Sclerotic edema Buschke in diabetes. Affiliation: Endocrinology and Nutrition Department, Fundación Jiménez Díaz-Capio, Madrid, Spain. CC BY 3.0

Scleredema Buschke (scleredema adultorum of Buschke)

Definition

Sclerotic edema is a rare condition of unknown cause, but often associated with it d diabetes mellitus, Streptococcal infection (In the pharynx) or Monoclonal gammopathy. The skin becomes hard and tight in typical areas of the upper body.

Occurrence

Scleral edema is defined as one rare disease, with closer occurrence is unknown. Most commonly, the disease occurs in middle-aged, obese people with diabetes.

Symptoms

Gradually the skin and subcutaneous tissue become hard, especially over the neck and back. The front of the chest can also be attacked, but usually not arms and legs.

Diagnosis

Disease picture with hard skin, disease history with disease disposition (Diabetes, infection or Monoclonal gammopathy).

• Absence of changes in arms and legs (as opposed to Systemic sclerosis)
• No Raynaud's phenomenon or sclerodactylia (as known Systemic sclerosis)
• No eosinophilia (as by Eosinophil fasciitis)
• No kidney failure and MRI contrast agent (as of Nephrogenic sclerosis)

Blood tests

• No special effects. Fasting blood sugar, anti-streptolysin (AST) and electrophoresis to detect any associated diseases. ANA and differential-counting (eosinophilic) leukocytes are examined to assess differential diagnoses (Systemic sclerosis, eosinophilic fasciitis)

Incorrect diagnosis (Similar conditions, differential diagnoses)

• Systemic sclerosis
  • Raynaud's phenomenon
  • Hard fingers
    • ANA with subgroups (CENP, SCI-70)
• Eosinophilic fasciitis
  • Arms and legs are attacked
  • Attached skin often warm and reddish (in active phase)
  • High eosinophilic white blood cells (in blood tests and in tissue tests)
• Generalized morfea
  • Similar to morfea with plaque, but spread (generalized) over different skin areas.
• Sclero- myxoedema
  • Tissue test shows fibroblast proliferation in the skin
• Nephrogenic systemic fibrosis
  • After MRI examination with contrast agent when renal failure is present

Treatment

If there are disabling diseases, these should be treated as well as possible. If significant stiffness, physical therapy may be helpful to counteract more stiffness. Light therapy with UVA1 is being tested in some people.

Medical prognosis

Spontaneous recovery is common, but the condition often lasts for several years. It is unusual that internal organs are attacked

Literature

• Naman R, 2016
• Lemes LR, 2016

• Bindevevssykdommer.no
• Vaskulitt.no