102 Citrullinated histone H3, a marker for neutrophil extracellular traps, is associated with poorer prognosis in recessive dystrophic epidermolysis bullosa patients with cutaneous squamous cell carcinoma

Abstract

Recessive dystrophic epidermolysis bullosa (RDEB) is a severe hereditary skin disease characterized by skin and mucosa fragility with blister formation due to type VII collagen deficiency. The major complication in RDEB patients is the development of squamous cell carcinoma (SCC) leading to premature mortality. Our study aims to correlate the immune profiles of SCCs with clinico-histopathological features in a cohort of RDEB patients. The immune cell profile was assessed by immunohistochemical analyses on 33 SCCs from 19 RDEB patients included between March 2016 and December 2021.