SCLERODERMA

Teaching Bangsal
SCLERODERMA

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 lipsmicrostomia
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.

Literature
1) Tony Burns. Stephen Breathnach. Neil Cox. Christopher Griffiths.
Rooks Textbook of Dermatology. 8th Ed. United Kingdom. WileyBlackwell :2012
2) Klaus Wolff. Richard Allen Johnson. Fitzpatricks Color Atlas &
Synopsis of Clinical Dermatology. 6th Ed. United Kingdom. Wiley
Blackwell. 2007