SMIJAL

Auto immune disorder characterised by tissue destruction due to the deposition of auto antibody and immune complexes with in it.

Production of antinuclear antibody – hallmark

Specific cause undefined. Researchers suggest that genetics,

hormones and environment contribute to the immune disregulation in lupus.

Age of onset: 30 years in females &40 years in males.

Skin lesions : fixed erythematous lesions that have a butterfly configuration over the cheeks & across the bridge of nose.

Also affect neck,shoulders,upper arm & fingers.

Produce itching & burning sensation & areas of haemorrhage which is intensified by sunlight.

Female to male prevalence ratio : 2:1 before puberty & 4:1 after puberty.

Extensive loss of hair from scalp. Kidney : fibrinoid thickening of glomerular capillaries. Heart : fibrinoid degeneration of epicardium and

myocardium & atypical endocarditis. SLE included under category of collagen desease.

Shows white hyperkeratotic plaque like areas & resemble lichen planus.

Hyperemia,edema and extention of lesion is more prominent in SLE than DLE.

Bleeding, petechiae, & superfitial ulcerations are present , which are surrounded by a red halo.

Vermilion border of lip is affected-lupus cheilitis. Xerostomia , altered taste sensation , chronic

periodontal desease etc are also reported. In some cases lesions under go malignant

transformation.

Collagen disturbance & degenerative features are prominent.

Atrophy with hyperkeratinisation of the oral epithelium.

Liquifactive degeneration of the basal cell layer.

Lymphatic infiltration & fibrinoid degeneration of the collagen fibers

Edema of sub epithelial CT with vascular dilatations.

Specific test was established with the discovery of LE cell inclusion by Hargraves and his associates.

Test : add blood serum from a person under suspicion to the buffy coat of normal blood.

If the patient is suffering from SLE , typical LE cells will develop.

Systemic steroid therapy.

Chronic , scarring ,atrophy producing , photosensitive dermatosis.

Not associated with autoantibody production.

Occurs in genetically predisposed individuals.

Heat shock protein is induced in the keratinocyte due to UV light exposure or stress & this protein act as target for T cell mediated epidermal cell toxicity.

Occurs in third & fourth decades of life. More common in women. Common sites : oral mucous membrane,

chest, back & extremities. Skin lesions of DLE also present a

butterfly distribution over the molar region & across the nose.

On forceful removal of covering scale , neumerous ‘carpet track’ extensions of the pialo sebacious channels appear.

Skin lesions enlarge at the periphery & epidermoid or basal cell carcinoma may develop from these lesions.

Involvement of scalp with lose hair is also common.

Reported in 20 -50 % cases of DLE. Begins as erythematous areas ,with out

induration & typically with white spots. superfitial, painful ulceration occur with

bleeding. No scale formation on skin. Seen in buccal mucosa,tongue,palate &

vermilion border of the lip. Tongue : atrophy of papillae & fissuring

are seen.

Hyper ortho or parakeratinisation of the surface epithelium.

Atrophied epithelium. Few lesions exhibit keratin plugging &

achanthosis. Basophilic degeneration of the collagen. Inflammatory cell infiltration till CT. Vasculitis absent.

scleroderma/dermatosclerosis/hide bound disease.

Systemic CT disease charecterised by vasomotor disturbances, fibrosis, subsequent atrophy of the skin, SC tissue, muscles & internal organs with associated immunologic disorder.

Blood circulation insufficiency in tissue due to abnormalities in arterioles & blood capillaries cause replacement of the normal CT by the dense collagen bundles & result in fibrosis or sclerosis of the tissue.

Genetic factors are also involved.(HLA B8,HLA DR3,HLA DR5,HLA DR52M,HLA DQB2).

Apoptosis and generation of free radicals are also involved.

Age: common in 30 to 50 years of age. Sex: female to male ratio is 3-6:1. Begins on the face, hands, or trunk. Typical indurated oedema of the skin,

neuralgia, paresthesia, arthritis, joint pain etc are present.

Erythema present.

Skin: yellow , gray , or ivory white waxy appearance.

Brown pigmentation of the skin is present as a late manifestation.

Calcinosis cutis is seen in the affected area.

Eyes become narrow. Skin becomes hardened & atrophied. So

that wrinkles do not form. It gives a mask - like appearance of face.

‘’Monalisa face.

restriction of muscle movements results in fibrosis.

Finally internal organs like GI tract, heart, lungs & kidney become affected by fibrosis.

Crest syndrome is variant of systemic sclerosis. It includes the following components.

1.calcinosis. 2. Raynaud’s phenomenon. 3.esophageal dysfunction. 4.scleroductyly. 5.tielangiectasia.

1.localised/circumscribed scleroderma/morphea. One or more well defined ,slightly elevated or depressed cutaneous patches . Lesions occur on the sides of the chest & and the thighs.

2.generalised/diffuse form/linear scleroderma . Occur as linear bands or ribbons on the face.

Monalisa face.* Multiple telangiectatic spots on the facial skin

& loss of facial esthetics and expression.* Pinched appearance of the face-mouse facies.* Trismus & restricted movements of the TMJ. * Pain, clicking sound & crepitations in TMJ.* Pursed lips & fish mouth.* Xerostomia due to atrophy of SG.* Trigeminal neuralgia.

Chicken tongue. Loosened teeth. Weakness of hands, decreasad TMJ

mobility, decreased salivary secretion, decreased mouth opening etc lead to poor oral hygiene.

Widening & thickening of PDL space. Bone resorption at condyle or ramus of

mandible. Osteomyelitis.

Atrophied oral epithelium with flattening of rete ridges.

Thickening & hyalinisation of the collagen fibres in the CT with atrophy of minor SGs.

BVs become scanty & its lumen become narrow due to perivascular fibrosis.

PDL thickness increased due to increased synthesis of collagen & oxytalin fibres.

Sweat glands, sebacious glands, & hair follicles absent.

No specific treatment.Systemic steroid therapy produces partial

remission.