Morphea is an autoimmune, inflammatory disorder characterized by excessive collagen deposition leading to localized skin induration and sclerosis without internal organ involvement (distinguishing it from systemic sclerosis). Subtypes include limited plaque, generalized, linear (including en coup de sabre and Parry–Romberg hemiatrophy), and deep morphea. Disease activity is marked by violaceous inflammatory borders and new/expanding lesions; activity typically transitions to a sclerotic, inactive phase over months to years, but relapses can occur. Early recognition and treatment—especially for linear and deep subtypes—prevent functional impairment and disfigurement. First-line therapies are high-potency topical corticosteroids or topical calcineurin inhibitors for superficial limited disease and methotrexate with or without systemic corticosteroids for active, extensive, or linear disease. Phototherapy (UVA1 or NB-UVB) is effective for many cutaneous forms.
Epidemiology and Risk Factors
- All ages; pediatric peak for linear morphea; female predominance.
- Triggers/associations: trauma (Koebner), radiation, infections, autoimmune diathesis; HLA associations variably reported.
Clinical Subtypes and Features
- Plaque (circumscribed): oval/sclerotic plaques with lilac ring (active border), central hypopigmentation/atrophy; trunk most common.
- Generalized: ≥4 plaques >3 cm in at least two anatomic sites; confluent induration.
- Linear: linear bands of sclerosis over limbs/face; may involve underlying fat, muscle, bone; en coup de sabre on forehead/scalp; Parry–Romberg (progressive facial hemiatrophy) overlaps.
- Deep morphea: morphea profunda, disabling pansclerotic morphea of childhood; deep tissue involvement.
- Activity signs: lilac/violaceous border, warmth, new lesions, extension; Inactivity signs: ivory sclerosis, atrophy, dyspigmentation.
Differential Diagnosis
- Systemic sclerosis (look for Raynaud’s, sclerodactyly, nailfold capillary changes, internal organ disease), lichen sclerosus (anogenital/porcelain-white plaques), eosinophilic fasciitis, lipodermatosclerosis, nephrogenic systemic fibrosis, scleromyxedema, chronic graft-versus-host disease.
Diagnosis and Workup
- Clinical diagnosis; skin biopsy confirms when uncertain: thickened collagen bundles, loss of adnexal structures, perivascular/periadnexal lymphoplasmacytic infiltrate.
- For linear/deep types: MRI or ultrasound to assess depth; dental/ophthalmologic/neuro eval for facial involvement; screen for limb-length discrepancies and joint contractures.
- Labs are nonspecific; ANA positive in a subset; autoantibodies (Scl-70/centromere) typically negative.
Management
- Limited superficial plaque disease
- High-potency topical corticosteroids (e.g., clobetasol) to active borders for 6–12 weeks; taper.
- Topical calcineurin inhibitors (tacrolimus 0.1% ointment) for face/intertriginous or maintenance.
- Intralesional steroids for small, progressing plaques.
- Phototherapy
- Medium/high-dose UVA1 (30–60 J/cm2) 3–5×/week for 6–12 weeks—effective for many plaque/generalized cases.
- NB-UVB or PUVA where UVA1 unavailable; NB-UVB useful for more superficial lesions.
- Systemic therapy (active, extensive, linear/deep, functional risk)
- Methotrexate 15–25 mg weekly (children: 0.3–0.6 mg/kg/week) with folic acid; combine with oral corticosteroids (prednisone 0.5–1 mg/kg/day) for 6–12 weeks then taper.
- Alternatives/intensification: mycophenolate mofetil for MTX intolerance/refractory; cyclosporine or IV pulse methylprednisolone in select severe pediatric cases.
- Refractory: rituximab or tocilizumab reported in small series; consider multidisciplinary input.
- Rehabilitation and Support
- Physical/occupational therapy for joint mobility; splinting for contractures.
- Dental/orthodontic and craniofacial surgery consults for facial linear disease sequelae; timing individualized after disease inactivity.
- Psychosocial support for cosmetic/functional impact.
- Monitoring
- Use activity/severity tools (e.g., LoSCAT: mLoSSI and LoSDI) to guide therapy.
- Treat until clear inactivity then maintain MTX for total 12–24 months to reduce relapse risk.
Prognosis
- Many cases remit with residual atrophy/dyspigmentation; linear/deep forms risk limb deformity, facial asymmetry, and disability without treatment.
References (recent guidelines and key reviews)
- Pediatric and adult morphea consensus statements, 2021–2024.
- UVA1/phototherapy evidence, 2021–2023.
- Methotrexate-based regimens and outcomes, 2022–2024.