SCLERODERMASCLERODERMATeaching Bangsal

CASE

•Name : Suriani•Sex : Woman•Date of Birth : 01-07-1975 / 38 years•Job : Housewife•Address : Desa Mario Bone•Status : Married•Religion : Islam•Diagnosis : Scleroderma DD/ SLE

ANAMNESIS• Autoanamnesis• Chief Complaints : Patient came in with the

complaint of pain sensation over the dark patches on forearms, thigh & legs. Firstly experienced with existence of dark patches with pain on the thigh region with 10 cm in diameter which progressively become bigger in time. Later on the dark patches harden. Approximately 2 years ago the dark patches appeared with later harden on both arms.

• Additional Complaints : Hair loss, Joints pain, Pain on scalp.

• Previous Disease History : none• Pain Scale : (+) Location : arms & legs.

Intensity :(0-10)= 6 Type : Chronic

Physical Examination

•Blood Pressure : 110/70•Heart rate : 70x/m•Respiratory Rate : 21x/m•Temperature : 37.0 ‘C

Skin Clinical Feature

•Location : Regio ext. Superior et inferior•Distribution : Bilateral•Effloresce :Macule, hyperpigmented,

nodule

LABORATORY•Random blood glucose 81mg/dl 140•Ureum 28mg/dl 10-50•Creatinin 1.0mg/dl <1.1•SGOT 29 <38•SGPT 18 <41•Total Cholesterol 179 200•LDL 136 <130•Uric Acid 8.8 2.4-5.7

DEFINITION

•A disorder of unknown cause in which there is localized sclerosis of the skin.1

•The term ‘scleroderma’ should strictly be confined to sclerosis of the skin, either localized or generalized, occurring in patients as the only or prominent feature.1

CLASSIFICATION• Systemic scleroderma can be divided into two

subsets: limited systemic scleroderma (lSSc) and diffuse systemic scleroderma (dSSc).

• lSSc patients comprise 60%; patients are usually female, older than those with dSSc; and have a long history of Raynaud phenomenon with skin involvement limited to hands, feet, face, and forearms.(acrosclerosis)

• dSSc patients have a relatively rapid onset and diffuse involvement, not only of hands and feet but also of the trunk and face, synovitis, tendosynovitis, and early onset of internal involvement.

ETIOLOGY AND PATHOGENESIS•Unknown. •Primary event might be endothelial cell

injury in blood vessels, the cause of which is unknown.

•Early in course, target organ edema occurs, followed by fibrosis, cutaneous capillaries are reduced in number remainder dilate and proliferate, becoming visible telangiectasia.

•Fibrosis due to overproduction of collagen by fibroblasts

CLINICAL MANIFESTATION

•Hands/feets:•Early : Raynaud phenomenon with digital

pain, coldness. Pain/stiffness of fingers, knees.

•Painful ulcerations at fingertips “rat bite necrosis”•Late : Sclerodactyly with tapering of

fingers (madonna fingers)

RAYNAUD PHENOMENON•Raynaud phenomenon (RP) is digital

ischemia that occurs on exposure to cold and/or as a result of emotional stress.

“Rat Bite Necrosis”

•Typical “rat bite” necroses and ulcerations of fingertips.

“Madonna Fingers”•Hands and fingers are edematous (non pitting); skin is without skin folds and bound down. Distal fingers are tapered.

•Face•Early : periorbital edema. •Late : edema and fibrosis result in loss of

normal facial lines, masklike (patients look younger than they are), thinning of lips, microstomia, radial perioral furrowing, beak-likesharp nose. Telangiectasia and diffuse hyperpigmentation.

Thinning of lips—microstomia•Radial

perioral furrowing

•Beaklike sharp nose

Scleroderma (dSSc)•Masklike facies

with stretched, shiny skin and loss of normal facial lines.

•Thinning of the lips and perioral sclerosis result in a small mouth.

LABORATORY EXAMINATIONS

•Dermatopathology - Early : mild cellular infiltrate around dermal blood vessels, eccrine coils, and at the dermal subcutaneous interphase.

•Autoantibodies – Autoantibodies react with centromere proteins or DNA topoisomerase

MANAGEMENT• Systemic glucocorticoids may be of benefit

for limited periods early in the disease• Vasodilating drugs (calcium-channel

blockers, angiotensin receptor antagonists, topical nitrates, and prostanoids)medical therapy for Raynaud phenomenon.

• Cyclophosphamide has shown some promising results in the treatment of cutaneous disease, Improving skin scores and maximal oral opening.

•Phototherapy and photochemotherapy especially with UVA1 have also shown some efficacy, at least for localized scleroderma.

•Methotrexate may have some efficacy for the skin thickening of diffuse scleroderma.

Literature1) Tony Burns. Stephen Breathnach. Neil Cox. Christopher

Griffiths. Rook’s Textbook of Dermatology. 8th Ed. United Kingdom. Wiley-Blackwell :2012

2) Klaus Wolff. Richard Allen Johnson. Fitzpatrick’s Color Atlas & Synopsis of Clinical Dermatology. 6th Ed. United Kingdom. Wiley Blackwell. 2007