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Inherited epidermolysis bullosa dystrophica and squamous cell carcinoma- A case report
E Gayathri1, K Anbukkarasi1, S Mary Lilly1
1Department of Pathology, Sree Balaji Medical College and Hospital, Bharath University, Chennai, Tamil Nadu, India.
Abstract:
Epidermolysis bullosa dystrophica (EBD) is an inherited disease of the structural proteins in the upper dermis, characterized by blister formation at the site of trauma followed by scarring. Skin fragility and blistering are the hallmarks of this disease. Cutaneous squamous cell carcinoma (cSCC) is a dreadful complication in the epidermolysis bullosa (EB) patients and common cause of death. The recent advances in distinct tumor microenvironment explain the aggressive nature of SCC in recessive Recessive Dystrophic Epidermolysis Bullosa (RDEB) patients and the use of collagen VII re-expression as a possible therapeutic measure. Regular follow-up is a must in preventing complications.
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