Kindler Syndrome: A Multidisciplinary Management Approach

Torres-Iberico R.; Condori-Fernández Y.; Apagüeño-Ruiz C.; Andia-Ticona M.; Pomar-Morante R. · Actas Dermo-Sifiliograficas (2020) · DOI: 10.1016/j.ad.2019.04.013

Resumen

Kindler syndrome is a very rare form of bullous epidermolysis. It is a hereditary condition caused by a mutation in the FERMT1 gene that encodes the protein kindlin-1. It is clinically characterized by trauma-induced blistering, diffuse skin atrophy, poikiloderma, pseudosyndactyly, and photosensitivity. The most common mucosal manifestations are conjunctivitis, ectropion, hemorrhagic gingivitis, periodontal disease, premature tooth loss, and severe colitis. We present the first 4 cases of Kindler syndrome diagnosed at the Instituto Nacional de Salud del Niño in Lima, Peru. These cases highlight the unique clinical presentation and multiple manifestations of this disease and show how a multidisciplinary management approach kept symptoms under control and significantly improved patient quality of life.