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Necrobiosis lipoidica ��NL) is a dermal connective tissue-involved disease characterized by scleroderma-like patches in the pretibial area. The pathogenesis of NL remains unclear, and is likely associated with diabetes mellitus, immunologic mechanism, collagen degeneration, blood plaque abnormality as well as trauma. No effective treatment is available for NL. Corticosteroid, immunotherapy, TNF antagonist, fumaric acid esters and surgical care may be applied in its treatment. This paper describes the latest progress in the research on NL pathogenesis, treatment and prognosis.