Morphea

Localized scleroderma, circumscribed scleroderma, morphea

A self-limited autoimmune connective tissue disorder belonging to the group of collagenoses, characterized by fibrosing changes of the dermis and subcutis. Involvement of internal organs or transition to systemic sclerosis is extremely rare.

• Peak incidence: 2–8 years (children), 20–50 years (adults)
• Female:Male = 3–6:1
• Incidence: approx. 0.4–2.7 per 100,000/year (Europe)
• 85–90% of cases are Caucasian
• No official national registry in Switzerland; estimated prevalence similar to Western Europe
• Associated with polyarthritis, autoimmune thyroiditis, lupus erythematosus

Type I: Plaque-type morphea

• Most common in adults (70%), children (30%)
• Commonly affects the trunk
• Subtype: Atrophodermia idiopathica et progressiva (Pierini-Pasini type)

Type II: Linear morphea

• Most common in children (65%), adults (10–30%)
• Affects extremities and face
• Subtypes:
  • Linear morphea of the limbs
  • En coup de sabre (forehead involvement)
  • Generalized linear types

Type III: Deep morphea

• Involves subcutaneous tissue, fascia, muscle, periosteum
• Example: morphea profunda

Special forms

• Eosinophilic fasciitis (Shulman type)
• Disabling pansclerotic morphea
• Sclerofascia
• Hemiatrophia faciei progressiva (Parry-Romberg syndrome)

• Unknown trigger, immune-mediated mechanism
• Suspected triggers: trauma (Koebner phenomenon), infections (e.g., Borrelia burgdorferi), medications, radiation
• Pathomechanism: dysregulation of TGF-β, Th17 cell infiltration, fibroblast activation
• Genetic predisposition (e.g., HLA-DRB1*04 association)

• Initial phase: erythematous, edematous lesions with a lilac-colored edge (“lilac ring”)
• Later: centrally ivory-colored, sclerotic, atrophic plaques
• Long-term: shrinkage, atrophy, pigmentary changes
• En coup de sabre: forehead sclerosis with possible CNS involvement (epilepsy, hemifacial atrophy)
• Systemic symptoms are rare

• Clinical examination is usually sufficient
• Skin biopsy in unclear or deep lesions
• No specific serologic activity markers
• Laboratory work-up (for differential diagnosis):
  • CBC, creatine kinase (in suspected myositis), ANA (positive in up to 80%), ENA panel
  • Not recommended anymore but frequently done anyway: Borrelia serology (to exclude Lyme borreliosis)

Instrumental Diagnostics

• Skin thickness: Modified Rodnan Skin Score
• Induration: 20 MHz high-frequency ultrasound, cutometer, durometer
• Deep tissue involvement: MRI (preferred), CT in selected cases
• CNS imaging in en coup de sabre and Parry-Romberg cases

• Trunk (approx. 60%)
• Legs (25%)
• Arms (10%)
• Face/head (5%), especially in linear forms

• Gradual onset of asymptomatic or slightly painful skin lesions
• Possible triggering event (trauma, infection)
• No systemic symptoms
• Family history often unremarkable for autoimmune diseases

Early stage (inflammatory):

• Perivascular lymphohistiocytic infiltrates
• Edematous collagen in dermis
• Panniculitis, loss of adnexal structures

Late stage (sclerotic):

• Dense dermal sclerosis
• Homogenized, thick collagen bundles aligned parallel to skin surface
• Subcutaneous fat loss
• Narrowed vessels, fibrotic septae

• Joint contractures (especially in linear lesions over joints)
• Growth disturbances (in children)
• Facial asymmetry, soft tissue atrophy (e.g., Parry-Romberg)
• Psychological burden, especially in visible areas

• Usually benign, with spontaneous stabilization after 3–5 years
• Linear forms may persist
• No progression to systemic sclerosis
• Relapses may occur, especially after discontinuation of therapy

• No known primary prevention
• Protection from trauma after diagnosis is advised

1. AWMF Guideline Circumscribed Scleroderma. AWMF Registry No. 027/060 (2024)
2. Peterson LS et al. Epidemiology of morphea (localized scleroderma). Arch Dermatol. 1997;133(9):1121–7.
3. Zulian F et al. Pediatric Morphea: A multicenter survey. Br J Dermatol. 2020;183(4):669–77.
4. Foeldvari I et al. Current treatment recommendations for pediatric localized scleroderma. Rheumatol Int. 2022;42(1):25–33.
5. Mertens JS et al. Update on pathogenesis of localized scleroderma. Br J Dermatol. 2021;185(5):912–20.
6. Li SC et al. Linear Scleroderma “en coup de sabre” and Parry-Romberg syndrome: Neurologic implications. Neurology. 2023;100(1):e54–e61.
7. Marzano AV et al. Morphea: a review of clinical, pathogenetic and therapeutic aspects. J Eur Acad Dermatol Venereol. 2019;33(6):1051–61.
8. Jacobe H et al. Assessment tools for morphea: updated consensus. Arthritis Care Res. 2022;74(2):185–95.
9. Balci S et al. Use of mycophenolate mofetil in therapy-resistant morphea. Pediatr Dermatol. 2021;38(3):562–6.
10. Kreuter A et al. Medium-dose UVA1 phototherapy in the treatment of localized scleroderma. J Am Acad Dermatol. 2020;82(2):409–17.