Dermatological scleroderma lecture

2021-03-24 12:00 AM

Four types of different types in their acidic composition. One-third of a person's protein is collagen, and bones and skin contain the most collagen

Outline

It is a disease of collagen (collagen).

Collagen is an insoluble fiber protein, a component of bones, cartilage, teeth, tendons, blood vessel walls, and skin, accounting for 35%.

There are 4 types: Type 1: in bones, skin, teeth.

Type 2: cartilage.

Type 3: in the aorta.

Type 4: in the basal membrane.

Four types of different types in their acidic composition. One-third of the human body protein is collagen, bones and skin contain the most collagen. If there is a disorder of the rate of collagen types, then a disease of the connective tissue is born. In the synthesis of collagen must have a certain ratio ie balance. When there is a disorder of the rate of synthesis leading to disease of colloid, keloid, and hypertrophic scarring and causing increased synovial membrane in rheumatoid arthritis.

Collagen is synthesized by fibroblasts (Fibroblast) or the equivalent occurs in 2 tenses. One is intracellular, one is extracellular. in the intracellularly produces pre-collagen, in the extracellular collagen, collagen biosynthesis is regulated by many factors:

Hormone: somatropin, several other factors (thyrocalcitonin).

Inhibitory hormones such as adrenal (cortison). Thymus.

Scleroderma

1753, the author Carlo Carzio defines scleroderma as a progressive lesion of the skin that corresponds to the overproduction of the colloidal fibres of the dermis. That phenomenon is called Carlo Carzio.

This disease in a severe form called systemic scleroderma when the organs of organs are most affected by the digestive system, joints, lungs, kidneys and heart. The lighter bodies only damage in the scale, muscles, bones just below the damaged skin, affecting only the function and aesthetics, in both forms there are abnormalities of fibroblasts. It is generalized that there are 2 types of scleroderma. The systemic type is composed of Acrosclérose diffuse or CRST (Calcinosis - Raynaud's Phenomenon- Sclero dactyly - Telangiectasie).

Raynaud's (Raynaud's) dilated tiny capillaries, also known as diffuse persistent sclerosis.

Local type: the plaque, the band (the knife cut in the forehead), the droplet, the coronary.

Pathogenesis

The cause is unknown, there is no damage to the capillaries, why there is an immune disorder: why there is an imbalance to synthesize collagen breakdown, or there is a dysfunction of colloid formation. Currently, people go into the following issues:

Pathology of small blood vessels: Based on the meta-structural and immunological reviews, there is a lack of quality and quantity. Protea inhibitors (protein breakdown enzymes) thereby produce the effect of the activation of cytotoxic serum factor on the endothelial cells of capillaries.

Platelets with their chemical mediators and growth factors may play a crucial role in fibrosis formation.

Comment: It is worth noting that the precursor to collagen increased mainly around the capillaries.

Immune disorders: Immune disorders detected in systemic scleroderma of unknown pathogenicity e.g. an abnormal increase in the function of LT helps to lead to an excessive increase in activated lymphocytes. collagen synthesis of fibroblasts.

Monocyte Interleukin I stimulate collagen production and is inhibited by M Interféro.

In addition, anti-nuclear antibodies to DNA, RNA, anti-muscle antibodies, Ig deposition, complement deposition and fibrinogen appear in the junction of the dermis and in the capillary blood vessel wall.

The synthesis and degradation of collagéne: as a result of the two factors mentioned above play a role in the accumulation of fibroblast subpopulations, with strong collagené synthesis. The growth of messenger RNA in collagen synthesis step 1 in scleroderma fibroblast culture medium.

In the interstitials of the terminal amines of the terminal aminotides of the pre-collagenes extracted by the enzymes, which in turn control fibroblasts, the abnormal overhead splitting leads to the formation of poor end aminopeptide fibers. chain with ineffective reverse control.

There are also some other factors such as:

There may be peripheral dysfunction.

Some plaque scleroderma can be a result of Lyme disease after a tick sting, due to spirochetes infection, so in the treatment people use penicilline.

Systemic scleroderma

Epidemiology

Meet in female / male = 3/1, starting age 20-50 years old.

Meet in many countries and races.

Diseases include skin lesions that aid in diagnosis and skin lesions that also cause severe functional disability. And internal damage leads to life-threatening.

Prognosis

Persistent disability makes patients

clinical

Skin damage:

Raynaud's syndrome (differentiating Raynaud's disease '):

95% have Raynaud's syndrome '.

Usually, the first sign precedes other signs from a few weeks to a few years.
As a poor prognosis, the distance between Raynaud's syndrome 'and the appearance of short regional sclerosis: eg hands on both sides, sometimes feet. This syndrome is easy to diagnose clinically.

Cold onset, beginning with severe paroxysm. One or more fingers are white, cold, almost insensitive, with pronounced vasoconstriction on the tip of the finger or the back of the hand after a few minutes the fingers become cyan and painful for a few minutes. This "syncope" episode is essential for diagnosis and differentiation from normal cyanosis.
The capillary test of the nail is necessary for diagnosis. Visible spleen capillary coronary enlargement and degeneration of blood vessels. These signs do not depend on the extent of skin damage. This test helps to save the need for angiography and biopsy. Raynaud's syndrome can have episodes, sometimes flares, sometimes quietly depending on the person.

Skin sclerosis:

Beginning with fingertips spreads in different patterns and has certain prognostic value.

Spread out on the face of the limbs and body.

In slow progression sclerosis of the finger, the foot is well developed. When the hardening begins to get heavy on the face, body, and calves.

In the form of rapid and severe progression, sclerosis develops very strongly, the sclerosis of the shoulder area such as the skin armour causes the loss of the breasts, the abdominal skin becomes as tight as the empty skin, the phenomenon of mummification of the lower extremities, the face blurring nose, narrowing the mouth around the mouth there are signs of easy-to-diagnose bicycle spokes. There are disorders of dark, pale skin color (mistaken for vitiligo).

Varicose capillaries, present in all forms of diffuse scleroderma or in the face and segments.

Deposits of calcium in the dermis are found in all scleroderma, even in localized forms, found in fingers and feet.

Thibierge and Weissenbach (CRST) syndrome:

The manifestations of the buttons, the palpable clump, are the result of local anaemia. These deposits are the cause of persistent pain ulcers, ulcers that discharge a chalk-like liquid.

Injury to other organs:

Kidney damage: Is the cause of death over half of the scleroderma patients die.

The treatment of malignant hypertension in 2/3 of patients with kidney damage often has acute kidney damage in cases of scleroderma with rapid skin damage after Raynaud's syndrome '.

In 15-30% of scleroderma patients with mild kidney damage, moderate Pruria, high blood pressure, sometimes the concentration of Nitr / blood discreetly needs to be checked by blood urea. In the severe form, the paroxysmal progress immediately to acute and is influenced by some factor such as systemic corticosteroid use, due to pregnancy.

Heart damage:
Early silent cardiomyopathy is secondary to silent cardiomyopathy secondary to cardiomyocardial microcirculation abnormalities.

Pericarditis is usually moderate on its own rather than chronic if it leads to chronic often with a poor prognosis.

Heart failure is often secondary to kidney damage, high blood pressure, and rarely due to pulmonary fibrosis.
Sometimes heart failure is caused by fibromyalgia (rarely).

The principle of scleroderma does not cause endocardial damage.

Digestive:

Esophagus: 75% of cases are latent in one third of the cases that usually appear early and are an important factor for diagnosis.

The small intestine is more common than the stomach and duodenum, which causes severe indigestion.

Hypersensitivity of scleroderma is medically treatable.

Other injuries:

Joint pain that follows the first signs of scleroderma is seen in 50% of cases, but this joint pain leaves no sequelae.

Osteoporosis is sometimes associated with fibrosis that usually occurs on the third knuckle of the fingers.

Perioral bone resorption in 30% of cases.

Muscle damage is clinically similar to dermatitis.

Liver damage: 50% cases of primary biliary cirrhosis.

Peripheral nerve damage is rare.

Prognosis and progression

The prognosis of exacerbation depends on the type according to Barnett's classification, but usually, the disease progresses over decades. Causes 1/2 of kidney damage (type 2 and 3) is less common in type 1. The cause of death is the heart, lungs, stomach, intestines.

The clinical manifestations and border syndrome:

Pediatric systemic scleroderma is less likely to damage the skin than in adults, but Raynaud's syndrome is usually absent. Alternating infections worsen symptoms.

In lethal progression, 1/2 fatal heart damage.

Kidney damage is less in children than in adults.

Slow growing children gain weight.

Systemic scleroderma and pregnancy: Does not appear to affect fertility, but spontaneous miscarriages, premature death, and death have a high incidence of about one-third of the reported cases, possibly due to fetal toxicity and premature delivery in systemic scleroderma. Two-thirds of cases have not been found to affect fertility progression. Severe illness during pregnancy, the author again said that the disease is significantly better during pregnancy if damage to many organs adversely affects the fetus.

Diagnose

According to the standards of the ARA (American Society of Rheumatology).

Main criterion: Transient skin infiltrates.
Substandard:

Toe sclerosis or ulcers or scarring of the toe.

Pulmonary fibrosis in 2 backgrounds.

Decisive diagnosis: Only 1 main criterion is needed.

If there is no major criterion, two thirds of the sub-criteria are needed (but there must be sclerosis of the fingers and feet).

Other diagnosis:

Raynaud's syndrome:

If this syndrome on both hands and feet is difficult to diagnose, it is necessary to look carefully: dilated capillaries, discreet sclerosis of the head and shoulder areas.

If Raynaud's syndrome 'has both sides, then scleroderma is more likely.

Acrosclérose (long-distance fiber):

Congenital, due to metabolic disorder, toxic, autoimmune, congenital patchy dermatitis.

Porphyrie, Mucinose, Bléomycine, D. Penicillin poisoning, Buschke's fibrosis, and neonatal edema.

Treatment

Treatment results are very conservative.

Treatment trial:

Use of Raynaud's anti-vasomotor and chronic sclerosis.
Nifédipine (Adalate) 20 mg / time x 4 times / day (not for pregnant women).

Use anti-inflammatory drugs: Prednisolon dose 1 mg/kg/day and reduce the dose when clinical progress is about 2 months.

If you can use Cyclosporin A, the better.

M Interferol can be used.

Taking D. Pénicillamine (Trolovol) dose gradually increased starting 50 mg to 100 mg up to 300 mg/day in January for up to 600 mg/day in month 2.
(Note no more than 900 mg/day).

Coagulation factor 13: (Fibrogammine) slow intravenous drip 2 vials / day for 21 days. Then 10 days / time x 1 vial.

Vit D 3 doses gradually increased.

Physiotherapy: take a warm bath.

Local scleroderma

Can

Scleroderma plaques:

There is 1 patch or any patches appear on the skin, first pink after slightly purple, the temperature at there is normal, the mark gradually enlarges and has a white iridescent, infiltrative color. Oval shape, size depends on each case, but it is different in size.

The damaged skin cannot be squeezed in, not wrinkled.

Lesions have a dull pink border (bandages) around the lesion 2 - 6 mm wide, and yellow color disappears when the lesion is stable or receding.

On the surface of the lesion there is no hair, no sweat, reduced sebum secretion, and sensation also decreases. When the scleroderma plaque leaves a dark color of different degrees, persistent dark color, there is a phenomenon of skin atrophy.

Pay attention to scalp damage that leads to hair loss (scarring without hair).

Scleroderma:

Common in the limbs. the face and body are very rare 

Common in young people.

There is a loss of muscle mass under skin damage.

Occasionally fibrous lesions are present: fibrous convex (mélorhéostose).

Leri syndrome has thickened bones in length, only at one end and has periosteal proliferation.

Start suddenly as if it was caused by a trauma, there may be one or more limbs at the same time and the one before the next cannot be resolved, the disease causes atrophic fibrosis in the limbs.

Face, scalp:

Coup de saber is most typical if it appears on the forehead with a split, the central edge 2 to 3 cm wide on the side of the scalp where the hair is slightly encroached on and causes hair loss. The bottom spread down the arch of the eyes, making the arch of the eyes spreading down the cheeks to the lips, the gums and the jaw can be atrophy.

Sometimes starting in the temples going down in front of the ear damages the lower jaw or starts in the chin.

It is necessary to distinguish facial scleroderma from facial atrophy (Romberg).

There is also a round drop scleroderma slightly concave like a film covering. Expressing white nacre-white spots. Sometimes the chromosome from the beginning.

Ring-shaped scleroderma: in the foreskin, fingers, toes.

Some other forms:

Vitiligo-type depigmentation clusters: greying hair, requiring monitoring may be discoloured scleroderma.

A certain patch of skin, red for a long time, scabs then form scleroderma.

Scleroderma previously has a blister or bullous lesions with blood.

Scleroderma with hypercalcemia in the dermis leads to ulcerative lesions.

Systemic extensive scleroderma does not have Raynaud's syndrome.

Unexplained scleroderma of Pasini and Pierini features atrophy of the skin seen in middle-aged and young adults, common location in the body. Injuries include 10-12 lesions. Each lesion is several centimetres in diameter. Not infected with hard skin but atrophy of the skin at the same time with skin chromosome. The disease is stable for several months, the prognosis is good.

Shulman-EO (Shulman) bursitis is seen as local scleroderma. There is no Raynaud's syndrome, which usually appears after an exertion. Scleroderma mainly in the dermis and muscle sac. No internal damage.

Treatment

Dimethylsulfoxide (DMSO) 50- 90% solution applied topically 1 time/day, correctly applied to the lesion. If the patient has extensive lesions then divided into batches.

Systemic corticosteroids at 0.5 mg/kg/day (Prednisolon) for a prolonged period of 3 months should be used early.

In the first 20 days should combine injections of 1 million Penicillin/day.