Cutaneous sclerosis

Specialty: Skin.

• diffuse skin tightening
• stiffened skin
• sclerodermiform induration

Why it occurs

• Diffuse progressive systemic sclerosis (multisystem autoimmune disease characterized by massive deposition of type I collagen in the dermis with loss of adnexa)
• Buschke scleredema syndrome (diffuse induration of the skin of the back, neck and shoulders frequently associated with long-standing insulin-dependent diabetes mellitus)
• Scleredema or scleromyxedema (abnormal deposition of mucin and collagen associated with monoclonal gammopathies of uncertain significance)
• Eosinophilic fasciitis or Shulman syndrome (deep sclerosis of the muscle fascia with peripheral eosinophilia after strenuous exercise)
• Chronic venous induration or lipodermatosclerosis in advanced stages in the lower limbs.

Initial workup

Measurement of skin thickness using the modified Rodnan scale. Laboratory tests for complete autoimmunity: antinuclear antibodies (ANA), anti-Scl-70 antibodies (anti-topoisomerase I), anticentromere antibodies, anti-RNA polymerase III antibodies (associated with renal crisis), serum protein electrophoresis and immunofixation, complete blood count (search for eosinophilia), and deep wedge skin biopsy that includes muscle fascia and muscle.

Standard management

• Methotrexate — systemic immunosuppressant indicated to limit the progression of cutaneous sclerosis in early phases of diffuse systemic sclerosis, dose of 15 to 25 mg once a week
• Mycophenolate mofetil — immunomodulator of choice in cases of cutaneous sclerosis with concomitant pulmonary interstitial involvement
• Penicillamine — chelating agent with antifibrotic properties, restricted use due to toxicity profile
• Urea-based protective emollient cream for daily mechanical massages that promote local tissue mobility.