Necrobiotic xanthogranuloma: Response to chlorambucil

Ryan, Emma; Warren, Lachlan; Szabó, Ferenc János

doi:10.1111/j.1440-0960.2010.00710.x

Cited by 15 publications

(11 citation statements)

References 6 publications

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“…Chlorambucil is usually used in low‐doses (2–4 g/day) and in NXG with paraproteinemia. However, cases without associated haematologic disease treated with Chlorambucil have also been reported . The disadvantages of this treatment are the multiple complications of an increased risk of secondary malignancy and bone marrow suppression.…”

Section: Resultsmentioning

confidence: 99%

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Treatment of necrobiotic xanthogranuloma – a systematic review

Miguel

Lukács

Illing

et al. 2016

Acad Dermatol Venereol

109

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Necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the periorbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. NXG prognosis is poor with several treatments showing variable results. Treatment of monoclonal gammopathy with alkylating agents does not necessarily influence the activity of the skin disease and vice versa. The aim of this systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query 'necrobiotic xanthogranuloma treatment', both in English and German, about 'human' subjects and published between 1980 and 2014, documenting adequate treatment for NXG. Mainly individual case reports, small case series and retrospective studies were found. Treatment options include topical and systemic corticosteroids, thalidomide, high-dose intravenous immunoglobulin (IVIG), chlorambucil, cyclophosphamide, fludarabine, rituximab, melphalan, infliximab, interferon alpha, cladribine, hydroxychloroquine, azathioprine, methotrexate, laser therapy, radiotherapy, surgery, PUVA, plasmapheresis and extracorporeal photopheresis. Randomized controlled trials and studies on long-term outcomes after treatment were not found and are necessary to focus on in the future.

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Section: Resultsmentioning

confidence: 99%

“…Ryan et al . suggested the use of Chlorambucil as first‐line therapy in selected patients with NXG . Cyclophosphamide is a potent immunosuppressive agent, which affects cells in all phases of the cell cycle and causes as main side‐effects, nausea, vomiting, alopecia, leucopenia and urotoxicity with haemorrhagic cystitis.…”

Section: Resultsmentioning

confidence: 99%

Treatment of necrobiotic xanthogranuloma – a systematic review

Miguel

Lukács

Illing

et al. 2016

Acad Dermatol Venereol

109

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“…Recently, no treatment modality has been shown consistently effective. Treatment includes systemic or intralesional corticosteroids, chlorambucil, melphalan, topical mechlorethamine, dapsone, intravenous immunoglobulins, and lenalidomide [5, 6, 7]. Extracorporeal photopheresis has been used as well as autologous stem cell transplantation [8].…”

Section: Discussionmentioning

confidence: 99%

Necrobiotic Xanthogranuloma in a Patient with Multiple Myeloma

et al. 2016

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An 82-year-old woman presented with a 9-month history of multiple, well-defined skin lesions on her neck and upper chest, progressively increasing in size. Histological examination of a skin biopsy showed a regular epidermis. In the dermis, granulomatous changes with central necrobiosis were found which extended focally into the subcutaneous fat. The necrobiotic areas were surrounded by Touton cells and foreign-body giant cells. Laboratory analysis revealed leucopenia. Serum electrophoresis and immunofixation disclosed the presence of an IgG-λ paraprotein. Bone marrow aspiration cytology showed 20% plasmatic cells. The skeletal X-ray revealed frontal and occipital osteolytic skull lesions as well as pubic osteolysis. Urinalysis was unremarkable without proteinuria. Based on the clinical, laboratory, and histological findings, necrobiotic xanthogranuloma in association with multiple myeloma was diagnosed. The patient was treated with bortezomib combined with oral dexamethasone. Follow-up at week 13 revealed the complete disappearance of the IgG paraproteinemia. However, the skin lesions remained unchanged. Therefore, bortezomib treatment was discontinued, and systemic corticosteroids were continued at a higher dosage. After the steroid treatment, the lesions markedly flattened.

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“…While combinations of alkylating agents and systemic steroids are the most commonly reported modality, systemic steroids alone are minimally effective and associated with quick relapses [2]. Other treatments include psoralen with ultaviolet A (PUVA) [13], intravenous immunoglobulin [14], thalidomide [15], clorambucil [16], and lenalidomide [17]. Surgical excision is an option in certain cases [18].…”

Section: Discussionmentioning

confidence: 99%

Atypical Necrobiotic Xanthogranuloma Associated with Epidermodysplasia Verruciformis

2014

J Clin Investig Dermatol

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Necrobiotic xanthogranuloma is a rare, idiopathic, and chronic systemic granulomatous disorder that is usually associated with paraproteinemia of the immunoglobulin (Ig)G kappa (κ) type. It is clinically characterized by yellowish red-to-brown indurated often ulcerating nodules or plaques that usually involve the periorbital regions of the elderly. We report an atypical case of NXG affecting only the left leg of an adolescent girl with both IgG kappa and Lambda paraproteinemias. In addition, she had associated epidemodysplasia verruciformis and severe lung disease.

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Necrobiotic xanthogranuloma: Response to chlorambucil

Cited by 15 publications

References 6 publications

Treatment of necrobiotic xanthogranuloma – a systematic review

Treatment of necrobiotic xanthogranuloma – a systematic review

Necrobiotic Xanthogranuloma in a Patient with Multiple Myeloma

Atypical Necrobiotic Xanthogranuloma Associated with Epidermodysplasia Verruciformis

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