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Results for “Necrobiotic Disorders”

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At least 19 recordsLinked to original sources
PubMed2005

Chondrodermatitis nodularis helicis as a marker of internal disease [corrected] associated with microvascular injury.

Chondrodermatitis nodularis helicis (CNH) is held to be an idiopathic degenerative process involving the upper dermis of the auricular rim. Chondrodermatitis typically occurs in elderly men where associations with underlying trauma and sun exposure have been postulated as potential inciting triggers. Its association as a marker of systemic disease is not well established. We describe 24 patients with CNH, in whom there were also significant underlying diseases largely associated with vascular injury including those of immune-based etiology and/or conditions which have been previously linked with granuloma annulare, another necrobiotic process of collagen. These patients with concomitant systemic disease were characteristically younger compared to the classic demographics described for CNH. In some cases, chondrodermatitis may represent an ischemic necrobiotic disorder of collagen, potentially defining an important sign of underlying systemic disease in younger patients.

Adolescent↗
PubMedDate not supplied

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.

Adult↗
PubMed2002

[Necrobiotic xanthogranuloma , a cutaneous disorder associated with monoclonal gammopathy].

INTRODUCTION: Necrobiotic xanthogranuloma is a rare cutaneous disorder usually associated with monoclonal gammapathy. We describe two new cases. EXEGESIS: A 70-year-old patient was affected by a monoclonal gammopathy. She presented with a diplopia related to a retro-orbital tumor. The biopsy showed inflammatory lesions. Five years later, inflammatory xanthomatous skin lesions appeared. Biopsy specimens gave the diagnosis of necrobiotic xanthogranuloma. A 70-year-old woman was referred for inflammatory cutaneous lesions. Clinical, biological investigations and skin biopsies led to the diagnosis of cutaneous sarcoidosis associated with monoclonal gammopathy. Four years later, she developed a nephrotic syndrome. New skin biopsy specimens showed a necrobiotic xanthogranuloma. CONCLUSION: Necrobiotic xanthogranuloma is a systemic disease. It is a rare non-Langerhans cell histiocytosis characterized by frequent cutaneous and ophthalmologic lesions and associated with monoclonal gammopathy. To our knowledge, retro-orbital involvement has never been reported in necrobiotic xanthogranuloma. Treatment is difficult.

Aged↗
PubMed2006

Aggressive histiocytic disorders that can involve the skin.

Histiocytoses are a heterogeneous group of disorders that are characterized by the proliferation and accumulation of reactive or neoplastic histiocytes. Three classes of histiocytoses have been defined: class I, Langerhans cell disease; class II, non-Langerhans cell histiocytic disease without features of malignancy; and class III, malignant histiocytic disorders. Although the disorders in classes I and II usually have a benign appearance on histology and are commonly non-aggressive and self-healing, some can cause debilitating or even fatal outcomes. Such cases beg the question: what stimulates aggressive behavior of a classically benign disease? New molecular information may now provide insight into the driving force behind many of the aggressive histiocytoses. In this article, we review Langerhans cell disease and seven aggressive histiocytoses that can involve skin, discuss histologic features that may forecast a poor prognosis, and discuss the molecular findings that help to explain the pathophysiology of these aggressive histiocytic disorders.

Histiocytosis↗
PubMed2006

[Long-term course of necrobiotic xanthogranuloma with ocular involvement].

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.

Eye Diseases↗
PubMed1994

[Necrobiotic xanthogranuloma in IgG kappa plasmacytoma and Quincke edema].

Necrobiotic xanthogranuloma is a non-X histiocytosis with unknown pathogenesis. It is associated with paraproteinaemia, and in rare cases with multiple myeloma. A decreased level of C1 inhibitor has been found in several cases without clinical manifestations of Quincke oedema. We report on a patient with necrobiotic xanthogranuloma and myeloma, in whom we found a decreased level of C1 inhibitor and recurrent episodes of manifest Quincke oedema. The indirect detection of auto-antibodies against the paraprotein with development of immune complexes is regarded as an explanation for the consumption of the C1 inactivator and the manifestation of Quincke oedema. The possibility of a causal relationship between paraproteinaemia and necrotic xanthogranuloma is discussed.

Adult↗
PubMed1995

[Necrobiotic xanthogranuloma with eye involvement. Overview and case report].

Necrobiotic xanthogranuloma is a rare disease marked by yellowish-brown plaques with destructive growth and a tendency to ulceration, which shows a characteristic histopathological pattern of palisading granuloma with foam cells, Touton's and foreign-body giant cells, bizarre multinuclear giant cells, cholesterol clefts and marked necrobiosis of the connective tissue. Necrobiotic xanthogranuloma is associated with IgG paraproteinaemia, and occasionally with an IgA paraproteinaemia. Ocular symptoms and visceral involvement are common. We report a case of a 53-year-old woman with periorbital necrobiotic xanthogranuloma, ocular involvement and IgG paraproteinaemia, who had undergone argon-laser treatment to no avail following a diagnosis of xanthelasma. Periorbital xanthelasma-like lesions with a tendency to ulceration in combination with unexplained ocular symptoms consisting of keratitis, scleritis, episcleritis or uveitis should suggest the diagnosis of necrobiotic xanthogranuloma, especially if they are associated with paraproteinaemia.

Diagnosis, Differential↗
PubMed2007

Necrobiotic xanthogranuloma successfully treated with autologous stem cell transplantation.

Paraproteinemia can be complicated by necrobiotic xanthogranuloma. Therapeutic options for this progressive disease are limited, and there is no agreement on a single best strategy. We report the case of a patient with a massive periorbital infiltration narrowing the palpebral fissure and blinding the patient. Conventional myeloma therapy had only limited benefit in our patient. However, he was successfully treated with high-dose chemotherapy followed by autologous stem cell transplantation, rendering the patient free of symptoms. This is the first report of autologous stem cell transplantation in a patient with necrobiotic xanthogranuloma.

Adult↗
PubMed2002

[Degos atrophic malignant papulosis. Not always malignant!].

A patient developed malignant atrophic papulosis with only cutaneous manifestation. Repeated coloscopy uncovered no malignant papules in the colon. Referring to the literature, the value of permanent anticoagulant therapy is discussed. In contrast to the term "malignant" atrophic papulosis, there also seems to be a variant with a benign clinical course.

Adult↗
PubMed1992

Necrobiotic xanthogranuloma in a human T-lymphotropic virus type 1 carrier.

A 67-year-old man who had necrobiotic xanthogranuloma associated with paraproteinemia is described. He was a human T-lymphotropic virus type 1 (HTLV-1) carrier who had a high titer of circulating anti-HTLV-1 antibodies and neurologic abnormalities that suggested HTLV-1-associated myelopathy. The patient's necrobiotic xanthogranuloma and neurologic symptoms improved after he received four 5-day courses of melphalan, 2 mg/day, and prednisolone, 20 mg/day, at 4-week intervals.

Aged↗
PubMed1995

Unusual clinical manifestation of a disfiguring necrobiotic granulomatous disease.

A 39-year-old woman had a disfiguring granulomatous disease of 36 years' duration. Nodules appeared on her lower extremities and later extended to her face; this led to ulceration and destruction of the nasal cartilage. This disease of unknown origin does not conform to any previously described entity. Some patients with a similar case presentation have been observed in France.

Adult↗