morphea; localized cutaneous scleroderma; linear scleroderma

Introduction

Cutaneous sclerosis, localized, linear or generalized, limited to the skin.

Classification

• localized (1-3 lesions)
• linear
• generalized: 4 lesions affecting two separate anatomic sites
• pansclerotic

Etiology

• unknown
• may be associated with infection due to Borrelia burgdorferi
• rare complication of immune checkpoint inhibitors

Epidemiology

• onset between 20-50 years in 75%
• linear morphea has earlier onset
• pansclerotic morphea begins before age 14
• female:male ratio 3:1

Pathology

• generally limited to the skin
• pansclerotic form involving
• dermis
• fat
• fascia
• muscle
• bone
• destruction of underlying structures may result in growth disturbance in children
• dermatopathology:
  • epidermis: normal to atrophic with loss of rete ridges
  • dermis
    • initially, edematous with swelling & degeneration of collagen fibers
    • later, lesions become homogeneous & eosinophilic
    • slight perivascular or diffuse infiltrate with:
      • lymphocytes
      • plasma cells
      • macrophages
    • later, dermis thickened with few fibroblasts & dense collagen
    • inflammatory infiltrate at dermal-subcutis junction
    • dermal appendages progressively disappear
    • pansclerotic form
      • fibrosis with disappearance of subcutaneous tissue
      • sclerosis of fascia

* histopathology image

Clinical manifestations

• morphea plaques
  • occur on the face, head, neck, trunk (especially around the belt line), & lower extremities
  • usually oval & initially are indurated, with a yellow to brown color
  • when lesions are active, they are surrounded by a violaceous halo
  • over time, plaques may appear more atrophic & less indurated
  • lesions may be mildly painful or painless
• discrete sclerotic plaques
  • early: violaceous plaques
  • later: white, shiny center with erythematous or violaceous periphery
  • may be solitary, linear or generalized
    • circumscribed
      • generally involves trunk
      • limbs
      • face
      • genitalia
      • axillae (less common)
      • perineum (less common)
      • areola (less common)
    • linear:
      • extremities
      • head with or without hemiatrophy of face
    • generalized (pansclerotic)
      • trunk
      • extremities
      • face
      • scalp
      • sparing of fingertips & toes (no Raynaud's phenomenon)
  • size 2-16 cm in diameter
  • hair follicles & sweat duct orifices often disappear
  • plaques are hard in texture
  • plaques are often hyperesthetic
  • rarely accompanied by atrophy of underlying structures
  • hemiatrophy of the tongue with linear morphea
• scarring alopecia with scalp involvement of plaque
• may begin as pruritic pink rash
• lichen sclerosus et atrophicus
• nail dystrophy with linear lesions of extremities or pansclerotic form
• purpura, telangiectasia & rarely bullae may be seen later in the course of the disorder
• systemic manifestations (internal organ involvement) extremely rare
• Raynaud's phenomenon extremely rare

* images

Laboratory

• serology for Lyme disease
• biopsy with silver stain to r/o Borrelia burgdorferi

Diagnostic procedures

• pulmonary function testing
• doppler echocardiogram if evidence of pulmonary hypertension
  • right heart catheterization if further evaluation of pulmonary hypertension is needed

Radiology

• morphea affecting the neck, face, or scalp should undergo MRI of the head to evaluate for possible involvement of adjacent brain tissue

Complications

• generalized (pansclerotic)
  • flexion contractures of the hands & feet
  • atrophy & fibrosis of muscle
  • restricted respiration
    • pulmonary hypertension
• linear
  • atrophy ocular tissues
  • atrophy of bone

Differential diagnosis

• progressive systemic sclerosis (scleroderma)
• limited cutaneous systemic sclerosis
  • associated with scleroderma autoantibodies, specifically anticentromere antibody, thus anti-nuclear antibody positive
  • mnifestations may include calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, & telangiectasis
  • localized plaques are not a feature
• scleredema:
  • characterized by fibro-mucinous induration of the upper back, neck, upper arms, & occasionally the face
  • most commonly seen in association with diabetes (scleredema diabeticorum) or paraproteinemia.
• eosinophilic fasciitis
  • eosinophilic fasciitis involves the fascia, not specifically the skin
  • affects the extremities with painful diffuse induration
  • spares the hands & feet
  • skin may also have an orange peel appearance
• eosinophilic myalgia syndrome
• acrodermatitis chronica atrophicans

Management

• no effective treatment
• symptomatic management as indicated
• systemic glucocorticoids
• treatment of Lyme disease
  • high dose parenteral penicillin or ceftriaxone
  • concurrent systemic glucocorticoids to reduce sclerosis
• toclizumab for glucocorticoid-resistant morphea due to immune checkpoint inhibitor

More general terms

• systemic sclerosis

Patient information

morphea patient information