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Morfeia

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Inflammatory disease primarily of the dermis and subcutaneous fat which leads to scar-like sclerosis.
 
Definition
  • Sclerosis (histologic term) → homogenized, abnormally enlarged and eosinophilic collagen with a paucity of admixed fibroblasts
    • ColagĂ©nio sem fibroblastos (esclerose)
    • ≠ Fibrose: aumento do colagĂ©nio e fibroblastos
 
Epidemiologia
  • Overall incidence: 4-27 per million per year
  • Age of onset: bimodal
    • 4-9 years (childhood)
    • 44-47 years
  • F:M = 3:1
 
Mecanismo: unknown
  • 3 components involved in formation of sclerosis
    • Vascular damage
    • Lymphocytic activation
    • Altered connective tissue production
        • notion image
  • Follows Blashko lines? Genetic mosaicism?
  • Antibodies against
    • single strand DNA (ssDNA)
    • Topoisomerase II alpha
    • Phospholipid-fibrillin 1
    • Histone
  • More than 1/3 of patients with plaque morphea have genital lichen sclerosus → two expressions of the same pathogenic process?
 
Etiologia/Triggers
Morphea
Systemic sclerosis
Local trigger
Systemic insult
Mechanical Trauma
X-Irradiation
Injection (Vaccination, Medications)
Infection (B. burgdorferi - theory abandoned)
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Apresentação clínica
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Classification
Peterson et al. 1995
Rook’s Book 10th Edition 2024
1. Circunscribed
2. Generalized/disseminated
3. Pansclerotic
4. Linear
5. Mixed
6. Deep
1. Limited
2. Generalized/disseminated
3. Linear
4. Pansclerotic
5. Mixed type (LS with Morphoea)
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  1. Plaque type (circunscribed, limited)
      • Slightly elevated, erythematous or violaceous, slightly edematous plaque
      • → centrifugal expansion
      • → central shiny white and peripheral violaceous or “lilac ring”
      • → post-inflammatory hyperpigmentation
      • Loss of hair glands → xerosis → pruritus
      • Variants
          1. Guttate morphea
          1. Atrophoderma of Pasini and Pierini
          1. Deep morphea
          1. Nodular or keloidal morphea
  1. Linear Morphea
      • Danger: in limbs, may involve fascia, muscles and joints
      • May have melorherostosis → linear hyperostosis
      • Variants
          1. Progressive hemifacial atrophy (Parry-Romberg Syndrome)
              • Trigeminal nerve distribution, including the eye and the tongue
          1. Linear morphoea en coup de sabre
              • Associated with CNS and ocular alterations
  1. Generalized Morphea
    1. Impaired thorax mobility → difficulty breathing
  1. Pansclerotic morphea AKA disabling pansclerotic morphea of children
    1. Typically before 14 years old
    2. Periodontal atrophy
    3. Lifelong disability
    4. DDx with Systemic Sclerosis difficult
DiagnĂłstico
  • ClĂ­nico - biĂłpsia se dĂşvida
  • Histologia
    • Sclerosis → + colagĂ©nio espessado e eosinofĂ­lico, fibrobastos escassos (≠ fibrose)
      • “homogenized, abnormally enlarged and eosinophilic collagen with a paucity of admixed fibroblasts”
    • BiĂłpsia quadrada - “squaro”derma
    • Retificação dos bordos
    • PlasmĂłcitos
    • Se eosinĂłfilos → diagnĂłstico diferencial com a fasceĂ­te eosinofĂ­lica
 
Investigations
  • ANA + (23-68%)
    • More severe
    • Deeper
    • More relapse
    • Helps to decide to treat more aggressively
  • Se morfeia em golpe de sabre
    • Consulta de oftalmologia
    • MRI (with or without contrast) → screen for CNS involvement
      • Every 1-2 years
  • Thermography
 
DDx
Morphea
Systemic Sclerosis
Eosinophilic Fasciitis
Assymetric
Symmetric
Symmetric
Histology: sclerosis
Histology: sclerosis
Histology: Eosinophils, thick fascia
No systemic involvement
Raynaud
Digital sclerosis
Gastrointestinal
Pulmonary
Eosinophilia
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  • Dupuytren contracture
 
Tratamento
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  • Systemic therapies (Bolognia)
    • Systemic corticosteroids for rapid disease control
    • MTX
    • MMF
    • JAK inhibitors (tofacitinib, baricitinib)
    • Hydroxichloroquine
    • Systemic retinoids (acitretin, isotretinoin) → vitamin A derivatives have antifibrotic effects and can modulate collagen metabolism
    • Tocilizumab (IL-6 blocker) in pansclerotic morphea in children
    • Abatacept (CTLA4 fusion protein that inhibits T-cell activation)
    • Dupilumab
  • Progressive hemifacial atrophy (Parry-Romberg Syndrome)
    • Treat aggressively - risk of progression to destruction the bone of the chin
    • Psychological and social support
    • 1st line
      • Corticosteroids (3 days 500mg of IV methyl prednisolone +/- oral)
      • Methotrexate (25mg)
    • If ongoing activity add in
      • MMF (500-1g)
      • MMF + Ciclosporin (low-dose in the acute phase)
      • Abatacept - must fail 2 DMARDs (takes up to 6 months to work)
    • If refratory consider
      • Tocilizumab
      • Apremilast
  • Treat resultant atrophy CAUTION
    • Autologous fat transfer +/- stem cells
    • Dermal fillers
    • Botox for muscle and TMJ spasm
    • Surgical correction (plastics, maxillofacial)
    • Risk of potential complications, change is anatomy - vessels more exposed and risk of serious harm/blindness