Necrobiosis lipoidica (Urbach-Oppenheimer disease)

• Oppenheim 1929
• Urbach 1932

Necrosis lipoidica diabeticorum, dyslipoidosis cutanea (Oppenheim-Urbach), Oppenheim-Urbach syndrome, Urbach syndrome, dermatitis atrophicans lipoides diabetica.

Granulomatous systemic disease.

• Prevalence (in diabetics): 3/1,000
• Peak frequency in middle age
• Women : men = 2-3 : 1
• In 35% of cases in non-diabetics
• About 10-65% (depending on the reference) also have diabetes mellitus
• A total of 1% of all diabetics have necrobiosis lipoidica

• Pathophysiology still not understood

• Associations
  • Diabetes mellitus
  • Crohn's disease
  • Ulcerative colitis
  • Systemic granulomatosis
  • Hypertension
  • Granuloma anulare
  • Cutaneous sarcoidosis

• Mostly localised pretibially and/or on the dorsum of the feet, irregularly bordered, a sclerotic plaque with a brownish rim. Central hair loss is often noticeable, ulceration may occur.

• More extensive form of NL:
  • Granulomatosis disciformis chronica et progressiva (GDCEP), can also be considered separate entity.

• Anamnesis
  • Systemic diseases
• Clinic
• Biopsy if necessary
• Laboratory
  • Exclusion of diabetes mellitus
    • Fasting blood glucose, HbA1c, oGTT (oral glucose tolerance test)
• Blood pressure measurement
• TSH
• If necessary for inflammatory bowel disease
  • Colonoscopy

Most often on the shins.

Urbach described in 1929 as NL diabeticorum, which was shortened to NL as it later became clear that also non-diabetic patients can be affected.

Degeneration of collagen (necrobiosis) in the dermis, surrounded by histiocytes, multinucleated giant cells, and lymphocytes. Granulomatous inflammation, lipid-laden macrophages being responsible for the yellow colour of the lesions. Thickening of vessel walls and obliteration of small vessels. The epidermis is usually atrophic.

Ulcerations, scarring, superinfection, malignant transformation

Treatment of diabetes

• Spontaneous healing occurs in 20%
• However, there is often a chronic course

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