Background: Surgical treatment of a recessive dystrophic epidermolysis bullosa patient is very difficult for both the surgeon and the anesthetist because of the fragility of the skin and abnormal nature of the tumor bed.
Objective: We report a case of 54-year-old Japanese recessive dystrophic epidermolysis bullosa patient with squamous cell carcinoma (SCC) of the lateral malleolus.
Methods: A tumor measuring 5.0 x 5.5 cm was surgically excised. The defect was then reconstructed by full-thickness skin grafting. To avoid airway complications, general anesthesia was administered using a face mask. Because the regional lymph nodes were swollen before surgery, the patient underwent sentinel lymph node biopsy.
Results: The patient remains well with no sign of recurrence or metastasis 7 months after surgery.
Conclusion: To preserve activities of daily living, surgery should be performed for squamous cell carcinomas arising in recessive dystrophic epidermolysis bullosa patients.
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http://dx.doi.org/10.1111/j.1524-4725.2004.30440.x | DOI Listing |
Oral Dis
January 2025
Facultad de Odontología, Universidad de Chile, Santiago, Chile.
Introduction: Junctional epidermolysis bullosa (JEB) is a rare genetic disease manifesting with skin and mucosal blistering. As part of the JEB, patients present with syndromic amelogenesis imperfecta (AI). Reports have described external crown resorption (ECR) in the teeth of patients with JEB, but its prevalence is unknown.
View Article and Find Full Text PDFPLoS One
January 2025
Assistant Professor of Health Information Management, Department of Health Information Technology, Ferdows Faculty of Medical Sciences, Birjand University of Medical Sciences, Birjand, Iran.
Minimum Data Set (MDS) enables integration in data collection, uniform data reporting, and data exchange across clinical and research information systems. The current study was conducted to determine a comprehensive national MDS for the Epidermolysis Bullosa (EB) information management system in Iran. This cross-sectional descriptive study consists of three steps: systematic review, focus group discussion, and the Delphi technique.
View Article and Find Full Text PDFJ Dermatol
January 2025
Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China.
Orphanet J Rare Dis
January 2025
EB House Austria, Department of Dermatology and Allergology, University Hospital of the Paracelsus Medical University, Salzburg, Austria.
Skinmed
January 2025
Baylor Scott & White University Hospital, Dallas, TX.
Filsuvez (birch triterpenes) topical gel received approval in 2023 for the treatment of epidermolysis bullosa (EB) in pediatric patients (aged ≥6 months) and adults. It promotes wound healing by modulating inflammation, encouraging new tissue formation, and maintaining the skin barrier. In a randomized, double-blind, controlled, parallel-group, phase III trial (EASE, NCT03068780), 223 patients were randomly assigned to two groups: the first group received treatment with birch triterpenes topical gel (study gel, n = 109), and the second group received treatment with vehicle gel (n = 114).
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!