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  1. Systemic Disease
    Diffuse scleroderma; acrosclerosis; CREST syndrome; overlap syndrome.
  2. Localized Scleroderma
    1. Morphea
    2. Linear scleroderma: En coup de sabre; Romberg's disease (with facial atrophy)
    3. Generalized morphea
  3. Sclerodermoid Conditions
    1. Genetic: Progeria, Werner's syndrome
    2. Occupational: vibration, silicosis
    3. Metabolic: congenital porphyria, carcinoid
    4. Immunologic: Chronic graft verse host
    5. Chemical: Polyvinyl chloride, bleomycin
    6. Malignancy: carcinoid, bronchoalveolar
    7. Postinfectious: Lyme
    8. Neurologic: limb immobilization
  4. Diagnostic Criteria Developed By The American Rheumatism Association
    1. Major criteria: Proximal scleroderma
    2. Minor criteria:
      1. Sclerodactyly,
      2. Digital pitted scars, loss of subcutaneous of the finger pad,
      3. basal pulmonary fibrosis.
        (97% certain of the diagnosis if one major or two minor criteria are present)  Others Findings Pulmonary, Gastrointestinal, Musculoskeletal, Cardiac, Renal.
C/F – Cutaneous
  1. Cutaneous
    1. Hands and face are the most frequently involved
    2. The fingers may be oedematous and swollen, and the skin feels tight and has a shiny appearance. With increasing severity, the skin becomes immovable or hidebound
    3. Fingers develop fixed flexion contractures, causing reduced hand mobility and muscle atrophy
    4. Palmar telangiectasia
    5. Digital pitted scars
    6. Nails: nails become very small and atrophic nail fold capillary dilatation may show ragged cuticles
    7. In dark-skinned patients, vitiligo-like hypopigmentation may occur
    8. As pigment loss spares the perifollicular areas, a "salt-and-pepper" appearance of the skin may be seen; this pattern is most common on the scalp, upper back, and chest
  2. Facial
    1. The skin is bound down and hard
    2. Forehead is smooth and shiny
    3. Lines of expression are smoothed out
    4. Nose becomes small and pinched
    5. Mouth opening is constricted and radial furrows appear, giving a pursed appearance
    6. Lower eyelids cannot be depressed by the fingers to show the conjunctivae, because of atrophy of the tissues
    7. Rarely, periorbital oedema
    8. Small mat-like telangiectases are frequently found on the face

Description: DSC02093.JPG

Digital pitted scaring

  1. Serologic Findings
    1. Anti-RNP: mixed connective tissue disease
    2. Anti-centromere: CREST syndrome
    3. Anti-topoisonmerase I (anti-Scl-70): progressive systemic sclerosis
  2. Skin Biopsy
    In the dermis there are thick bundles of collagen running parallel to the skin surface, with entrapment of sweat glands and adenexal structures. The overlying epidermis tends to be thin and atrophic.
    1. Immunology
      1. ANA
      2. CREST: anti-centromere antibody positive
      3. MCTD: associated with ENA, anti-U1RNP positivity
      4. Systemic sclerosis: ANA, Scl 70 antibody positivity
  3. Sclerodermatous Syndromes
    Many conditions have widespread induration of the skin. It is beyond scope here to discuss individual conditions, but they should be borne in mind in the differential diagnosis of a sclerodermatous condition. Some conditions are more responsive to treatment than systemic sclerosis.
  4. Auto-immune or connective tissue diseases:
    1. Generalised morphea
    2. Progressive systemic sclerosis
    3. CREST syndrome (calcinosis, Raynauds, oesophageal dysmotility, sclerodactyly, telangiectasia)
    4. Mixed Connective Tissue Disease (MCTD): DM, SLE, scleroderma Eosinophilic fasciitis
    5. Chronic graft versus host disease
  5. Environmental agents or drugs:
    1. Bleomycin cancer chemotherapy.
    2. Eosinophilic myalgic syndrome following L-tryptophan ingestion.
    3. Occupational vinyl chloride, epoxy resin.
  6. Metabolic or associated conditions:
    1. Porphyrias: porphyria cutanea tarda, variegate porphyria, hereditary corpo-porphyrias
    2. Porphyria like conditions: pseudoporphyria, epidermolysis bullosa acquisita.
    3. Diabetic chrieopathy, scleredema.
  7. Infective, malignant, infiltrative conditions:
    1. Papular mucinosis, scleromyxoedema.
    2. Primary systemic amyloidosis.
    3. Cutaneous T-cell lymphoma.
    4. Lepromatous leprosy
​​Disease Severity Assessment by Modified Rodnan skin assessment score- 17 site assessment system most widely used.
  1. Treatment
    1. European League Against Rheumatism (EULAR).
    2. Scleroderma Trial and Research group (EUSTAR).
    3. Recommendations for the treatment of systemic sclerosis.
Treatments that have been tried include penicillamine, colchicine, ketotifen, cyclosporine, g -interferon, plamapheresis, PUVA and extra-corporeal photochemotherapy. Treatment is mainly symptomatic, or to prevent complications. Raynauds phenomenon and digital ischaemia may be controlled with calcium channel blockers such as nifedipine, supplemented with pentoxifylline, dipyridamole, intravenous prostacyclin infusion.

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