Bagian Ilmu Kesehatan Kulit dan Kelamin REFERAT

Fakultas kedokteran Agustus 2019

Universitas Pattimura

SCLERODERMA

created:
Priskilla L Belseran
NIM. 201884039

Consulents:
dr. Hanny Tanasal Sp.KK
 Introduction
• Scleroderma is a rare, multisystem,
autoimmunological processes,
• Women > man
• Involves multiple cell types (endothelial cell,
epithelial cells, fibroblast and lymphocytic
cells)
• Laboratorium finding
• Prognostic clinical patern
• Multisystem disease  autoimmunological
processes
• Vascular endothelial cell injury, extensive
activation of fibroblas.
• variability Characterized in the extent  skin
& organ involvement
 Epidemiology
• Woman > Man (Ratio 3:1  14:1)
• 30 – 50 years
• Incidence rates increased from 0.6 to
16/million/years
• Los  0.3-3 case/years
 Etiology
• Idiopatik
• At first  dysfungsi endotel
 Classification

Localized
Scleroderma
Systemic

Figure 1.2 Classification of Scleroderma

 Linear morphea
• Linear lession extend the length of
the arm or leg and follow line
blaschko
• Begins during the first decade of
life Figure 1.5 En coup de sabre
• Lessions occure parasagittally on
the frontal scalp and extend partly
down the forehead (en coup de
sabre )
• Involved lower extremity
 Systemic Scleroderma

• Diffuse sclerosis
– sclerosis (proximal and distal
extremity, trunchus)
– Progressive, multyorgan demage
• Limited sclerosis Figure 1.8 CREST syndrome :
Fascial telangiectases
– syndrome CREST
• Overlap syndrome (Rare)

Figure 1.9 Mtted talengiectases of the

palms
 Organ Manifestation
• Vasculopathy
Raynaund phenomena
– Recurrent attacks of
vasospasm of small digital
arterioles/arteries at fingers
and toes
– RP appears suddenly, paintful
pallor/ischemia of single or
several digits/toes
– Severe cases cyanosis 
tricolore phenomenon Fig 1.9 A. Raynaund phenomena.
B. Limited disease with puffy fingers
 Skin Manifestation
• Puffy fingers sklerosis
• Sclerodactily
• teleangiektaksis,
 Skin extra manifestation
• Heart
• Larynx
• Lung
• GIT
• Kidney
 Examination

• Hystologi
• Laboratorium
 Differential diagnosis

• eosinophilic fasciitis,
• scleroderma circumscript,
• genedodermatosis sclerodermiform
 Therapy
• Morphea :
– Fototerapi
– Imunomodulator
 Systemic

Anti-inflammatory
Anti-fibrotic
Systemic corticosteroid
Penicillamine
Cyclophospamide
Penniciline infusion
Methotrexate
Azathioprine

Vasoactive
Low dose aspirin
Additional Recomendation
Calcium channel blocker Multidisciplinary care
Prostaglandin physical therapy
Angiotensin receptor blockers
Endotelin receptors antagonist
 Prognosis
• Prognosis is varies
• Morbidity including pneumonia, cardiac,
pulmonary dan renal.
THANK YOU