[Cutaneous squamous cell carcinomas: a complication of severe forms of hereditary epidermolysis bullosa].

Soins

Service de dermatologie, Centre de référence des maladies rares de la peau et des muqueuses d'origine génétique (Magec) Nord, Site Saint-Louis, Hôpital Saint-Louis, AP-HP, 1, avenue Claude-Vellefaux, 75010 Paris, France. Electronic address:

Published: November 2023

AI Article Synopsis

  • Patients with severe hereditary epidermolysis bullosa have improved life expectancy and quality of life, but still face serious complications like cutaneous squamous cell carcinomas, which can be life-threatening from adolescence.
  • These carcinomas commonly appear on chronic wounds or scars in non-sun-exposed areas, and they are typically multifocal and recurrent, making diagnosis challenging.
  • Regular skin monitoring during dressing changes is crucial for early detection, while research is ongoing to explore non-surgical treatment options for affected patients.

Article Abstract

Despite an increase in life expectancy and quality of life for patients suffering from severe forms of hereditary epidermolysis bullosa, the occurrence of one or more cutaneous squamous cell carcinomas remains a sometimes serious complication, sometimes life-threatening as early as adolescence. These carcinomas occur preferably on chronic wounds or dystrophic scars in areas not exposed to the sun, and are generally multifocal and recurrent. Their clinical and histological diagnosis is difficult. Regular medical and paramedical monitoring of the skin during dressing repairs enables early detection and rapid, curative surgical management. The pathophysiology of these cutaneous carcinomas is the subject of research aimed at proposing non-surgical alternatives to the patients concerned.

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http://dx.doi.org/10.1016/j.soin.2023.09.010DOI Listing

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