| Literature DB >> 21886433 |
Etienne Cardin-Langlois1, Dominique Hanna, Maxime St-Amant, Fréderic Croteau.
Abstract
Kindler syndrome is a rare, autosomal, recessive genodermatosis characterized by trauma-induced acral blisters in infancy and childhood, photosensitivity and progressive poikiloderma. Very few cases in the literature report an association with squamous cell carcinoma, even though it is a very well-known, long-term complication. A case involving a 23-year-old woman with a history of Kindler syndrome who was admitted to the department of plastic surgery (Sherbrooke University, Sherbrooke, Quebec) with an extensive ulcerated squamous cell carcinoma of the right hand is presented. A local excision of the tumour was initially performed, but positive margins and clinically palpable axillary lymphadenopathy over the course of hospitalization necessitated below-elbow amputation and lymph node dissection. To the authors' knowledge, this is the second reported case of aggressive metastatic squamous cell carcinoma of the hand in a patient with Kindler syndrome.Entities:
Keywords: Aggressive squamous cell carcinoma; Bullous epidermolysis; Kindler syndrome; Photosensitivity; Poikiloderma
Year: 2010 PMID: 21886433 PMCID: PMC2940973
Source DB: PubMed Journal: Can J Plast Surg ISSN: 1195-2199