1. SCLERODERMA DEFINATION :-
CHRONIC MULTISYSTEM DISORDER OF UNKNOWN ETIOLOGY CHARECTERISED CLINICALLY BY THICKENING OF SKIN CAUSED BY ACCUMULATION OF CONNECTIVE TISSUE AND BY STRUCTURAL & FUNCTIONAL ABNORMALITIES OF VICERAL ORGANS-GIT,LUNGS,HEART,& KIDNY

2. CLASSIFICATION OF SCLERODERMA
SYSTEMIC SCLEROSIS LIMITED CUTANEOUS DISEASE DIFFUSE CUTANEOUS DISEASE SINE SCLERODERMA OVERLAPE SYNDROME
LOCALIZED SCLERODERMA MORPHEA LINER SCLERODERMA En COUP de sabre
CHEMICALLY INDUCED SCLERODERMA
TOXIC-OIL SYNROME
VINYL CHLORIDE
BLEOMYCINE
PENTAZOCINE
EPOXI & AROMATIC HYDROCARBONES

3. FIBROBLAST ACTIVATION
PATHOGENESIS
HOST
GENETIC SUSCEPTIBILITY
INFECTION
ENVIRONMENTAL FACTORS
MICROCHIMERISM
IMMUNE
T CELL ACTIVATION
MACROPHAGE ACTIVATION
AUTOANTIBODIES
CYTOKINES
VASCULAR
ENDOTHELIAL CELL INJURY
VASCULAR OCCLUSION
TISSUE HYPOXIA
FIBROBLAST ACTIVATION
FIBROSIS

4. CLINICAL PRESENTATION OF S S
RAYNAUD’S PHENOMENON 90 TO 100 %
SKIN THICKENING TO 100 %
TELENGIECTASIA TO 85 %
ARTHRALGIAS TO 70 %
ESOPHAGEAL DYSMOTILITY ~ 80 %
PULMONARY FIBROSIS ~40 %
MYOPATHY ~50 %
RENAL CRISIS ~ 15 %
CARDIAC DISEASE ~ 10%

5. SUBSET OF SYSTEMIC SCLEROSIS
DIFFUSE
LIMITED
SKIN INVOLVEMENT
RAYNAUD’S PHENOMENON
ORGAN INVOLVEMENT
NAIL FOLD CAPILLARIES
ANTINUCLEAR ANTIBODIES
DISTAL & PROXIMAL EXTREMITIES,FACE,
TRUNK
ONSET WITH IN 1 YEAR OR AT THE TIME OF SKIN CHANGES
PULMONARY,
RENAL,GIT
DILATATION & DROPOUT
ANTITOPOISOMERASE 1 ~ 40 %
DISTEL TO ELBOW,FACE
MAY PRECEDS SKIN LESIONS
ORGAN INVOLVEMENT 10TO 15 YEARS AFTER OF DISEASE
DILATATION WITHOUT DROPOUT
ANTICENTROMERE
60 to 80 %

12. MANAGEMENT D-PENICILLAMINE 750 TO 1000 mg/DAY IMMUNOSUPPRESIVE
CYCLOPHOSPHMIDE
CORTICOSTEROIDS
CYCLOSPORINE
ANTITHROMBOCYTE AGENTS
VASODILATORS
CALCIUM CHANNEL BLOCKERS-NIFEEDIPINE
PENTOXIPHYLLINE
SKIN CARE
PHYOTHERAPY