Necrobiotic Xanthogranuloma

Necrobiotic Xanthogranuloma (NXG) is a rare non-Langerhans histiocytosis often associated with paraproteinemia or hematologic disease. NXG is usually characterized by yellow-orange, reddish-brown, or violaceous papules and nodules which may progress to form infiltrative plaques, with lesions often occurring in a periorbital distribution. Histopathologic study typically demonstrates bands of inflammatory granulomatous tissue separated by zones of necrobiosis, with the dermis and subcutaneous tissue most commonly affected. Historically, the diagnosis has been made based on consistent clinical and histologic findings, although diagnostic criteria have recently been proposed. Research on NXG is limited due to the condition’s rarity, making comparisons between therapeutic agents difficult. Numerous systemic and localized treatments have been documented with varying responses. Necrobiotic Xanthogranuloma (NXG) is a rare form of non-Langerhans histiocytosis first described by Steven Kossard and Richard Winkelmann (1924 -2012) in August 1980.1 NXG is characterized by yellow-orange, reddish-brown, or violaceous papules and nodules, which progress to form infiltrative plaques.2 While most commonly found on the periorbital skin, lesions may occur across multiple sites, including the trunk, extremities, and extracutaneous tissues.2 The histopathology is characterized by zones of necrobiosis separating bands of granulomatous infiltrate extending from the dermis to the subcutaneous tissue, with the epidermis often unaffected.1 Touton-type giant cells, foreign-body giant cells, and foam cells are also frequently appreciated, and cholesterol clefts may be present.3 Systemic paraproteinemia is associated with over 80% of patients with NXG and may precede or follow the initial presentation of cutaneous lesions for several years.4–8 Evaluation for potential underlying disease and effective treatment is necessary. As NXG is a rare condition, research is limited to case reports and retrospective studies, limiting assessment for efficacy of therapeutic agents. Numerous systemic and localized therapies have been used with varying response.2,9,10 We review the epidemiology, pathogenesis, clinical presentation, histopathology, diagnosis, and treatment of necrobiotic xanthogranuloma.