SCLERODERMA.

This is chronic diseases manifestation scleroses connective tissue and other organs.

Etiology and pathogenesis is not discovery. There is presupposing by infection so as usually manifestation after acute and chronic diseases. Fibrous transformation connective tissue associate with superfluous syntheses of collagens by fibroblasts.

For pathogenesis scleroderma has role neuro-endocrinology and vessels transgression. As a role is separation focal and diffusion scleroderma. By the focal scleroderma usually damage only skin and by diffusion form damage skin and other organs.

Focal sclerodema. There are several clinical forms, general is patch scleroderma. By this form characterized appears a little number rounding fireplaces. They of this developing go to 3 stages: spot, plague end atrophy. Beginner diseases unnoticed with appear 1 and several violet-rouse roundish point several size. Gradual center of point is turn pale and beginning condense, fireplaces soon transformer by more compact plagues yellow-with color with smooth brilliant surface. On peripheral border plagues are reserving violet garland this zone peripheral grown.

Hairs on the plagues are falling out, skin picture be smoothed, sweating and sebum secretion stoppage, mucous membrane dry. Skin on the damage fireplaces not collection fold. In this form fireplaces may be stay uncertain time (mounts, years) and then graduated transformed with scary atrophy. They center soften, fall in and all plague transformed by fireplaces atrophy. This damage often not calls subjective sensation.

More rare forms focal scleroderma is linear, meeting usually by children. Fireplaces there is sight linear and settle usually longitudinal on the extremely and on mains linear forehead called blow of saber.

Scleroderma diffusion. Beginning usually is with weakness, sub fibril temperature, arthralgia, parastesia, tingling sensation, light itch. Then process characterized is universal damage skin and going by these developing 3 stages:

- tumor or edema,

- indurations,

- atrophy

On the back ground prodromal phenomenon developing compact tumor of the skin. Skin transform is yellow-gray color, skin on the damage fireplaces not collection fold and by press of finger dimple not formation. Then changing of stage indurations compact of the skin increase and it is stand motionless. Surface of the skin is look even and brilliant, face is mask, not mimic, mouth narrowing (microstomia) and movement difficulty. Ill in this stage compare with mummy. This condition may be protracted indefinite time. Gradual developing is atrophy of damaging skin.

Join: deformation to be related to compacted periarticulate tissue and composite calcium periarticulate – symptom Tibergy-Weisenbaxa.

Weight: weight loss to be related to muscles atrophy, miastenicum syndrome.

Digestive tract: dysphagia, esophagitis, gastritis, colitis.

Lung: bilateral pneumoscleroses = lungs hypertension.

Heart: firstly cardioscleroses, cardiomegalia, endocarditic=heart defect.

Vessels: symptom Reino.

Kidney: glomerulonefritis.

CNS: transgression sensation.

CREST syndrome:

C calcinoses

R Reino

E esophagitis

S sclerodactilia – osteolises finger, shorten and deformation.

T teleangiectasia.

Clinically diagnoses.

- disproteinemia - > a2 and Y globulins (20-60)

- C-reactive protein

- fibrinogen,

- >ESR – erythrocyte sedimentation rate (20-60)

- ANF by highly titer – this is autoantibody against nuclear. 3-5:1000 leucoc.

- LE cells 2-7 %

- RF 50%

- oxiprolin in the urine – showing for transgression change collagen.

- in the urine – protein, casts, erythrocytes.

- biopsy of skin – fibrous complication.

Date: 2014-12-21; view: 996