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CLASSIFICATION OF SCLERODERMA

DISORDERS

The simplest division of the ss related

disorders is into localised and

systematicDisorders other than ss cause skin

thickening are DM ,hypothyrodism,

nephrogenic systemic fibrosis,

eosinophilic fascitis ,amyloidosis,exposure to certain drugs, toxins, &

enviromental exposure

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LOCALISED

SCLERODERMADivited to

Linear scleroderma

Localised morphea

Generalised morphea

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Linear scleroderma

Most commonly occuring inchildhood

On one side of bodyFace or scalp lesion is

accompanied by marked

abnormalities of underlyingmesenchymally derived tissue,including skull

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MORPHEA

Divided into localised & generalised

Patches of sclerotic skin whitch develop on thetrunk and limbs of previously normal texture

Localised morphea refers to the presence of one or

more circomescribed isolated plaques of scleroticskin

Involving the trunk and limbs

It can lead to widespread skin sclerosis

Generalised morphea can be distinguishd fromdiffuse ss that it spare hands and face and notassociated with major vascular symptoms orvisceral disease

It is not benign and may require use ofimmunosupressive and antifibrotic agents

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Guttate morphea frequent involveshoulders and chest

Multible small [2-10 mm in diameter],

hypopigmented and pigmented papuleswith minimal sclerosis

Morphea profunda is subcutaneous variantwith significant inflammatory componentand poorly defined skin borders

Bullous disease is rare Ab to DNA topoisomeraseII is very

prevelant in localised form of ss includinggeneralised morphea

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Systemic sclerosis

Skin and internal organs

involvement

Skin sclerosis score grade skinsclerosis in 17 sites

0[normal] 1[equivocal sclerosis]

2[definite sclerosis] 3[hidebound]

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SS SUBSETS

Diffuse cutaneous SSc[dcSSc]

Limited cutaneous SSc[leSSc]

SS sine scleroderma in which pthave only internal organsinvolvement

Enviromental induced scleroderma

Overlap syndrome in which featuresof SSc coexist with elements ofother rheumatic disorders

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Limited cutaneous SSc

Skin sclerosis restricted to thehands and to a lesser extent theface and neck

Have prominent vascularmanifestations

Suffer from CREST syndrome

Presence of severe vascularabnormalities on nailfold capillarymicroscopy

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DIFFUSE CUTANEOUS

SScHave extensive skin sclerosis

Have greater risk for the development

of significant renal lung and cardiacdisease

Extension of skin sclerosis proximal to

wrists

Over proximal limbs and trunk but

commonly sparing the upper back

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SSc SINE

SCLERODERMA Rare form of the illness

Vascular features and visceral

fibrosis of systemic disease

Without skin sclerosis

Prognosis like LcSSc

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ENVIROMENTAL INDUCED

SCLERODERMA

History to exposure to an

agents suspected to induce SSc

Vinyle chloride, epoxy resin,pesticides and a number of

organic solvents used in paints

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OVERLAP SYNDROMES

Features of SSc in combination

with manifestaion of other

rheumatic diseases such as SLE,dermatomyositis or rheumatoid

arthritis

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Classification of early

disease Use of capillary nailfold microscopy

and serologic testing forautoantibodies

Early diagnosis of SSc in absence ofsclerodermal skin change by RP plusabnormal wide field capillarymicroscopy or RP plusautoantibodies[ anticentromere-antitopoisomerase-antifibrillarin-anti-PM-Scl-or anti-RNA-polymeraseI or II

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INTERMEDIATE

CUTANEOUS SSc We recognised this disorder in

some European countries

Chractarised by an intermediatepattern of skin changes

extented onto but not beyond

the forearms

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CERTAIN NOTES

Pt with Lc SSc have very high mortality rate

High mortality rate with pt with isolated pulmonaryhypertension

Pt with LcSSc who develop significant lung fibrosis

or acute renal failure have a poor prognosis asthose with DcSSc

HLA-DR52a is associated with an increased risk oflung fibrosis

Pt with antibodies to topoisomerase-I[Scl-70] areassociated with an increased risk of pulmonary

fibrosis in both the DcSSc and Lc-SSc

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Anticentromere antibodies[ACA] arealmost always indicative of LcSScand are seen in the classical CREST

AUTOIMMUNE Raynaud phenomenonare characterized by abnormalnailfold capillaroscopic findings andthe presence of positive antinuclearAb anticentromere Abantitopoisomerase-I or antiRNAPOLYMERASEiOR ii Ab may behaving prescleroderma