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ID: 45

Short Link: https://gregory-ms.com/articles/45/

Discovery Date: 23 February 2021, 11:11:00 UTC

Published Date: 2021-01-01 00:00:00

Source: PubMed

Link: https://pubmed.ncbi.nlm.nih.gov/33614724/?utm_source=Chrome&utm_medium=rss&utm_campaign=pubmed-2&utm_content=10guX6I3SqrbUeeLKSTD6FCRM44ewnrN2MKKTQLLPMHB4xNsZU&fc=20210216052009&ff=20210223163220&v=2.

Manual Selection: none

Machine Learning Gaussian Naive Bayes Model: false


Skin Appendage Disord. 2021 Jan;7(1):66-70. doi: 10.1159/000511743. Epub 2020 Dec 16.


INTRODUCTION: Koenen tumors are benign, cutaneous manifestations of tuberous sclerosis. These are disfiguring, painful, and challenging to treat as they frequently recur. We report a case of long-standing, multiple Koenen tumors affecting all twenty nails in an elderly female who was successfully treated with a combination of topical sirolimus 1%, surgical excision, and electrofulguration.

CASE REPORT: A 57-year-old lady presented with multiple, asymptomatic periungual, and subungual tumors affecting all twenty nails since 27 years. Cutaneous examination revealed confetti macules, ash-leaf macule, and shagreen patch over trunk. Nail biopsy was compatible with Koenen's tumor. Computerized tomography of brain showed diffuse patchy sclerosis. The tumors were treated with topical sirolimus 1% ointment for 10 months with excellent regression. Electro-fulguration for both great toenails and surgical excision of right thumbnail periungual fibroma was done. 1% sirolimus was advised after the surgical treatment. There were no adverse effects or recurrence of tumors over a 2-year follow-up.

DISCUSSION: Topical sirolimus 1% was effective in tumor regression and preventing new tumor formation. Larger tumors that interfered in daily chores were treated with excision and electrofulguration. Thus, a combination treatment for this rare presentation of tuberous sclerosis provided optimum results.

PMID:33614724 | PMC:PMC7879313 | DOI:10.1159/000511743

Noun Phrases in Title

  • Multiple Koenen Tumors
  • a Rare Entity
  • Combination Treatment
  • 1% Topical Sirolimus Electrofulguration
  • Excision
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