Case 16 -

Necrobiotic Xanthogranuloma Noreen Walsh, MD

Necrobiotic xanthogranuloma was first described in 1980 by Kossard and Winklemann. It is a chronic, destructive, granulomatous, inflammatory process which mainly affects the skin/subcutis and the eyes. There is usually an accompanying paraproteinemia. The cutaneous lesions are characterized by multiple violaceous, indurated plaques and nodules which tend to exhibit a yellowish hue and can ulcerate. They show a predilection for involvement of periorbital skin, but this is not invariable and other parts of the face, the trunk and the limbs are often involved. Ophthalmic involvement has included iritis, scleritis, episcleritis and uveitis, as well as orbital rupture leading to blindness. Case 16 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 16 - Slide 2 Click to view with ImageScope Click to view with a Web-Based Viewer Histopathologically, necrobiotic xanthogranuloma is within the spectrum of cutaneous diseases characterized by palisading granulomata. Biopsies generally show extensive involvement of the dermis and subcutis by `necrobiosis' with associated granulomatous inflammation. The histiocytic population usually includes lipid-laden cells, sometimes of Touton-type, as well as multinucleated cells. Lymphoid nodules and collections of plasma cells are often seen. Secondary involvement of vessel walls by granulomatous inflammation is not uncommon. Aggregates of cholesterol clefts are variably present. While necrobiosis lipoidica diabeticorum is the main consideration in the histopathological differential diagnosis, florid granuloma annulare and xanthomatous conditions which accompany hematological disorders also need to be excluded. Apart from cutaneous and ocular manifestations of necrobiotic xanthogranuloma there are rare reports of cardiac, pulmonary and laryngeal involvement by the disease. Some patients have had hepato-splenomegaly, others arthritis or arthralgias, and a few, neuropathies. Other findings of variable frequency have included a plasmacytosis, a plasmaproliferative or lymphoproliferative disorder and multiple myeloma. Rarely patients have had hyperlipidemia or hyperglycemia. The course of the untreated disease is a chronic destructive one. In contrast to local modalities of therapy (with the exception of radiotherapy for localized disease) various types of systemic chemotherapy have proven most beneficial. Patients with this disease have died of various causes, sometimes due to hematological malignancies such as multiple myeloma, but also to other diseases unrelated to necrobiosis lipoidica. References Kossard S, Winklemann RK. Necrobiotic xanthogranuloma. Australas J Dermatol 1980; 21: 85-88. Kossard S, Winklemann RK. Necrobiotic xanthogranuloma with paraproteinemia. J Am Acad Dermatol 1980; 3: 257-270. Robertson DM, Winklemann RK. Ophthalmic features of necrobiotic xanthogranuloma with paraproteinemia. Am J Ophthalmol 1984; 97: 173-83. Finan M, Winklemann RK. Necrobiotic xanthogranuloma with paraproteinemia: A review of 22 cases. Medicine (Baltimore) 1986; 65(6): 376-88. Finan MC, Winklemann RK. Histopathology of necrobiotic xanthogranuloma with paraproteinemia. J Cutan pathol 1987; 14(2):92-99. Mehregan DA, Winklemann RK. Necrobiotic xanthogranuloma. Arch Dermatol 1992; 128:94-100. McGregor JM, Miller J, Smith N, Hay RJ. Necrobiotic xanthogranuloma without periorbital lesions. J Am Acad Dermatol 1993; 29: 466-9. Kossard S, Chow E, Wilkinson B, Killingsworth M. Lipid and giant cell poor necrobiosis lipoidica. J Cutan Pathol 2000; 27(7):374-378. Cyclophosphamide-dexamethasone pulsed therapy for treatment of recalcitrant necrobiotic xanthogranuloma with paraproteinemia and ocular involvement. Br J Dermatol 2005; 153:440-466. Wee SA, Shupak JL. Necrobiotic xanthogranuloma. Dermatol Online J 2005; 11(4):24.