| necrobiotic xanthogranuloma (NXG) is a rare inflammatory histiocytic disease of the skin. Xanthogranuloma of the central nervous system is rare and few cases have been reported. To the authors' knowledge, there has been no previously reported case of NXG in which an intracranial lesion was found. This 52-year-old man, in whom NXG with all its cutaneous manifestations had been diagnosed, presented with three episodes of generalized tonic-clonic seizures. A contrast-enhanced computerized tomography scan of his brain revealed a bifrontal, dura-based mass lesion. The lesion was excised and reported to be an NXG that was similar, but not identical to the skin lesions. The patient was placed on a regimen of antiepileptic drug and chlorambucil after surgery. ( info) |
12/26. Upper and lower eyelid reconstruction for severe disfiguring necrobiotic xanthogranuloma. BACKGROUND: necrobiotic xanthogranuloma is a rare disease featuring generalized xanthomatous inflammatory skin lesions associated with paraproteinemia and possible lymphoproliferative diseases. Eyelid involvement can be unilateral or bilateral and ranges from minor xanthelasma-like lesions to severe ulcerative disease with consecutive keratitis and scleritis. CASE REPORT: The authors report the case of a 67-year-old woman with extensive necrobiotic xanthogranuloma involving the eyelids, head and neck, anterior chest, and both upper and lower extremities. Periorbital involvement caused severe upper and lower lid ectropium with chronic conjunctival inflammation and unilateral exposure keratitis. During a persistent period of low disease activity, granulomatous lesions and scars were widely excised, lids partially shortened and large full-thickness skin grafts applied. Uninvolved parts of the upper arms had to serve as donor sites, as other possible donor sites were not available. After successful reconstruction of the left side and no local recurrence of the disease, the right side was corrected in the same way. Full eyelid closure was achieved and skin grafts healed without complications. No recurrence of the disease appeared at the sites of operation, despite continuous new lesions elsewhere. CONCLUSION: Severe cicatricial eyelid deformation caused by necrobiotic xanthogranuloma can be treated with success by excision and free skin grafting. The mechanisms of recurrence at excision sites described by others remain unclear, but at least during phases of low activity, the described treatment is safe and recurrence is not to be expected. ( info) |
| A 53-year-old woman with necrobiotic xanthogranuloma presented as infiltrated eyelid plaques, with later development of arm and thigh lesions. Clinical features, including association with immunoglobulin g-kappa paraprotein, and pathological findings were typical of this disorder. Treatment for 15 months with varying combinations of prednisone and multiple chemotherapeutic agents (melphalan, cyclophosphamide and chlorambucil) has led to minimal or no improvement in the clinical lesions or paraprotein. The case demonstrates some of the difficulties in managing this unusual disorder. ( info) |
14/26. Granulomatous bronchiolitis with necrobiotic pulmonary nodules in Crohn's disease. A 37-year-old man with extensive Crohn's disease of the stomach, small and large intestine for almost a decade developed respiratory symptoms and radiological findings suggestive of pneumonia that failed to resolve with antibiotic treatment. Computed tomography scanning of his lungs showed extensive changes with cavitated parenchymal nodules. Histological evaluation of an open lung biopsy showed granulomatous bronchiolitis and pulmonary necrobiosis. Treatment with steroids and immunosuppression resulted in complete resolution of his clinical symptoms of pneumonia and abnormal computed tomography imaging changes. Granulomatous bronchiolitis and necrobiotic nodules may be a manifestation of Crohn's disease in the absence of microbial agents, including mycobacteria or fungal agents. While a multiplicity of complex pulmonary changes may occur in Crohn's disease, their clinical recognition and precise pathological definition may be particularly important if treatment with a biological agent, such as infliximab, is being considered. ( info) |
| necrobiotic xanthogranuloma (NXG) with paraproteinemia is a rare non-x histiocytosis with conspicuous lesions mainly located on the periorbital skin. A 68-year-old woman, with a previous history of lymphoplasmacytic lymphoma associated with IgG monoclonal gammopathy over a period of almost 4 years, presented typical lesions of NXG on the periorbital regions and left buttock. Treatment with dioxide carbon laser resulted in great improvement of cutaneous lesions, and no evidence of relapse after a 12-month follow-up. The association of NXG with lymphoplasmacytic lymphoma has not been previously described to our knowledge. The treatment of these lesions represents a true challenge for the clinician and palliative treatment of cutaneous lesions of NXG with CO(2) laser may constitute an alternative treatment in selected cases. ( info) |
16/26. An unusual cause of refractive chronic bilateral leg ulceration. BACKGROUND: Ulceration of the lower extremities is a common disorder that can be very painful. It occurs most frequently in the elderly population as a result of venous hypertension. We report an unusual case of a 32-year-old man with an 11-year history of extensive, painful, bilateral leg ulcers resistant to conventional treatment necessitating above-knee amputation of the left leg. Eventually, the patient was treated with prednisone, which led to almost complete healing of the ulcers of the right leg. OBJECTIVE: The objective of this report is to discuss a rare cause of chronic bilateral leg ulceration. methods: Detailed histopathologic examination showed a complex pattern of overlapping features of several specific dermatologic conditions, including necrobiosis lipoidica (NL), necrobiotic xanthogranuloma (NXG), and the destructive form of granuloma annulare (GA). CONCLUSION: The characteristics of our patient suggest a variant of a cutaneous necrobiotic disorder that has not been previously reported. When clinicians are confronted with recalcitrant ulcerations in the lower extremity, this group of disorders should be considered in the differential diagnosis. ( info) |
| 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. ( info) |
| necrobiotic xanthogranuloma is a systemic disease associated in most cases with monoclonal paraproteinaemia. Although the causative role of the paraproteinaemia is supposed, the pathogenesis of this disease remains unknown. We report a 65-year-old woman with a 28-year history of disseminated indurated plaques involving her face, trunk and extremities. Since 1994 an IgG kappa paraproteinaemia was present. Current biopsies showed typical histological features of necrobiotic xanthogranuloma. We treated this extraordinary widespread condition with melphalan and prednisolone. Although the serum levels of IgG kappa light chains and gamma globulins decreased, the cutaneous plaques extended rapidly in size and number, which casts doubt on the causative significance of the paraproteinaemia in the pathogenesis of necrobiotic xanthogranuloma. The paraproteinaemia in patients with necrobiotic xanthogranuloma may rather reflect a secondary phenomenon than the originating cause. Pathogenetic and therapeutical concepts based thereon that have been proposed so far should be critically reconsidered. ( info) |
19/26. Necrobiotic palisading granulomas associated with breast carcinoma. AIMS: The presence of granulomas within the stroma of carcinomas and in the lymph nodes draining carcinomas has been well described. To date, however, there have been few studies examining the occurrence and relevance of necrobiotic granulomas occurring in association with breast carcinoma.methods/RESULTS: Four cases of breast carcinoma with necrobiotic granulomas were examined using periodic acid Schiff and Ziehl Neelsen stains for fungi and tubercle bacilli and with immunohistochemistry using CAM 5.2, cytokeratin 7, and cytokeratin AE1/3 for tumour cells. In one case the stroma was involved, in the other three cases the lymph nodes contained necrobiotic granulomas. In two of the cases, one with stromal and one with lymph node involvement, the necrobiotic granulomas contained necrotic tumour cells.CONCLUSION: In this study the features of four cases of breast carcinomas with necrobiotic granulomas are examined and their relevance explored. Close scrutiny of such granulomas is necessary to avoid underdiagnosis of metastatic disease. ( info) |
| A 67-year-old man presented with a 13-year history of slowly enlarging yellow-red plaques on the face and lower extremity. A biopsy specimen was consistent with necrobiotic xanthogranuloma. necrobiotic xanthogranuloma is a slowly progressive histiocytic disease that is associated with paraproteinemia in most cases; however, its pathogenesis remains unclear. Although there is no first-line therapy, anecdotal reports have shown variable benefit with chemotherapeutic agents. ( info) |