Background. Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology ...Background. Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. Case report. A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset of corticosteroid dependency, iatrogenic Cushing’ s syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient. Discussion. Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.展开更多
Necrobiotic xanthogranuloma is a rare cutaneous condition that can be mistaken for atypical necrobiosis lipoidica. It has a strong association with a paraproteinaemia, which may progress to frank haematological malign...Necrobiotic xanthogranuloma is a rare cutaneous condition that can be mistaken for atypical necrobiosis lipoidica. It has a strong association with a paraproteinaemia, which may progress to frank haematological malignancy. We describe four patients with variable cutaneous features, and their treatment response.展开更多
The clinical features of necrobiotic xanthogranuloma (NX)have been presented in a series of case reports, but there are hardly any reports on the clinical long- term course of this rare and usually chronic entity. The...The clinical features of necrobiotic xanthogranuloma (NX)have been presented in a series of case reports, but there are hardly any reports on the clinical long- term course of this rare and usually chronic entity. Therapeutic recommendations are mostly based on individual observations, lacking general therapeutic guidelines. To illustrate a typical chronic and progressive course of NX, we report the case of a 64- year- old woman with periorbital NX, ocular involvement and IgG paraproteinemia. Diagnosed with NX for the first time in 1993, the patient was already presented in this journal in 1995. Since then a series of therapeutic options including medication with dapsone, chlorambucil, interferon- alpha, clofazimine, melphalan, fumaric acid esters, surgical treatment and other physical therapies such as radiation, plasmapheresis, and photodynamic therapy have been applied. None of these therapeutic approaches, however, showed a satisfying long- term effect. At present the patient is undergoing cyclophosphamide- dexamethasone pulse therapy.展开更多
文摘Background. Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. Case report. A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset of corticosteroid dependency, iatrogenic Cushing’ s syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient. Discussion. Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.
文摘Necrobiotic xanthogranuloma is a rare cutaneous condition that can be mistaken for atypical necrobiosis lipoidica. It has a strong association with a paraproteinaemia, which may progress to frank haematological malignancy. We describe four patients with variable cutaneous features, and their treatment response.
文摘The clinical features of necrobiotic xanthogranuloma (NX)have been presented in a series of case reports, but there are hardly any reports on the clinical long- term course of this rare and usually chronic entity. Therapeutic recommendations are mostly based on individual observations, lacking general therapeutic guidelines. To illustrate a typical chronic and progressive course of NX, we report the case of a 64- year- old woman with periorbital NX, ocular involvement and IgG paraproteinemia. Diagnosed with NX for the first time in 1993, the patient was already presented in this journal in 1995. Since then a series of therapeutic options including medication with dapsone, chlorambucil, interferon- alpha, clofazimine, melphalan, fumaric acid esters, surgical treatment and other physical therapies such as radiation, plasmapheresis, and photodynamic therapy have been applied. None of these therapeutic approaches, however, showed a satisfying long- term effect. At present the patient is undergoing cyclophosphamide- dexamethasone pulse therapy.
