Connective Tissue DiseasesSchool of MedicineUniversity of Sumatera Utara

Classification of connective tissue diseaseLupus erythematosus DermatomyositisSclerodermaRheumatoid arthritissjogrens syndromeMixed connective tissue diseaseEosinophilic fascitis

Basic of them all is a complex array of autoimmune responses

Lupus erythematosusDiscoid lupus erythematosusSystemic lupus erythematosus

Discoid Lupus erythematosusYoung adultWomen: men = 2:1Clinnical Findings :Dull red macule with adherent scale with follicular plugging, which heal with atrophy, scarring and pigemantary changing, telangiekatasis.Side: usually above the neck. Favorite are scalp, bridges of the nose, malar areas, lower lips and ears

Generalized dle is less common than localized DLE and is usually suerimposed on alocallized discoid case.Progression from DLE to systemic lupus erythematosus (SLE) is uncommon.

Differential diagnosisSeborrheic dermatitisRosaceaLupus vulagrisSarcoidDrug eruption

TreatmentAvoid exposure to sunlight, excessive cold, to heat and traumaUse a high SPF sun screenLocal: potent or super potent corticosteroidIntralesional triamsinolon acetonide 2,5 to 10 mg/ml

SystemicAnti malarials. Hydroxychloroquine 6,5 mg/kg/day. Chloroquine 250 mg/dayQuinacrineSystemic corticosteroid for widespread or disfuguring lesion

Systemic Lupus ErythematosusYoung to middle aged womenSkin involvement occurs in 80% of casesDiagnosis of SLE are based on four of the American Rheumatism Assosiations 11 criteria

Cutaneus manifestationButterfly facial erythemmabullous lesionDiffuse, non scarring hair lossMucous membrane lesion eq conjunctivitis, episcleritis, vaginal ulcerLeg ulcerCutaneeous angiitisCalcinosis cutis

Systemic manifestationArthralgiaRenal involvementMyocarditisCNS involvement VasculitisConvulsionEpilepsyRetinitisIdiopathic trombocytopenic purpura

EtiologyGeneticAltered immune responDrugs such as hydralazine, procainamid, sulfonamid, penicillin, anticonvulsan, minocycline and isoniazid

Laboratory findingsAnemia hemolyticThrombocytopeniaLymphopeniaLeukopeniaErythrocyte sedimentation rate is elevatedCoombs tes positifRgeumatoid factor positif

Immulogic findingsANA testLE cell testDs DNAAnti SM antibodyLupus band testANA pattern

Differential diagnosisDermtaomyositisToxic erytema multiformeAcute rheumatoid feverDrug eruptionSjogrens syndrome

TreatmentAvoid exposure to sunlight and use a high SPF sun screenAntimalarial: hydroxychlotoquin or chloroquinCorticosteroid: 1000 mg of prednisolone IV daily for 3 days , followed bt oraal prednisone 0,5 to 1 mg/kg/daillyImmunosuppressive therapy: azathiopreine, methotrexate and cyclophosphamide

SclerodermaScleroderma is sclerosis of the skin characterized by the appearance of circumscribe or diffuse, hard, smooth, ivory colored areas that are immobile and give the appearance of hidebound skin

Form of Scleroderma :Localized scleroderma: morphea and linear sclerodermaSystemic scleroderma: Progressive systemic sclerosis and CREST syndrome

MorpheaTwice common in women as in menOccurs in childhood as well as adultSkin findings: Rose aor violaceoous macules with smooth,, hard, somewhat depressed, yellowish whte or ivory-coloured lesion.The marfin surrounded by llight violaceous zone or by telangiectasesSite: trunk, extremities

Generallized morphea : widespread involvement by indurated plaques with hypo or hperppigmentation . There is no systemic involvementLinear scleroderma: linear lession extend the lenght oh the arm or leg, beginning in the first decade of life. May also occur parasagittally onthe frontal scalp and extend part way down the forehead (en coup de sabre)

Systemic sclerodermaProgressive syastemic sclerosis (PSS) is a generalized disorder of connective tissue in which there is fibrous thickening of the skin combine with fibrosis and vascular abnormalities in certain internal organs.

Women are affected three times more commonly than menPeak age onset between the third abbbnd fifth decadeRaynauds phenomenon is the first manifestation

Skin findings:Raynauds phenomenonSki becomes smooth, yellowish, and firm and shrink Site: face expressionlessHands: clawllike (sslerodactily)

Internal involvementGastrointestinal: esophageal atonyPulmonary fibrosisCardiac involvement: dyspnea, palpitationRenal failureSkeletal involvement: articular pain, swelling and inflammation

CRESTSystemic sclerosis that limited to the hands, or somtime hands and lower face. If it is associated with calcinosis, Raynauds phenomena, esophageal dysnotility, sclerodactily and telangiectasia is called CREST syndrome

Laboratory findingsANA test antibodies to nuclear RNP

Differential diagnosisMyxedemaScleredemascleromyxedema

PathogenesisPrimary vascular damageAutoimmune mechanismMicrochimerisminfection

Treatmentcalcium channel blocker nifedipin 10 mg four times daily. Oral calcitriolAzathioprine or cyclophosphamidePrednisone D-penisilamineLow dose UVA 1