Lecture of Pemphigus disease

2021-04-16 03:11 PM

Autoimmune disease, which has autoantibodies to circulating IgG in the blood against the keratinocytes cell surface, destroys the intercellular bonds that induce hydration in the epidermis.

Outline

Define

Pemphigus is a severe burns skin disease, which can cause human substance, progressing acute or chronic, is an autoimmune disease, blistering of the epidermis of the skin and mucous membranes that causes acantholyses.

Leaven

Autoimmune disease, which has autoantibodies to circulating IgG in the blood against the keratinocytes cell surface, destroys the intercellular bonds that induce hydration in the epidermis.

Relevant HLA - DR4, DQ8, DR6, DQ5.

Commonly aged 40-60.

Men and women are equally ill.

Self-antibody IgG binds to the epidermal cell surface glycoprotein and breaks the junctions leading to acantholyses.

Clinical form

Common Pemphigus (P. vulgaris)

A lesion is a blister on a single, healthy base of skin. But this skin is very easy to slip off. Nikolsky sign (+). Often there is a sudden mouth, especially on the dome of the mouth a few weeks, a few months ahead of the burn due to water scald there and fragile.

Blisters burn water a few centimetres in diameter, fragile and bland.

Clinical: a disease often occurs suddenly in a healthy person or in people who may collapse.

Mainly bullous: appears first somewhere on the skin or mucosa of the mouth, oropharynx. The bullae can be as small as green papaya seeds, apples, eggs, bullae and scalds, which are bland (not stretched), fragile, breakable, burn water that grows on healthy skin, after which it will flow easily. blood, squamous secretions.
There are cases where the bullae appear immediately enlarged.

There are cases when small and gradually enlarged due to the phenomenon of thorns.

It is possible that the bullae are linked 2 to 3 together with a large stretch of stretchable bullae (like Duhring) that can be wrinkled. It contains lemon yellow aqueous substances, sometimes becomes cloudy and can become real pus.

This blister appears on a normal-looking skin with no red background (Duhring can be seen on a red background). If the blister is superinfected, there is still a red background underneath.

Blisters are scattered on the skin most often on the limbs, through many progressions the bullae gradually spread to and spreads to the whole body, the bullae can even penetrate the nail around the nail causing inflammation around the nail which is difficult to diagnose.

Often wanted to diagnose Tzanck cells.

These blisters either dry or get wet and spread over the body to form crusts. When you look at it, you can see it slipping away, leaving a dark stain. And pus ulcers when slipping the skin leave ulcers sometimes in an area with diverse blisters, ulcerative sores, scaly ulcers.

But actually, the advanced stages of the disease only last 8-15 days per blister. Then break into a slit or a scaly secretion. Large blister blisters last for months when they do not leave scarring. Except for necrotic pemphigus when it is cured, leaving scars.

Signs of Nikolsky (+): for fingers to glide strongly on the skin, the skin slips under the hands due to broken bridges between the spiny cells. This marker is of great value but is only positive in exacerbations when it is still seen in Duhring.

Other mechanical properties Dšhring: At Dšhring there is an aura of itching, but when blisters have appeared, the patient feels burning pain. Sometimes severe burning pain if the slit spreads.

Mucous lesions in Pemphigus can be frequent, with the oral mucosa being first damaged.

Sometimes the first injury is on the lips, on the inside of the cheeks, there are irregular slips, sometimes very large, bright red may be on which there is a white film like a fake membrane on the gums so that when there is a slump around Roots of the mucous membranes in the palate are scattered with separate standing erosions or concentrated standing.

Other mucous membranes such as the pharynx, pharynx, nose, genitals, and synaptic membranes can also be damaged. Lesions in this mucosa make patients feel very pain and difficult to eat.

Systemic symptoms: the whole condition of the affected patient may not have a fever at first, then persistent fever may become intermittent, especially when there is an infection.

Gastrointestinal disturbances: appear at the end of the disease, anorexia, vomiting, diarrhoea, and mental disorder also appear. Oedema, possibly kidney damage.

Adrenal function: 17 ketosteroids in urine decreased, with adrenal damage detected during the autopsy.

Blood: leukocytes increased moderately.

The whole state of cachexia has lesions in the viscera.

Fluid disturbances, especially the metabolism of water, protein, and salt.

Progression is usually bad: death from 6 months to 2 years, progressing in waves, the condition getting a little better but it does not return to normal.

The patient died from cachexia and complications such as pneumonia, bronchitis, increased blood urea.

In the final stage, skin damage will be better, but death due to visceral damage, there is also a case of self-healing? Some gave neither benign pemphigus nor a variant of Duhring.

Histopathology: Blister is located in the epidermis mainly in the Malpighi layer. The spiny cells around the bladder are broken, connecting them, soaked in water, to swell to a large round nucleus called acantholyses.

Infiltrates are present in the dermis, but rarely.

About Tzanck cell diagnosis: is a valuable diagnostic method that allows for faster diagnosis than biopsy. Scraping the substrate burns water smeared on a microscope slide to see that the spine cells have broken the intradermal junction called Tzanck (+) cytology.

If the lesion in the mouth is very valuable to diagnose bullous scissors, use the tip of the pen to scrape off the bottom of the blister and spread it on a glass slide to be dyed, and see polygonal spiny cells.

If Duhring, on bronchoscopy, there were no papillary cells, but some cells in the dermis.

Visceral damage is only visible during the autopsy.

Lesions in many organs of the liver, kidneys, spleen, heart, bone marrow lymph nodes, lungs and secretory glands, especially the adrenal glands.

Pathogenesis: common disease in the frail elderly over 40 years of age occurs suddenly in healthy, non-contagious people, not of a family nature.

Histopathology shows:

Burns water in the epidermis.

Acantholyses is caused by the loss of junctions between epidermal cells.

The degeneration of some epidermal cells due to ferment proteclytique that dissolves the spiny cell bridges in the patient's skin.

Eosinophil infiltrates.

Direct immunofluorescence shows IgG and C3 deposited in the damaged area and the surrounding skin.

Blood: detection of autoantibodies to IgG. This autoantibodies against skin glycoprotein 130 K.

Direct skin immunofluorescence shows IgG and C3.

Immunofluorescence indirectly detects IgG in the patient's serum 75-90%, especially during the active phase of the disease.

Regarding the cause of the search for bacteria has not yet concluded: some theories about the virus, injecting water into the chicken lungs to incubate some dead chicken lungs, have some deformities but have not been clearly recognized.

There is a widely accepted autotoxique. A theory of endocrine factors is not clear. It is now clearly demonstrated that this is an autoimmune disease, the autoantigens are desmosomes in the dendritic cells, the autoantibody is IgG.

Differential diagnosis:

Duhring had a million in advance:

Red, painful. Variety of burning redness, oedema, bullous bullae, after application or oral administration of Ioduakali.

Special allergy to iodide:

Tetiodes (+).

Epidermolysis bulleuse:

A boisterous family birth defect that grows on a normal base of skin, but occurs in the area of ​​the pressed skin. Usually, a newborn is born, usually in the heel, around the mouth, and up. The blister is relatively deep, so when it slips, it leaves the ulcer with a scar at the skin. Progression to age does not go away.

Impetigo:

Immediately due to streptococcus, bullous growth on a skin background under red inflammation. At first in 1-2 hours after breaking open the crust is closed. Nykolsky (-), the body is rarely affected.

Erythema polymorphed:

The basic lesion is oedema. On the centre papules, there are large and small blisters, depending on the bright red colour, large or small puffiness or less water.

Look in the middle of the dark red around the bright red called the badge, creating many concentric circles.

Treatment:

The prognosis is very bad in the past 100% of the time death, since the introduction of corticosteroids is better, reducing the mortality to 40%.

The most effective drug is corticosteroids.

Corticoid at the first dose 300-500 mg/day, then reduce the dose very slowly and gradually down until there is no new lesions, it is called the maintenance dose. Maintain until all lesions are on the skin and mucous membranes.
Prolonged use must pay attention to complications of stomach ulcers, Cushing acne syndrome.
Currently, methotrexate has many good signs of progress, taking 15-20 mg once a week for 15 days, preventing BC and HC lowering. Damage to the liver and kidneys can cause teratogenesis (pregnancy is prohibited after 1 year of drinking).

Pemphigus vegetate (Pemphigus vegetate)

The basic injury is bullae.

The disease begins like the common pemphigus by lesions in the oral mucosa or on the skin but often localized to special locations: in the armpits, groin, buttocks, under the breast, around the navel.

The blister quickly ruptures, sometimes unnoticed by the patient and develops into a warty tissue. scaly and watery upper. This damage sometimes smells very unpleasant, often the lesions or link together to form a large plate, sometimes it occupies the entire back, abdomen, sometimes around the nails ... but there is still an image of water bubbles around the edge of the crisscrossed lilies or a lumpy patch 1 cm wide by 10 cm wide, a stench, presumably bullae.

After a period of progression or treatment, these plaques dry and solidify and then gradually lower, leaving a dark patch of skin.

Nykolsky signs still (+) around the plaque.

Mechanical properties are itchy, burning but erratic except for painful lesions of the oral mucosa.

Blood: Acidity is increased (eosinophils increase).

Disease progression: the disease progresses from a few months to a year, then dies from cachexia, complications in the kidneys, digestive system, respiratory system.

Histopathology: blister in Pemphigus is bullous.

Pemphigus is normally located in the spiny cell layer but here bullae appear only very early. Later the dendritic cells proliferate and it becomes erosive. So in the later stage, there are no bullae, but only deep lesions develop deeply and develop the surface of the epidermis. There are a few places made up of small abscesses. In which many eosinophils.

In the dermis, there is an infiltrate of many eosinophils that resemble conventional purulent dermatitis or Hallopeau's persistent dermatitis.

On the diagnosis of Tzanck cells found many cells round or polygonal.

On the aetiology of pathogenesis: an autoimmune disease. Pemphigus is just a body of common pemphigus because it develops on the body especially on it.

Differential diagnosis:

Syphilis II papules: papules that are higher than the face of the skin, sometimes rough but firm (pemphigus is still soft), in addition to papules, other symptoms: cranes, roseola, alopecia, serum reactions are always (+), compared coincide (+).

Tuberculosed varicose (tuberculosed varicose): it is pulpy, purple-red base, little pus when squeezed, the tuberculosis is pricked correctly, it falls down gently.

Purulent dermatitis: initial lesions are bullous, pyoderma, dermatitis, pus soft, normal whole, Tzanck cell diagnosis must be used to distinguish mainly.

Treatment:

Use corticoid, antibiotics or use aureomycin 1 g / day for a long time, tetracycline, or use aureomycin plus quinacrine.

Spot anti-infection wash saline solution, purple medicine and apply the colour solution, burn the wipes with 10% silver nitrate or curettage, burn electricity, CO2 laser.

Pemphigus scaling (Pemphigus foliage)

The basic injury is still bullous. Extremely fluffy bullae are located in the roof of the epidermis. Very shallow, so it breaks easily to reveal a shallow slit or a scab. On a water, blister grows wave after wave, creating scabs overlapping the other (Nikolsky (+)). Lesions spread to the whole body quickly spread out like red scaly red skin. The mucosa is less damaged but often causes hair and nail loss. Progressive lasting 2-3 years or longer, in some cases the disease subsides but is temporary. The patient's overall condition is kept longer. Later, the patient started to weaken, and then ceased due to the combination of diseases such as pneumonia, gradually decreased HC, BC eosinophilia increased slightly.

Common Pemphigus varieties have disorders of water, salt, and protein metabolism.

Histopathology: The blister forms like other Pemphigus but is localized much shallower near the seed layer, mainly under the horny layer, so the remainder of the mucus layer is almost normal. In the affected area, there is a pronounced leprosy phenomenon and the spiny cells float in the plasma and then it swells, it separates one from the other. At the stage of erythema, there is no blistering of cranes to see very little, but still, see the phenomenon of thorns. In the shallow dermis, there is slight oedema, with infiltrates due to inflammatory cells. The cells of acidosis, eosinophils are abundant in the epidermis, dermis, and the organizational picture is very similar to the normal exfoliative erythema.

Aetiology: Like the other Pemphigus, not clear, most classified as the root of the common Pemphigus.

Another diagnosis:

At first, it should be differentiated from ordinary Pemphigus, later on, many scabs overlap the other.

Dhuring: sometimes Scaly Pemphigus is initially like Dšhring's disease, after which there are many leaf scabs.

Treatment:

Spot: antiseptic.

Systemic: mainly corticosteroids.

Antimalarial drugs nivaquin, quinacrine in which there are many acidic cells.
In the dermis, many cells are infiltrated, which resembles conventional pyoderma or Hallopeau's persistent mastitis. 

Pemphigus (pemphigus séborheique)

Beginning in adults, first with blisters or scaly red patches similar to adipose dermatitis: localized on the face and sometimes a red lupus-like appearance. It may also start as small, uneven, un-stretched bullae that stand separately. Specially located in areas with abundant pulp such as the chest, back, breast.

Bullous: small, irregular, easily broken into streaks. Sometimes many blisters are combined with a large bubble and also slip off with a slippery array.

Lesions are usually erythema, scaling, scaly secretions and blisters, rarely burn because it is thin, fragile, so only red, scaly.

Localized in many areas with many sebaceous glands: back, shoulder, bone and nose, armpit, under the breast, on the scalp, and the limbs are less damaged. The scales are usually thick and flake off into pieces.

In some cases, the dry, thin flakes come off easily. If the lesion on the body sometimes resembles a psoriatic keratosis.

Early, later, Nikolssky (+) signs may not be seen next to dry blisters.

Scaly red plaque: often associated with blisters and dry eczema-like lesions. This burning area is also in the nasal grooves, cheeks, on the nose, on the forehead, in the area behind the ears, on the scalp.

Progression: long term, more persistent, and benign, in some cases switching to the common Pemphigus or the squamous Pemphigus.

Histopathology: leprosy phenomenon. The blister is shallow just below the stratum corneum, in the dermis with a deep layer. 

Treatment: Steroids are less effective here than another Pemphigus. 

The most active antibiotic is aureomycin.