1/6. Necrobiotic cutaneous T-cell lymphoma.We report 3 patients with granulomatous cutaneous T-cell lymphoma (CTCL) who showed necrobiosis histologically with palisading granulomas. Although granulomatous change may be present in up to 4% of cases of CTCL, necrobiosis is rare. Misdiagnosis may occur if epidermotropism is minimal or if atypical cells are masked by the granulomatous infiltrate. T-cell receptor gene analysis confirmed the presence of clonal T-cell populations in lesional skin from all 3 cases.- - - - - - - - - - ranking = 1keywords = necrobiosis (Clic here for more details about this article) |
2/6. necrobiotic xanthogranuloma associated with a benign monoclonal gammopathy.necrobiotic xanthogranuloma (NXG) is a disorder characterized by indurated, yellow-red nodules or plaques, primarily involving the face and, less frequently, the trunk and extremities. NXG may be associated with paraproteinemia, multiple myeloma, and hypertension. Histologically, xanthogranulomatous features with hyaline necrosis or necrobiosis are present. No first-line treatment has been established. This disease is a chronic process, and a patient's prognosis depends on the degree of extracutaneous involvement and the presence of visceral malignancies. We describe a patient with typical cutaneous and histologic findings of NXG with an associated monoclonal gammopathy.- - - - - - - - - - ranking = 0.5keywords = necrobiosis (Clic here for more details about this article) |
3/6. Clinicopathologic features of ulcerative-atrophic sarcoidosis.BACKGROUND: sarcoidosis is a chronic granulomatous disease of unknown etiology. Cutaneous disease is common and includes two clinicopathologic categories: granulomatous infiltration or a reactive phenomenon. In the granulomatous infiltrative group, clinical manifestations can be variable. Ulcers in sarcoidosis are uncommonly recognized and have been categorized previously under the rubric of atrophic, necrobiosis-like, or ulcerative sarcoidosis. patients AND methods: We evaluated retrospectively sarcoidosis patients presenting to the Johns Hopkins Department of dermatology between June 1989 and May 2002. Multiple skin biopsies were performed for histopathologic evaluation. Investigation for extracutaneous manifestations, including routine serologic assays, chest radiography, pulmonary function tests, electrocardiogram, and angiotensin-converting enzyme level, and referral for ophthalmologic examination were performed in all patients. RESULTS: Of 147 consecutive patients presenting with cutaneous sarcoidosis, seven demonstrated ulcerative-atrophic sarcoidosis lesions. All patients were African-American (five females and two males). All patients had ulcers surrounded by atrophic necrobiosis lipoidica-like plaques on the pretibial areas. All patients had other mucocutaneous manifestations of sarcoidosis, with the majority having evidence of internal disease. Combined immunosuppressive and immunomodulatory therapy was effective in controlling the cutaneous manifestations of all patients with ulcerative sarcoidosis. CONCLUSIONS: The ulcerative variant is a poorly defined subset of cutaneous sarcoidosis. Trauma, superimposed on atrophic plaques, appears to be the principal mechanism of this rare variant of cutaneous sarcoidosis.- - - - - - - - - - ranking = 1keywords = necrobiosis (Clic here for more details about this article) |
4/6. 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.- - - - - - - - - - ranking = 0.5keywords = necrobiosis (Clic here for more details about this article) |
5/6. 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.- - - - - - - - - - ranking = 0.5keywords = necrobiosis (Clic here for more details about this article) |
6/6. Necrobiosis in solitary mastocytoma: coincidence or pathogenesis?The coexistence of solitary mastocytoma and necrobiotic changes resembling granuloma annulare in the same lesion has not been reported to our knowledge. A 3 1/2-year-old child with a plaque on the arm clinically and histologically consistent with solitary mastocytoma showed characteristic necrobiotic foci indistinguishable from granuloma annulare. We speculate that mast cell degranulation may be involved in the pathogenesis of necrobiosis by altering fibroblast enzyme activity and/or producing prolonged inflammatory reactions.- - - - - - - - - - ranking = 0.5keywords = necrobiosis (Clic here for more details about this article) |