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Connective Tissue

Diseases
School of Medicine
University of Sumatera Utara

Classification of connective tissue


disease

Lupus erythematosus
Dermatomyositis
Scleroderma
Rheumatoid arthritis
sjogrens syndrome
Mixed connective tissue disease
Eosinophilic fascitis

Basic of them all is a complex array of


autoimmune responses

Lupus erythematosus

Discoid lupus erythematosus


Systemic lupus erythematosus

Discoid Lupus erythematosus

Young adult
Women: men = 2:1
Clinnical 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 diagnosis
Seborrheic dermatitis
Rosacea
Lupus vulagris
Sarcoid
Drug eruption

Treatment
Avoid exposure to sunlight, excessive
cold, to heat and trauma
Use a high SPF sun screen
Local:
potent or super potent corticosteroid
Intralesional triamsinolon acetonide 2,5 to
10 mg/ml

Systemic
Anti malarials. Hydroxychloroquine 6,5
mg/kg/day. Chloroquine 250 mg/day
Quinacrine
Systemic corticosteroid for widespread
or disfuguring lesion

Systemic Lupus Erythematosus

Young to middle aged women


Skin involvement occurs in 80% of
cases
Diagnosis of SLE are based on four of
the American Rheumatism
Assosiations 11 criteria

Cutaneus manifestation
Butterfly facial erythemmabullous lesion
Diffuse, non scarring hair loss
Mucous membrane lesion eq
conjunctivitis, episcleritis, vaginal ulcer
Leg ulcer
Cutaneeous angiitis
Calcinosis cutis

Systemic manifestation

Arthralgia
Renal involvement
Myocarditis
CNS involvement
Vasculitis
Convulsion
Epilepsy
Retinitis
Idiopathic trombocytopenic purpura

Etiology
Genetic
Altered immune respon
Drugs such as hydralazine, procainamid,
sulfonamid, penicillin, anticonvulsan,
minocycline and isoniazid

Laboratory findings
Anemia hemolytic
Thrombocytopenia
Lymphopenia
Leukopenia
Erythrocyte sedimentation rate is
elevated
Coombs tes positif
Rgeumatoid factor positif

Immulogic findings
ANA test
LE cell test
Ds DNA
Anti SM antibody
Lupus band test
ANA pattern

Differential diagnosis
Dermtaomyositis
Toxic erytema multiforme
Acute rheumatoid fever
Drug eruption
Sjogrens syndrome

Treatment
Avoid exposure to sunlight and use a high SPF
sun screen
Antimalarial: hydroxychlotoquin or chloroquin
Corticosteroid: 1000 mg of prednisolone IV
daily for 3 days , followed bt oraal prednisone
0,5 to 1 mg/kg/dailly
Immunosuppressive therapy: azathiopreine,
methotrexate and cyclophosphamide

Scleroderma

Scleroderma 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 scleroderma
Systemic scleroderma: Progressive
systemic sclerosis and CREST syndrome

Morphea

Twice common in women as in men


Occurs in childhood as well as adult
Skin 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 telangiectases
Site: trunk, extremities

Generallized morphea : widespread


involvement by indurated plaques with
hypo or hperppigmentation . There is no
systemic involvement
Linear 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 scleroderma

Progressive 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 men
Peak age onset between the third
abbbnd fifth decade
Raynauds phenomenon is the first
manifestation

Skin findings:
Raynauds phenomenon
Ski becomes smooth, yellowish, and
firm and shrink
Site:
face expressionless
Hands: clawllike (sslerodactily)

Internal involvement
Gastrointestinal: esophageal atony
Pulmonary fibrosis
Cardiac involvement: dyspnea,
palpitation
Renal failure
Skeletal involvement: articular pain,
swelling and inflammation

CREST
Systemic 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 findings
ANA test
antibodies to nuclear RNP

Differential diagnosis
Myxedema
Scleredema
scleromyxedema

Pathogenesis
Primary vascular damage
Autoimmune mechanism
Microchimerism
infection

Treatment
calcium channel blocker nifedipin 10 mg
four times daily.
Oral calcitriol
Azathioprine or cyclophosphamide
Prednisone
D-penisilamine
Low dose UVA 1