Necrobiosis Lipoidica

Excerpt

Necrobiosis lipoidica is a rare, chronic, and idiopathic disease characterized by collagen degeneration that causes skin lesions, typically on the anterior shin surface. This disease is classically associated with diabetes mellitus, typically type 1, and carries a risk of ulceration. Skin changes include thickening of the blood vessel walls, collagen deterioration, granuloma formation, and fat deposition. A major complication of the disease is ulcer formation, often occurring after trauma. Infections are uncommon. Moreover, if necrobiosis lipoidica becomes chronic, it may rarely progress to squamous cell carcinoma.

Necrobiosis lipoidica was first mentioned as dermatitis atrophicans lipoidica diabetica by Oppenheim in 1929. However, in 1932, Urbach renamed the disease necrobiosis lipoidica diabeticorum. In 1935, the first case was reported by Goldsmith in a nondiabetic patient. Subsequently, in 1948, Meischer and Leder described more cases of nondiabetic patients. In 1960, Rollins and Winkelmann published further information about cases of nondiabetic patients. Hence, the suggestion was put forth to exclude diabetes mellitus from the name of the disease. Today, a broader term—necrobiosis lipoidica—encompasses all patients with these characteristic lesions with or without diabetes mellitus.

Necrobiosis lipoidica is highly associated with diabetes mellitus and, particularly, high insulin requirements. However, necrobiosis lipoidica is also highly associated with metabolic syndrome, obesity, hyperlipidemia, hypertension, celiac disease, and autoimmune thyroid disease, suggesting additional causative etiologies. Necrobiosis lipoidica is a multifactorial skin disease with many possible contributing factors, for which ongoing new research and developments elucidate mechanisms for possible therapeutic interventions.