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Detailed information of LOCALIZED SCLERODERMA
LOCALIZED SCLERODERMA
DEFINITION:
A multiorgan rheumatologic disease characterized by fibrosis
limited to the skin, subcutaneous tissue, and muscle and rarely
involving the visera.
EPIDEMIOLOGY:
- incidence: more common that Progressive Systemic Sclerosis
(PSS) (PSS)
- age of onset:
- more common in childhood than PSS
- risk factors:
PATHOGENESIS:
2. Types (2)
CLINICAL FEATURES:
2. Skin Manifestations
- one or more oval or round circumscribed indurations that
become hard and white
- found in skin anywhere on trunk or extremities
- characteristic violet border found early in disease
2. Linear
- one or more linear streaks on scalp, face, extremities
- found in skin but involve underlying subcutaneous tissue,
tendons, muscle, and bone -> contractions & deformities
- scleroderma en coup de sabre - lesions of face or scalp
- associated with significant local growth arrest -> atrophy
of the skin, subcutaneous tissue, and bone
3. Visceral Manifestations
- occasionally develops late suggesting an evolution of the
local to systemic forms of scleroderma
- occasionally develop other connective tissue diseases: SLE,
MCTD
INVESTIGATIONS:
- ANA present in 37-67%, pattern speckled, homogeneous, or
nuclear
- +/- anti-DNA antibodies
- increased Ig
- RF seronegative
MANAGEMENT:
- Morphea - regresses spontaneously thus no treatment needed
- Linear - may cause significant functional and cosmetic
problems
2. Drugs
- Morphea - penicillamine is drug of choice
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Pediatric Database - LOCALIZED SCLERODERMA
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