Scleroderma: Classification

There are two main forms as well as several subtypes of scleroderma:

• Chronic cutaneous circumscritical scleroderma (ICD-10 L94.-: Other localized diseases of connective tissue): confined to the skin and adjacent tissues such as subcutaneous fat, muscle, and bone; most common form of scleroderma A distinction is made between the following subtypes:
  • Plaque type (morphaea) – localization: trunk, usually multiple foci.
    • Sharply demarcated, round-oval foci.
    • Up to 15 cm in size
    • Three-phase development: 1. erythema (skin redness), 2. sclerosis (hardening), 3. atrophy (decrease)/pigmentation.
    • The single focus has a bluish-red border (“lilac ring” = sign of local disease activity).
    • The sclerosis is plate-like and ivory in color.
    • In the final stage, the single focus is brown pigmented and shrunken (= extinguished disease activity).
  • Linear type – affected are the extremities.
    • The focus is band or strip-shaped as well as sclerotic-atrophic.
    • The risk of joint contractures (stiffening of joints) is increased.
    • Soft tissue and muscle atrophy and defect condition are also possible.
    • Possibly “saber cut type” or hemiatrophia faciei (atrophy of one half of the face (soft tissue and bone)).
  • Special forms:
    • Superficial special forms – erythematous form (erythema, atrophy); guttate form (numerous small individual foci).
    • Generalized form – extensive skin involvement.
    • Deep special forms – subcutaneous scleroderma (nodular-keloid (proliferating) foci); eosinophilic fasciitis (Shulman syndrome) (affects the extremity fascia (fascia = soft tissue components of connective tissue) and subcutis (subcutis), not affecting the hands and feet; acute onset, chronic course).
• Systemic scleroderma (SSc, ICD-10 M34.-: Systemic sclerosis): it can also affect the internal organs, especially the gastrointestinal tract (gastrointestinal tract; in 90% of cases, the esophagus (esophagus) is affected), lungs (in 48% of cases), heart (in 16% of cases) and kidneys (in 14% of cases). Cases) may be affected; chronic systemic disease of connective tissue with no boundary The spread tends to be in two directions: diffuse, progressive, indistinctly bounded, no circumskripten single foci or systemic, no skin boundary A distinction is made between the following subtypes:
  • Limited-cutaneous systemic scleroderma (lSSc) – internal organs are rarely and late affected.
    • Acral type (I)
      • Hands and face are affected (acras (body ends such as nose, chin, ears, hands) and distal extremities (lower leg, foot, forearm, hand)).
      • Very low progression (progression).
      • Relatively good prognosis
    • Acral progressive type (II)
      • Hands and face are affected (acral and distal extremities).
      • Extension to arms and trunk
      • Esophageal sclerosis
  • Diffuse cutaneous scleroderma (synonyms: diffuse systemic scleroderma (dSSc); progressive systemic sclerosis) – rapid progression.
    • Central type (III) – diffuse scleroderma.
      • Begins on the thorax (chest) and face
      • Frequent and rapid sclerosis (hardening) of skin (all external skin) and internal organs
      • Poor prognosis
  • Special forms:
    • CR(E)ST syndrome (ICD-10 M34. 1) – combination of calcinosis cutis (pathological (abnormal) deposition of calcium salts), Raynaud’s syndrome (vascular disease caused by vasospasm (spasm of blood vessels)), esophageal dysfunction (Esophageal dysmotility; Esophageal dysfunction), sclerodactyly (scleroderma of the fingers), telangiectasis (usually acquired dilation of small, superficial skin vessels); special form of limited systemic scleroderma; slow progression (progression).
    • Overlap syndromes
      • Symptom overlap with dermatomyositis (muscle inflammation with skin involvement)/polymyositis (belongs to the collagenoses; systemic inflammatory disease of skeletal muscle with perivascular lymphocytic infiltration), mixed collagenoses, Sjögren’s syndrome, primary biliary cirrhosis

Systemic sclerosis (SSc) classification criteria.

Legend

• MCP: metacarpophalangeal joints.

Interpretation

• With a sum of at least 9 points, the disease can be classified as SSc.