are carried out with ointments containing corticosteroids and antibiotics, as well as oil solution vitamin A. If pemphigus is complicated by candidiasis, it is prescribed antifungal drugs. To accelerate the epithelization of erosions and ulcers on the oral mucosa, laser therapy (helium-neon and infrared laser) is indicated.

However, even with the correct and timely treatment The prognosis for true (acantholytic) pemphigus remains serious. Patients who long time are taking corticosteroid medications and need sanatorium and resort treatment (gastrointestinal and cardiovascular). Insolation is strictly contraindicated for them.

Psoriatic triad

Application: for the diagnosis of psoriasis and differential diagnosis of similar diseases.

When scraping psoriatic papules (plaques) with a glass slide, a consistent triad of pathognomonic morphological signs is noted: “stearin spot phenomenon” - the appearance large quantity silver-white scales. This is reminiscent of the scales that appear when a drop of a stearin candle is scraped; “terminal film phenomenon” - after complete removal a shiny translucent film appears on the scales; “the phenomenon of pinpoint bleeding or blood dew” (Polotebnov’s or Auspitz’s symptom) - with further scraping of the film, droplets of blood appear on its surface due to the destruction of the capillaries of the papillary dermis.

With parapsoriasis, the following phenomena are observed:

Symptom of a “wafer” - when you carefully scrape the papule, the scales covering it are removed entirely, without breaking or forming small chips, as with psoriasis.

Symptom of purpura or Broca's symptom - after removal of the “wafer”, with continued scraping, small intradermal hemorrhages appear on the surface of the papule, which do not disappear with diascopy.

“Apple jelly” symptom and Pospelov’s sign

Application: for the diagnosis of lupoid tuberculosis of the skin.

Apple jelly symptom

When pressing with a glass slide on the surface of the tuberculous tubercle, the color of the tubercle changes. At the same time, under the pressure of the slide, the dilated vessels of the tubercle collapse, and the bloodless yellowish-brown color of the infiltrate, like the color of apple jelly, clearly appears.

Pospelov's or "probe" sign

Allows to identify pathognomonic diagnostic sign with tuberculous lupus. With light pressure on the surface of the tubercle with a button-shaped probe, it easily sinks into the depths of the tissue (Pospelov's symptom). For comparison, when pressing on healthy skin nearby, the resulting pit is restored faster than on a tubercle.

Nikolsky's symptom P.V. and Asbo-Hansen

Application: for the diagnosis of acantholytic pemphigus and the differential diagnosis of bullous dermatoses.

When you pull a piece of the bladder cover with tweezers, a detachment of the upper layers of the epidermis occurs in the form of a gradually narrowing band on an apparently healthy skin.
Friction with a finger (sliding pressure) on apparently healthy skin, both between the blisters and at a distance, also quite easily causes rejection (shifting) of the upper layers of the epidermis.

Note: this symptom also occurs in other skin diseases in which there is acantholysis (chronic benign familial pemphigus, etc.), but it is caused only in the lesion (Nikolsky’s regional symptom according to N.D. Sheklakov, 1967).

A variant of this symptom is the phenomenon of an increase in the area of the bladder when pressure is applied to its central part, described in true pemphigus by G. Asboe-Hansen.

Tzanck cell research

Application: for the diagnosis of pemphigus vulgaris and differential diagnosis of bullous dermatoses.

For monomorphic rashes of blisters on the skin and erosions on the oral mucosa of unknown origin, the fingerprint smear method is used for the possible detection of acantholytic cells (Pavlova-Tzanck) found in pemphigus vulgaris. Cytological feature true pemphigus acantholytic cells (Tzanck cells) should be considered used as a diagnostic test. Acantholytic cells are characteristic of pemphigus, but can also be detected in other diseases (herpes, chicken pox, bullous variety of Darier's disease, chronic benign familial pemphigus, etc.).

Detection technique: A piece of sterile student gum (but you can also firmly attach a fat-free glass slide to the surface of the erosion) is pressed firmly to the bottom of the fresh erosion and transferred to the slide. Usually several prints are made on 3-5 glasses. They are then air-dried, fixed and stained using the Romanowsky-Giemsa method (like regular blood smears). Acantholytic cells are smaller in size than ordinary cells, have a very large nucleus of intense purple or violet-blue color, occupying almost the entire cell. It contains two or more light nucleoli. The cytoplasm of the cells is strongly basophilic, around the nucleus it is light blue, and along the periphery it is blue or dark purple (“rim of concentration”). Often a cell has several nuclei. The polymorphism of cells and nuclei is sharply expressed. Acantholytic cells may be single or multiple. Sometimes there are so-called “monstrous cells”, characterized by their gigantic size, abundance of nuclei and bizarre shapes. At the beginning of the disease, acantholytic cells are not found in every preparation or are not detected at all; at the height of the disease there are many of them and “monstrous” cells appear.

Jadasson's test

Application: for the diagnosis of Dühring's dermatitis herpetiformis and the differential diagnosis of bullous dermatoses.

Sample with potassium iodide(Jadasson test) in two modifications: cutaneously and internally. On 1 cm2 of apparently healthy skin, preferably the forearm, an ointment with 50% potassium iodide is applied under a compress for 24 hours. The test is considered positive if erythema, vesicles or papules appear at the site of application. At negative sample after 48 hours it is repeated: now the ointment is applied to the pigmented area of the skin at the site of the former rash.

At negative result Prescribe 2-3 tbsp orally. 3-5% potassium iodide solution. The test is considered positive when signs of exacerbation of the disease appear.

Method for detecting scabies mites

Application: for diagnosing scabies.

A drop of 40% lactic acid is applied to the scabies element (tract, vesicle, etc.). After 5 mcn, the loosened epidermis is scraped off with a sharp eye spoon until capillary bleeding appears, slightly including the adjacent healthy skin. The resulting material is transferred to a glass slide in a drop of lactic acid, covered with a coverslip and immediately examined under a low magnification microscope. The result is considered positive if a mite, eggs, larvae, empty egg membranes, or at least one of these elements is detected in the preparation.

Examination of scales, hair, nails for pathogenic fungi

Application: for the diagnosis of dermatomycosis and differential diagnosis of similar diseases.

For research on pathogenic fungi Using a scalpel, scrapings are taken from the affected areas of the skin, mainly from their peripheral part, where there are more fungal elements. In case of dyshidrotic rashes, the covers of vesicles or blisters and scraps of macerated epidermis are taken with tweezers or cut off with pliers. Hair from the peripheral part of infiltrative-suppurative conglomerates or follicular nodular elements is also taken using a scalpel and tweezers. The changed areas of the nail plates, together with subungual detritus, are cut off with pliers.

For rapid diagnosis (within 1-30 minutes) of mycoses, quickly clearing compounds are used. Thus, skin scrapings after treatment with a 10% solution of sodium disulfide in ethanol in a ratio of 3:1 can be microscoped after 1 minute, nail sections - after 5-10 minutes.

Balser test(iodine test)

Application: for diagnostics versicolor and differential diagnosis of similar diseases.

When the affected areas and surrounding normal skin are lubricated with 3-5% tincture of iodine or a solution of aniline dyes, the lesions are colored more intensely. This is due to the greater absorption of the dye due to the loosening of the stratum corneum of the epidermis by fungi.

Symptom Unny-Darye

Application: for diagnosis of mastocytosis (urticaria pigmentosa).

When you rub the spots or papules of mastocytosis with a finger or a spatula for 15-20 seconds, they become swollen, rise above the surrounding skin, and their color becomes brighter. These phenomena are associated with the release of histamine from mast cell granules.

Allergy skin testing

Application: for the diagnosis of allergic dermatoses.

Most allergy tests are based on reproduction allergic reaction in a patient through exposure to the minimum required amount of allergen. Most often these reactions are carried out on the patient's skin. Initially, a drop or epidermal skin test is used with small dilutions medicinal product. If the droplet or epidermal test is negative, a scratch test is performed. At negative result With a scratch test, patch or intradermal tests are performed. It is not recommended to do skin testing while taking multiple medications at the same time. All tests, except for the provocative one, must be performed with a control, which is provided by solvents. Skin tests are contraindicated in acute period illnesses, severe concomitant diseases internal organs, nervous system, pregnancy, thyrotoxicosis, old age of the patient.

Drip: A drop of the test solution is applied to the skin (abdomen, inner surface of the forearm, back) for 20 minutes, and the sample area is outlined with ink. The result is taken into account after 20 minutes, 24-72 hours.
Appliqué(compress, patchwork): pieces of gauze (4-6 layers) measuring 1.5/1.5 or 2.0/2.0 cm, moistened with the test solution, are applied to the skin (abdomen, inner surface of the forearm, back), covered with compress paper, strengthened with adhesive plaster or bandage. The result is taken into account after 24-72 hours.
Scarification: A drop of the test substance is applied to the skin (abdomen, inner surface of the forearm, back) pre-treated with alcohol, through which scratches are made with a sterile needle or scarifier without the appearance of blood. The reaction is read after 10-20 minutes and 24-48 hours.
Intradermal: in the area of the skin of the flexor surface of the forearm, 0.1 ml of the test solution is injected strictly intradermally with a tuberculin syringe. The reaction is taken into account after 20 minutes and 24-48 hours.
Provocative: 1/4 of a single therapeutic dose of the test drug is given to the oral cavity, and the tablet or solution must be kept without swallowing. Reads in 10-20 minutes.

If an allergic reaction begins (swelling, itching, burning, rash), spit out the drug and rinse the mouth.

Accounting for allergic reactions.

1. Immediate (after 20 minutes):

negative - with a blister diameter of 6-7 mm;
weakly positive - with a blister diameter of 7-10 mm;
positive - when the blister diameter is over 10 mm.

2. Delayed (after 24-48 hours):

negative - papule 3 mm or erythema less than 10 mm in diameter;
weakly positive - papule 3-5 mm or erythema with edema 10-15 mm;
positive - papule more than 5 mm or erythema with edema more than 15-20 mm in diameter.

Skin biopsy

Application: for the diagnosis of dermatoses.

Choosing a biopsy site has great importance. A small morphological element can be taken as a whole. Cavity elements should be taken as fresh as possible; in case of lymphomas and granulomatous changes, the old element is taken, all others are biopsied at the height of development. Eccentrically growing elements and lesions are biopsied in the marginal zone. In the presence of several lesions that differ clinically, when the diagnosis depends on the result histological examination, it is advisable to collect from several places. The biopsy should always include subcutaneous fat.

Local anesthesia is carried out with a 0.5% solution of novocaine with the addition of a 0.1% solution of adrenaline (30:1). Subject to the rules of asepsis and antiseptics, a deep excision of the desired area is performed with a scalpel, capturing all layers of the skin. The wound is closed with 1-2 stitches, which are removed after 7-10 days.

The cheapest and most long-term way to fix (for months) the taken material is to immerse it in 10% water solution formalin (1 part 40% formalin solution and 9 parts distilled water).

Note: the biopsy is performed with the patient’s consent, which is noted in the medical history.

Shoe disinfection technique

With a cotton swab moistened with a 25% formaldehyde solution (1 part formaldehyde and 3 parts water) or a 40% solution acetic acid, wipe the insole and inner surface of the shoe. Then the shoes are placed in plastic bags for 2 hours. After airing for at least a day, the shoes can be put on. Stockings, socks, and underwear are disinfected by boiling for 10 minutes.

And a rim of basophilic cytoplasm; observed with acantholysis.

Big medical dictionary . 2000 .

See what “acantholytic cells” are in other dictionaries:

See Acantholytic cells... Large medical dictionary

- (A. Tzanck, 1886 1954, French dermatologist and hematologist) see Acantholytic cells ... Large medical dictionary

I Cell (cytus) is the basic structural and functional unit that determines the structure, life activity, development and reproduction of animals and plant organisms excluding viruses; elementary living system, capable of metabolism with... ... Medical encyclopedia

- (A. Tzanck, 1886 1954, French dermatologist and hematologist) see Acantholytic cells (Cell) ... Medical encyclopedia

Pemphigus vera- honey True pemphigus is accompanied by the appearance of blisters on unchanged skin or mucous membranes, which tend to generalize and merge. Frequency. Up to 1% of all skin diseases. Clinical picture and classification. Vulgar... ... Directory of diseases

Allergic stomatitis and dermatostomatitis, including their autoimmune forms, include the following main diseases: I. Chronic recurrent aphthous stomatitis and Behçet Touraine's syndrome. II. Exudative erythema multiforme and syndrome... ... Wikipedia

I Examination of the patient Examination of the patient is a complex of studies aimed at identifying the individual characteristics of the patient, establishing a diagnosis of the disease, justifying rational treatment, and determining the prognosis. Scope of research on O... Medical encyclopedia

Well-known dermatovenerologist, Honored Scientist of the RSFSR, Corresponding Member of the USSR Academy of Medical Sciences, Major General medical service. In 1919 he graduated from the Military Medical Academy and since 1921 he worked in the Clinic of Skin and Venereal Diseases of the Academy... ... Large biographical encyclopedia

I Darier's disease (J. Darier, French dermatologist, 1856 1938) is a hereditary dermatosis caused by a violation of keratinization of the epidermis. Inherited in an autosomal dominant manner. At the heart of D.'s development. there is a defect in synthesis and maturation... ... Medical encyclopedia

- (Greek pemphix, pemphigos bladder + eidos species; synonym parapemphigus) a disease characterized by the formation of subepithelial blisters on the skin and mucous membranes, reminiscent of the manifestations of pemphigus (Pmphigus). There are P. bullous and... ... Medical encyclopedia

Meaning of ACANTHOLYTIC CELLS in Medical Terms

ACANTHOLYTIC CELLS

(c. acantholyticae, synonym: tsanka cells) rounded cells of the spinous layer of the epidermis and epithelium of the mucous membranes, containing a large nucleus and a rim of basophilic cytoplasm; observed with acantholysis.

Medical terms. 2012

See also interpretations, synonyms, meanings of the word and what ACANTHOLYTHIC CELLS are in Russian in dictionaries, encyclopedias and reference books:

CHEST INJURIES in the Medical Dictionary:
CHEST INJURIES
Injuries chest make up 10-12% traumatic injuries. A quarter of chest injuries are severe injuries requiring emergency surgical intervention. Closed damage...
CELL in the Encyclopedia Biology:
, the basic structural and functional unit of all living organisms. Cells exist in nature as independent single-celled organisms (bacteria, protozoa and...
Pemphigus vera in the Medical Dictionary:
PEMPHIGOID BULLOUS in the Medical Dictionary:
Pemphigus vera in the Big Medical Dictionary:
True pemphigus is accompanied by the appearance of blisters on unchanged skin or mucous membranes, which tend to generalize and merge. Frequency. Up to 1%...
PEMPHIGOID BULLOUS in the Big Medical Dictionary:
Bullous pemphigoid is a benign chronic skin disease; primary element- a bubble that forms subepidermally without signs of acantholysis. Clinical picture - Appearance...
TZANKA CAGES in Medical terms:
(a. tzanck, 1886-1954, French dermatologist and hematologist) see Acantholytic cells ...
TZANCK CELLS in Medical terms:
see Acantholytic cells...
CYTOLOGY in the Great Soviet Encyclopedia, TSB:
(from cyto... and...logy), the science of cells. C. studies the cells of multicellular animals, plants, nuclear-cytoplasmic complexes that are not divided...
EXPERIMENTAL EMBRYOLOGY in the Encyclopedic Dictionary of Brockhaus and Euphron.
CYTOLOGY in the Encyclopedic Dictionary of Brockhaus and Euphron.
CENTROZOME in the Encyclopedic Dictionary of Brockhaus and Euphron.
CENTRAL NERVOUS SYSTEM in the Encyclopedic Dictionary of Brockhaus and Euphron.
CHARAL in the Encyclopedic Dictionary of Brockhaus and Euphron.
PHAGOCYTES
cells that have the ability to capture and digest solids. However, there appears to be no sharp difference between the entrapment of solids and liquids. At first …
PLANT TISSUE in the Encyclopedic Dictionary of Brockhaus and Euphron.
ANIMAL FABRICS in the Encyclopedic Dictionary of Brockhaus and Euphron.
SYMPATHETIC NERVOUS SYSTEM in the Encyclopedic Dictionary of Brockhaus and Euphron.
PROTOPLASMA OR SARCODE in the Encyclopedic Dictionary of Brockhaus and Euphron.
HEREDITY in the Encyclopedic Dictionary of Brockhaus and Euphron:
(physics.) - By N. we mean the ability of organisms to transmit their properties and characteristics from one generation to another, as long as the longest period lasts ...
EMBRYONAL LEAVES OR LAYERS
EXPERIMENTAL EMBRYOLOGY* in the Encyclopedia of Brockhaus and Efron.
CYTOLOGY in the Encyclopedia of Brockhaus and Efron.
CENTROZOME in the Encyclopedia of Brockhaus and Efron.
CENTRAL NERVOUS SYSTEM in the Encyclopedia of Brockhaus and Efron.
CHARAL in the Encyclopedia of Brockhaus and Efron.
PLANT PHYSIOLOGY in the Brockhaus and Efron Encyclopedia:
Contents: Subject F. ? F. nutrition. ? F. growth. ? F. plant forms. ? F. reproduction. ? Literature. F. plants...

Etiology and pathogenesis of true pemphigus. There are supporters of the viral theory. Positive RSC was detected in the cold with antigens prepared from the contents of blisters and sera of patients with pemphigus. A. T. Akopyan discovered a cytopathogenic effect in the blood serum and contents of the blisters of patients with pemphigus, which is probably due to the action of the virus. Using an electron microscope, virus-like formations were discovered in pemphigus cells. Mice, rats and rabbits infected with material from patients with pemphigus died due to symptoms of severe exhaustion with the development of paralysis.

Using a scanning microscope, the presence of T- and B-lymphocytes and unidentified bacteria was established directly on acantholytic cells, which are an antigenic factor. Using the method of indirect immunofluorescence in the serum of patients with pemphigus in active phase diseases, antibodies to the components of the intercellular substance of the epidermis were detected in a titer of 1: 120, 1: 240 when treated with luminescent serum against human IgG. With direct immunofluorescence N.Ya. Ezhov identified tissue-bound IgG in the intercellular areas of the epidermis. According to T. Nishikava, antibodies to the intercellular substance of the epidermis healthy person are present in extracts from homogenates of bullous eruptions in pemphigus. A correlation has been established between the titer of circulating antibodies to the intercellular substation of the epidermis and the severity of pemphigus.

The deposition of immunoglobulins, especially IgG, in the intercellular substance of the epidermis indicates the action of some infectious, viral or bacterial factor. The appearance of intraepithelial acantholytic blisters, identical to those of pemphigus, on the oral mucosa of monkeys as a result of repeated injections of serum or blistering fluid from pemphigus patients with high titers of intercellular antibodies supports this concept.

The successful use of glucocorticoids in the treatment of patients with pemphigus renewed interest in the endocrine theory. A correlation between the degree of suppression of adrenal cortex function and the severity and prevalence of the process has been proven. This corresponds to the results of the autopsy: in patients with pemphigus who had not yet been treated with hormones, atrophy of the adrenal cortex was noted.

The enzymatic theory of pemphigus pathogenesis is based on the fact that acantholysis is a consequence of increased activity of both proteolytic and glycolytic enzymes due to inhibition of the activity of their inhibitors as a result of the antigen-antibody reaction.

It is currently assumed that the leading factor in the formation of pemphigus is a pathological immune complex formed as a result of complex, deep metabolic, neuroendocrine and enzymatic disorders.

Pemphigus symptoms. Depending on the predominant clinical manifestations of the disease, they are distinguished 4 forms of true acantholytic pemphigus: vulgar (usual), vegetative, foliate (exfoliative) and seborrheic. In children, pemphigus is rarely observed, mainly between the ages of 2 and 15 years and mainly in girls.

Pemphigus vulgaris differs in a malignant torpid course and is observed in approximately 75% of cases in relation to the other three clinical varieties. Bullous rashes in the mouth are the usual initial manifestations of pemphigus, often precede skin lesions and provide the basis for recognizing the disease even before the appearance of skin rashes. P. Fabri and P. Panconesi, describing immune changes in 8 children from 31/2 to 16 years old suffering from pemphigus vulgaris, observed combined damage to the mucous membranes of the oral cavity, pharynx and genitals with widespread blisters on the torso. The blisters quickly open and form sluggish, slowly epithelial erosions.

Pemphigus vulgaris can occur in benign and malignant types. The benign course is characterized by rapid regeneration of the epidermis, the tendency of the process to spontaneous remission, or under the influence of steroid therapy. The general condition remains almost unchanged; severe visceral and neuroendocrine disorders are not observed. The malignant variety of pemphigus vulgaris in children, as a rule, does not occur, but if it does occur, it is characterized by asthenia, quick loss body weight, complications of secondary pyogenic infection. Rashes on the skin and mucous membranes are accompanied by septic fever, complications of the kidneys, heart, and lungs. In the blood - high ESR, eosinophilia, increased concentrations of sodium and chlorides against the background of decreased protein concentrations, especially gamma globulins and immunoglobulins.

Circulating antibodies against the intercellular substance of the epidermis with fixation of immunoglobulins (mainly IgG) and complement cause acantholysis. Its clinical expression is Nikolsky's symptom; peeling of the upper layers of the epidermis far beyond the boundaries of the bladder due to traumatic influence. A similar detachment of the epidermis also occurs when apparently healthy skin is rubbed at the location of the blistering rash. The acantholytic process can also be identified by the Asbo-Hansen symptom: with light pressure on the surface of the bubble, the liquid peels off the adjacent apparently healthy areas of the epidermis, and the size of the bubble increases before the eyes; If two bubbles are located next to each other, they merge.

Despite the great diagnostic value of Nikolsky's symptom, it is not considered pathognomonic, since it can also be observed in other diseases (congenital epidermolysis bullosa, Lyell's syndrome, etc.).

Detachment of the epidermis with subepidermal blisters is called a false Nikolsky symptom, or a symptom of pen and focal subepidermal detachment. Unlike the true Nikolsky symptom, it is caused only along the periphery of erosions. The true Nikolsky symptom is positive in patients with pemphigus only in the acute phase, and in other periods of the disease it can be negative.

Pemphigus vegetans. IN initial stage flaccid blisters that appear on apparently healthy skin, very similar to the blisters of ordinary pemphigus, quickly open, and papillomatous growths soon appear on the erosions covered with a grayish coating. Most often, rashes appear in large folds (axillary, inguinal, behind ears or in the navel area). When localized at the anus and in the vulva area, the vegetations acquire a fungoid, condylomatous character. Nikolsky's symptom is positive in the stage of disease progression. The latter is accompanied by pain and burning.

Leaf-shaped (exfoliative) pemphigus in children is observed somewhat more often. Suddenly, flabby, collapsed blisters appear, which, unlike pemphigus vulgaris, form on an erythematous base. The blisters quickly dry out without going through a full development cycle, forming lamellar, leaf-shaped crusts, under which fluid again accumulates, resulting in lesions with cortical accumulations resembling puff pastry. The process is prone to generalization like erythroderma, affecting the face, scalp heads and nails. Nikolsky's symptom is pronounced. Mucous membranes are rarely affected. Subjectively, itching, burning, and pain are noted. IN childhood pemphigus foliaceus is different severe course and a worse prognosis than in adults.

Seborrheic (erythematous) pemphigus - Senir-Usher syndrome often occurs without pronounced blisters. The disease begins in most cases on the face and then spreads to the scalp, chest, back and limbs. Layered massive crusts and scales on an erythematous-edematous background are located on the nose, zygomatic parts of the cheeks (“butterfly”). On the scalp, erythematous-squamous lesions resemble seborrheic dermatitis. On the chest and back, many lesions with blisters are surrounded by a hyperemic edematous border, and layered crusts, saturated with serous-purulent discharge, are similar to pemphigus foliaceus. In the area of the face and scalp, the manifestations are very similar to cicatricial erythematosis, but upon removal of the crusts, moist erosive surfaces are exposed and acantholytic cells are found in imprint smears from them. Nikolsky's sign near the blisters is often positive. Manifestations on the mucous membranes of the mouth and genitals are rarely observed. Seborrheic pemphigus is characterized by itching, burning and pain.

Pemphigus diagnosis. Pemphigus vulgaris is determined by the presence of blisters that appear on unchanged skin, which in children are often located on the body and torso. The diagnosis is confirmed by the positive symptoms of Nikolsky, Asbo-Hansen, and the detection of an IgG subulate layer fixed on the intercellular substance and antibodies to IgG in the blood during an immunofluorescence study.

Differential diagnosis is carried out with a number of dermatoses. With exudative erythema multiforme, the rashes are polymorphic, blisters on an edematous erythematous background are located on the extensor surfaces of the extremities. Along with the blisters, there are blisters and edematous papules with a slightly sunken, liquid center, reminiscent of a “bird's eye”. Erosions on the mucous membranes of the oral cavity are bordered by an edematous erythematous border, merge, and often spread to the red border of the lips and adjacent areas of the skin. Nikolsky's sign is negative, there are no acantholytic cells in the impression smears.

With Dühring's dermatitis herpetiformis, the rash is also polymorphic, with a characteristic herpetiform arrangement. Matter positive test Jadasson with ointment of 50% potassium iodide, eosinophilia in the blood and in the contents of the bladder, detection of immunoglobulin A at the epidermal border by immunofluorescence. Since in bullous pemphigoid blisters form under the epidermis, the fixation of IgG and complement fraction S3 is determined between the epidermis and dermis, and circulating autoantibodies against the basement membrane with a titer of 1:320 to 1:1280 are found in the plasma of patients.

In children, due to very imperfect immunity, looseness and immaturity of the cellular components of the epidermis, IgG fixation is possible both in the intercellular substance of the styloid layer and at the dermoepidermal border, and then they talk about the coexistence of pemphigus vulgaris and pemphigoid.

The bullous form of toxicerma, which occurs as Lyell's syndrome, is characterized by an acute, violent onset with high fever and general malaise. Against the background of rapidly spreading erythema, as well as on apparently healthy skin, flabby, thin-walled blisters with serous or serous-hemorrhagic contents form. The blisters open, forming weeping, quickly merging bleeding erosions, bordered by fragments of the epidermis.

Along with the skin, the mucous membranes of the mouth and genitals are affected. Nikolsky's symptom is sharply positive. However, acantholytic cells and IgG fixation on the intercellular substance of the spinous layer are not detected. Pemphigus vulgaris differs from simple blistering and herpes zoster by large blisters without a tendency to group and in a linear arrangement.

Diagnosis pemphigus foliaceus is supported not only by the positive Nikolsky symptom and the detection of acantholytic cells, but also by histological examination data, revealing severe acantholysis, fixation of IgG in the intercellular substance of the upper layers of the epidermis (usually even in the granular layer).

Pemphigus vegetans, when vegetations appear on erosive surfaces, they are differentiated from vegetative Hallopo pyoderma, in which there is a deep dermal infiltrate of the base of the blisters with the presence of strepto-staphylococcal flora in the contents, without acantholytic cells and IgG fixation in the styloid layer.

To be sure diagnosis of erythematous pemphigus Cytological and immunofluorescence studies are performed. The presence of acantholytic cells, IgG deposition in the intercellular areas of the Malpighian layer or at the level of the dermoepidermal border, a high titer of serum autoimmune antibodies (up to 1: 320) are sufficient grounds for diagnosis of seborrheic pemphigus.

Treatment of pemphigus. True acantholytic pemphigus represents one nosological form, therefore the treatment of patients is the same for all its clinical options. Special meaning It has early start treatment. Glucocorticoids are prescribed, especially to children, carefully selecting the maximum therapeutic dose, and minimal support. Typically, the initial dose of prednisolone or urbazone at the age of 5 to 15 years does not exceed 10-20 mg, dexamethasone - 2-3 mg per day. Triamcinolone is not recommended due to the risk of myasthenic phenomena. Since glucocorticoids have a catabolic effect, anabolic steroids such as Nerobol 0.0001-0.0002 g per 1 kg of body weight, Neroboletta, Dianobol, Retabolil must be prescribed in combination with them. To replenish microelements, calcium gluconate, panangin, potassium orotate, etc. are used. Worth attention new drug Senton, combining prednisolone with potassium, calcium, vitamins and methylandrostenediol.

Considering the inhibitory effect of glucocorticoids on immune mechanisms and the possibility of secondary infection, in complex therapy include antibiotics and immunostimulants (methyluracil, pentoxyl, thymosin, etc.). Symptomatic therapy prescribed individually according to indications, depending on general condition sick. Thus, to stimulate the adrenal cortex and reduce the dose of glucocorticoids, it is rational to use adrenocorticotropic hormone (ACTH) 10-20 units per day. Hemotransfusions, infusions of native plasma (50-100 ml), plasma substitutes (neocompensan, hemodez), and gamma globulin are also indicated. A. Haim and A. Shafrir (1970) showed that estrogens in combination with glucocorticoids can reduce the maintenance dose. However, prescribing cytostatics for this purpose in childhood is dangerous.

To activate the regeneration processes of the epidermis, a complex of vitamins is used: aevit, calcium pantothenate, riboflavin and folic acid. The amount of carbohydrates and sodium chloride in food should be limited, but the content of high-quality proteins, vitamins and microelements should be sufficient. External therapy combined with a well-organized sanitary and hygienic regime. To prevent secondary pyococcal infection, use medicinal baths with decoctions oak bark, chamomile, potassium permanganate solution, etc. On erosion, apply gentle disinfectants: Alibur liquid, 1-2% solutions of pyoctanin, gentian violet, methylene blue. After this, ointments with glucocorticoids are applied to the affected areas: locacorten, flucinar, oxycort, hyoxizone, lorinden C, dermozolon, etc.

Prognosis for acantholytic pemphigus. Complex rational treatment of patients with pemphigus using glucocorticoids allows in some cases to change the fatal course of pemphigus. We observed patients in a state of remission after discontinuation of maintenance doses of hormones for 3 to 12 years. The same optimistic information was published by N. S. Smelov and T. P. Mizonova and other authors. Of decisive importance for extending the period of remission and preventing relapses are dispensary observation and rational employment. Offer to supply every patient individual card(as is customary for people suffering cardiovascular failure). It must contain brief information about the nature of the disease and instructions about the regimen for taking glucocorticoid hormones if you have to provide emergency medical care to the patient (accident, emergency surgery, loss of consciousness).