![]() |
Case Report
1 Internal Medicine Resident, Department of Medicine at the University of Florida, Gainesville, FL 32610, USA
2 Department of Medicine at the University of Florida, Gainesville, FL 32610, USA
3 Associate Professor, Department of Medicine at the University of Florida, Gainesville, FL 32610, USA
Address correspondence to:
Matthew D Bloom
1600 SW Archer Road, Room 4102, Gainesville, FL 32610-0277,
USA
Message to Corresponding Author
Article ID: 100097Z10MB2021
Introduction: Netherton syndrome (NS) is a rare multisystem disorder with both cutaneous and extra-cutaneous manifestations. The syndrome includes the presence of congenital ichthyosis, exhibited by hyperkeratosis and abnormal skin scaling over the entire body. In rare instances, NS has been associated with the development of squamous cell carcinoma (SCC).
Case Report: A 54-year-old male with a history of NS and recurrent squamous cell carcinoma presented with an enlarging left inguinal mass. Biopsy of the mass was positive for a new invasive squamous cell carcinoma with regional lymph node involvement and invasion of the left femoral vein. The patient underwent wide local resection, superficial and deep regional lymph node dissection, and resection of the left femoral vein. His hospital course was further complicated by multiple infections and progression of his cancer. He eventually transitioned to hospice care before receiving any additional treatment for his malignancy.
Conclusion: This case highlights a patient with NS and a history of recurrent inguinal squamous cell carcinoma. Further investigation is needed to better highlight the complexity of factors associated with the development of squamous cell carcinoma in patients with NS and other forms of ichthyoses.
Keywords: Netherton syndrome, Squamous cell carcinoma
NIH/NINDS R01NS102624
Author ContributionsMatthew D Bloom - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Janine A Gilkes - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Samer Z Al-Quran - Analysis of data, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Coy D Heldermon - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2021 Matthew D Bloom et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.