When should Localized Scleroderma (LSA) be suspected in a patient?

When to Suspect Localized Scleroderma (LSA)

Suspect localized scleroderma in children presenting with skin hardening, sclerotic plaques, or linear lesions, particularly when involving the face, head, or limbs, with or without extracutaneous manifestations such as joint involvement or neurological symptoms. 1

Key Clinical Presentations by Subtype

Circumscribed Morphea

• Isolated porcelain-white plaques with areas of induration confined to skin and subdermal tissues 1
• Lesions may show follicular delling and occasional hyperkeratosis 1
• Typically self-limited but requires early recognition to prevent complications 2

Linear Scleroderma (Most Common in Children)

• Linear sclerotic bands affecting limbs, trunk, face, or head 1
• En coup de sabre variant: linear depression on forehead/scalp 3
• High risk for functional impairment and disfigurement if untreated 3
• May cross joints, causing contractures and growth defects 4, 3

Generalized Morphea

• Multiple plaques involving at least two anatomical sites 1
• More extensive than circumscribed but still confined to skin/subcutaneous tissues 2

Pansclerotic Morphea

• Severe, extensive involvement affecting multiple body areas 1
• Represents the most aggressive form requiring urgent intervention 1

Critical Extracutaneous Features Requiring Immediate Evaluation

Facial/Head Involvement

• Ophthalmological screening is mandatory at diagnosis for all patients with face/scalp lesions, including uveitis screening 1
• Brain MRI is highly recommended for all patients with face/head involvement, regardless of neurological symptoms 1
• Orthodontic and maxillofacial evaluation required at diagnosis and follow-up 1

Musculoskeletal Involvement

• Complete joint examination including temporomandibular joint assessment at diagnosis and follow-up 1
• MRI indicated when lesions cross joints to assess deeper tissue involvement 1
• Linear lesions have highest risk for joint contractures and limb length discrepancies 3

Epidemiological Red Flags

Age and Demographics

• Mean age at onset: 7.3 years, though can present from birth 1
• Female to male ratio: 2.4:1 1
• Incidence: 3.4 cases per million children per year 1
• Linear subtype represents the vast majority of pediatric cases 1

Distinguishing from Systemic Sclerosis

Key Differentiating Features

• Localized scleroderma is confined to skin and subdermal tissues with rare internal organ involvement 1
• Absence of Raynaud's phenomenon (characteristic of systemic sclerosis) 5
• No visceral fibrosis affecting heart, lungs, kidneys, or GI tract 3, 5
• Histologically: more prominent inflammatory infiltrate and papillary dermis involvement in localized form 6

When Systemic Evaluation is Needed

• Nailfold capillaroscopy should be performed if features suggest systemic sclerosis (puffy fingers, specific antibodies) 7
• Consider systemic sclerosis if patient has diffuse skin involvement proximal to elbows/knees 5

Immediate Action Required

Referral Pathway

• All children with suspected localized scleroderma must be referred to a specialized pediatric rheumatology center 1
• Early referral is critical as treatment must start before complications develop due to high morbidity risk 2
• Early diagnosis and intervention greatly improve outcomes 3

Common Pitfalls to Avoid

• Do not delay referral waiting for biopsy confirmation - clinical diagnosis is often sufficient with typical features 1
• Do not assume benign course - despite being "localized," significant morbidity from disfigurement and functional impairment is common 3
• Do not overlook extracutaneous screening - ophthalmological, neurological, and musculoskeletal involvement can occur even without obvious symptoms 1
• Do not miss the window for intervention - active disease requires immediate treatment to prevent irreversible damage 2, 3