Therapy of necrobiotic xanthogranuloma - case series andreview of the literature

Necrobiotic xanthogranuloma is a rare disease that is part of the non-Langerhanscell histiocytoses. It is characterized by yellowish skin lesions, which are typicallyperiorbitally localized. Extracutaneous manifestations of all organs are possi-ble and can cause potentially life-threatening complications. The disease alsobelongs to the facultative paraneoplasias and is often associated with parapro-teinemia. These aspects should be considered regarding further diagnostics. Dueto the rarity of the disease, there are no standardized guidelines for therapy sofar. The combination of prednisolone and chlorambucil as well as intravenousimmunoglobulins seem to be effective therapeutic options. We present four casesfrom our clinic as well as the current results of the literature in this mini-reviewand would like to highlight the therapeutic challenge as well as the need for thedevelopment of guidelines