1/3. Conjunctival necrobiotic granuloma in xeroderma pigmentosum.PURPOSE: We report clinical and histopathologic findings of a conjunctival lesion associated with xeroderma pigmentosum. methods: A Saudi girl with known xeroderma pigmentosum presented with pain and photophobia of the right eye and an elevated temporally located perilimbal mass. RESULTS: The mass was resected successfully and has not recurred during 1-year follow-up. Histopathologic examination of the tissue showed a necrobiotic granuloma with associated histiocytic infiltration. The patient had no systemic disease, lipid was not detected in the histiocytic lesion, no Touton giant cells were present, and there was no evidence of elastolysis. CONCLUSION: Ocular malignancies occur in association with xeroderma pigmentosum, but benign lesions that mimic a malignancy may occur.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/3. necrobiotic xanthogranuloma associated with choroidal infiltration and syncytial giant cell hepatitis.A 31-year-old woman developed necrobiotic xanthogranuloma (NXG), a thickened choroid, and syncytial giant cell hepatitis, a previously unreported association. NXG and syncytial giant cell hepatitis may have a common autoimmune pathogenesis.- - - - - - - - - - ranking = 6keywords = giant (Clic here for more details about this article) |
3/3. necrobiotic xanthogranuloma associated with paraproteinemia and non-Hodgkin's lymphoma developing into chronic lymphocytic leukemia: the first case reported in the literature and review of the literature.A 56-year-old married female presented in May 1998 with a 5-month history of xanthelasma of the eyelids, followed 4 months later by two enlarged lymph nodes of the left side of the neck and three of the left axilla. At the same time, she developed xanthomatous patches on the face, neck, and shoulders (Fig. 1). The cutaneous lesions were xanthomatous nodules and plaques, affecting the periorbital regions. Later, the whole face was affected, followed by ulcerated lesions on the scalp, chest, back, and extremities (Fig. 2). The skin lesions became painful, pruritic, ulcerated tumors (Fig. 3). In July 1998, computed tomography (CT) scans of the chest and abdomen with contrast medium showed pretracheal, bilateral axillary, right retrochural, paracaval, aortocaval, and para-aortic lymph node enlargement. These findings were suggestive of lymphoma. CT scan also showed slight heterogeneous hypodensity in the upper part of the right lobe of the liver, suggesting fatty infiltration. The spleen, pancreas, and suprarenal glands appeared normal. One cervical and two left axillary lymph nodes were excised. They revealed total replacement of the nodular architecture by a diffuse proliferation of mature lymphoid cells having small nuclei and a crumbled chromatin pattern, and very rare mitosis. It was concluded from the lymph node biopsies that these changes were typical of non-Hodgkin's lymphoma, diffuse and small cell type, of low-grade malignancy. A bone marrow aspirate showed a marrow heavily infiltrated by lymphoid cells with some immaturity. The megakaryopoiesis was adequate. Trephine biopsies showed similar changes. iron stores appeared to be absent. The bone marrow picture was consistent with diffuse, well-differentiated non-Hodgkin's lymphoma, developing into chronic lymphocytic leukemia (CLL). endoscopy showed antral-type gastric mucosa exhibiting mild chronic gastritis. skin biopsy from a fresh lesion on the back showed a diffuse inflammatory cell infiltrate with collections of histiocytic cells. It also showed necrobiotic foci, surrounded by mixed inflammatory cells, dark palisaded foamy histiocytes, and a few Touton giant cells. These findings are compatible with necrobiotic xanthogranuloma (NXG) (Figs 4 and 5). blood film showed normochromic, normocytic erythrocytes with anisopoikilocytotic leukocytes and normal platelets. The sedimentation rate was 90 mm in the first hour. The blood picture also showed monoclonal IgG paraprotein (3170 mg/dL) of the kappa light chain type. The patient was treated by the oncologist for her lymphoma, and was given Cytoxan, prednisolone, endoxan, Leukeran, and melphalan. She showed an excellent response to pulsed treatment with steroids (60 mg prednisolone orally daily for 5 days, repeated every month for 6 months). She also responded to Leukeran at a dose of 5 mg daily for 5 days every month for 6 months, and showed regression in the size of the lymph nodes. The treatment of her skin lesions was unsatisfactory in spite of the fact that she was given cyclosporine and both systemic and topical corticosteroids.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |