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Quiz

Progressive infiltration of the upper body

Figures

Fig 1. Thickened skin
Figure 1. Grossly thickened skin producing coarse folds.
Figure 2. Deep skin biopsy showing thickened dermis but no significant inflammation.
STEVEN KOSSARD, FACD

A 37-year-old man gave a 12-year history of slowly progressive swelling of his skin, particularly over his back and shoulders (see Figure 1), and proximal arms. The process had been asymptomatic and he had no associated Raynaud’s phenomenon. The man had a background of diabetes that had been managed with diet and oral hypoglycaemic agents for 10 years.
A skin biopsy from the back showed a marked increase in the thickness of the dermis with preservation of the dermal appendages (Figure 2). There was no gross collagen sclerosis or significant inflammation. The collagen bundles were separated by scant mucin.

Differential diagnosis

Progressive widespread induration of the skin may be caused by a number of conditions.
  • Scleroderma may produce diffuse thickening of the skin and is often associated with Raynaud’s phenomenon. Skin biopsy shows sclerosis of the dermis with thick collagen bundles, loss of appendages and inflammation. Tests for autoantibodies such as Scl-70 may be positive.

  • Scleromyxoedema is a rare skin disorder associated with sclerosis of the face and trunk, and rows of small papules in a radiating distribution. Skin biopsy shows increased numbers of fibroblasts, mucin and a lymphoplasmacytic infiltrate. Serum studies often show a paraprotein.

  • Nephrogenic systemic fibrosis presents as cutaneous sclerosis in patients who are usually on chronic renal haemodialysis. The process usually involves the limbs but may extend to the trunk. Skin biopsy shows dermal mucinosis, fibrosis and lymphocytic inflammation. The process may involve organs such as the liver, lungs and heart. Gadolinium used for MRI has been identified as the trigger inducing the fibrosis, which is progressive.

  • Scleroedema is the correct diagnosis and may be associated with diabetes or paraproteinaemia, or may follow an acute upper respiratory tract infection. Extracutaneous involvement of the heart, pleura, oropharynx, liver, spleen, bone marrow and muscle may be found. A deep skin biopsy is needed because only a thick dermis may be seen. Ultrasound may also be helpful in evaluating skin thickness.
    Treatments include oral steroids, antimalarials and immunosuppressives, but they are generally ineffective. Individual cases have responded to electron beam or PUVA therapy. Cases linked with streptococcal pharyngitis should be treated with antibiotics and are often self-limited.

Keypoint

Scleroedema is a rare, progressive thickening of the skin involving the upper trunk and limbs, and may be associated with diabetes.

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