An evaluation of the efficacy of topical application of salicylic acid for the treatment of familial cylindromatosis.

BACKGROUND: Familial cylindromatosis is a rare genetic disorder, giving rise to neoplasms of the skin appendages. We have recently shown that loss of the cylindromatosis tumour suppressor gene leads to activation of NF-kappaB, a transcription factor having antiapoptotic activity. This provides a pos...

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Główni autorzy: Oosterkamp, H, Neering, H, Nijman, S, Dirac, A, Mooi, W, Bernards, R, Brummelkamp, T
Format: Journal article
Język:English
Wydane: 2006
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author Oosterkamp, H
Neering, H
Nijman, S
Dirac, A
Mooi, W
Bernards, R
Brummelkamp, T
author_facet Oosterkamp, H
Neering, H
Nijman, S
Dirac, A
Mooi, W
Bernards, R
Brummelkamp, T
author_sort Oosterkamp, H
collection OXFORD
description BACKGROUND: Familial cylindromatosis is a rare genetic disorder, giving rise to neoplasms of the skin appendages. We have recently shown that loss of the cylindromatosis tumour suppressor gene leads to activation of NF-kappaB, a transcription factor having antiapoptotic activity. This provides a possible explanation for the deregulated growth of cylindromas. In cell-based assays, salicylate can prevent NF-kappaB activation caused by loss of the cylindromatosis gene, suggesting that salicylic acid application might be a potential treatment for cylindromatosis. OBJECTIVES: To assess the effectiveness of topical application of salicylic acid on familial cylindromas. METHODS: Cylindromas in five patients from four different cylindromatosis families were treated with twice daily and then once daily topical salicylic acid. Clinical response was determined by serial tumour measurements. RESULTS: In total 17 cylindromas in five patients were studied: 12 target lesions and five control lesions. The median size of the cylindromas was 1.0 cm (range, 0.6-2.8 cm). Two of the 12 cylindromas showed a complete remission. Another eight lesions showed some response, but not sufficient to qualify as partial remission. The control lesions remained stable or increased in size. CONCLUSIONS: Salicylic acid is a well-tolerated and potential new treatment for cylindromatosis.
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spelling oxford-uuid:6e869066-82bc-4d4c-9bfc-3f2999b2f7412022-03-26T19:25:05ZAn evaluation of the efficacy of topical application of salicylic acid for the treatment of familial cylindromatosis.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:6e869066-82bc-4d4c-9bfc-3f2999b2f741EnglishSymplectic Elements at Oxford2006Oosterkamp, HNeering, HNijman, SDirac, AMooi, WBernards, RBrummelkamp, TBACKGROUND: Familial cylindromatosis is a rare genetic disorder, giving rise to neoplasms of the skin appendages. We have recently shown that loss of the cylindromatosis tumour suppressor gene leads to activation of NF-kappaB, a transcription factor having antiapoptotic activity. This provides a possible explanation for the deregulated growth of cylindromas. In cell-based assays, salicylate can prevent NF-kappaB activation caused by loss of the cylindromatosis gene, suggesting that salicylic acid application might be a potential treatment for cylindromatosis. OBJECTIVES: To assess the effectiveness of topical application of salicylic acid on familial cylindromas. METHODS: Cylindromas in five patients from four different cylindromatosis families were treated with twice daily and then once daily topical salicylic acid. Clinical response was determined by serial tumour measurements. RESULTS: In total 17 cylindromas in five patients were studied: 12 target lesions and five control lesions. The median size of the cylindromas was 1.0 cm (range, 0.6-2.8 cm). Two of the 12 cylindromas showed a complete remission. Another eight lesions showed some response, but not sufficient to qualify as partial remission. The control lesions remained stable or increased in size. CONCLUSIONS: Salicylic acid is a well-tolerated and potential new treatment for cylindromatosis.
spellingShingle Oosterkamp, H
Neering, H
Nijman, S
Dirac, A
Mooi, W
Bernards, R
Brummelkamp, T
An evaluation of the efficacy of topical application of salicylic acid for the treatment of familial cylindromatosis.
title An evaluation of the efficacy of topical application of salicylic acid for the treatment of familial cylindromatosis.
title_full An evaluation of the efficacy of topical application of salicylic acid for the treatment of familial cylindromatosis.
title_fullStr An evaluation of the efficacy of topical application of salicylic acid for the treatment of familial cylindromatosis.
title_full_unstemmed An evaluation of the efficacy of topical application of salicylic acid for the treatment of familial cylindromatosis.
title_short An evaluation of the efficacy of topical application of salicylic acid for the treatment of familial cylindromatosis.
title_sort evaluation of the efficacy of topical application of salicylic acid for the treatment of familial cylindromatosis
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