1.2.3 Localized scleroderma

Grading & Level of Importance: C




Morphea, scleroderma circumscriptum.


Where present, most frequently between 20-40 years, 15%


Uncommon localized connective tissue disease with inflammatory border (lilac ring).

Aetiology & Pathogenesis

Unknown. In some cases tick bite induction proven.


Signs & Symptoms

Centrifugally spreading erythema with progressive central white-yellow induration with loss of adnexal structures. Border often with purple tones (lilac ring) as sign of disease activity.

Severe course with involvement of deeper structures possible, especially with linear scleroderma ("en coup de sabre" on scalp), facial hemiatrophy.


Varies with classification.


1. Limited forms: plaque-type morphea, guttate morphea, atrophoderma of Pierini-Pasini
2.Generalized forms: generalized circumscribed scleroderma, disabling pansclerotic morphea, eosinophilic fasciitis
3. Linear forms: linear circumscribed scleroderma including type "en coup de sabre", Parry-Romberg syndrome
4. Deep morphea (see www.AWMF guidelines).

Laboratory & other workups

1. General

  • routine laboratory tests
  • Borrelia serology
  • ANA

2. In children with severe linear forms: rheumatoid factor.


Interface dermatitis in early phases with varying intensity of inflammatory cellular infiltrates and changes of collagen structure depending on time of the disease course. Identical to progressive systemic sclerosis (see there).


Plaque-type morphea: usually self-limited within years leaving sclerotic, pigmented scars. Linear circumscribed scleroderma and hemifacial atrophy: chronic with significant impairment of growth and function.


Depends on localization (face, overlying joints), depth and extension (pansclerotic morphea).

Generally, no systemic involvement (in contrast to progressive systemic sclerosis). In patients with linear type "en coup de sabre" or Parry-Romberg syndrome, possible underlying bone and CNS involvement.


Clinical features, histology. In patients with linear type "en coup de sabre" or Parry-Romberg syndrome, cranial magnetic resonance imaging (MRI) and high resolution CT scan should be performed to document bone involvement and exclude CNS involvement.

Differential diagnosis

Lichen sclerosus (et atrophicus), acrodermatitis chronica atrophicans (borreliosis), dermatosclerosis associated to severe chronic venous insufficiency , progressive systemic sclerosis, eosinophilic fasciitis.

Prevention & Therapy

No causal therapy available.

  1. Topical therapy: corticosteroids; calcipotriol
  2. Intralesional corticosteroids
  3. Systemic therapy: methotrexate; corticosteroids; hydroxychloroquine
  4. Phototherapy: PUVA; bath PUVA; UVA-1
  5. Physical therapy: range of motion exercises; manual lymphatic drainage



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