Manifestaciones orales de la incontinencia pigmentaria (Síndrome de BlochSulzberger) Reporte de caso

Abstract

A 13-year-old female patient went to the pediatric dentistry service of the Oral Health Institute of the Central Air Force Hospital of Peru in September of 2018. She sought care to have her temporary partial prosthesis renewed. The extraoral clinical examination showed scar lesions on the skin of the nasal region; the intraoral evaluation showed the loss of multiple teeth, wide and thin edentulous ridges, alterations in dental morphology, as well as the inconsistency of the dental eruption with the patient’s age. The radiographic examination showed alteration and delay in dental eruption, impaction of a tooth in the jaw, lack of root development and persistence of temporary teeth. The definitive medical diagnosis was BlochSulzberger Syndrome, also referred to as “pigmentary incontinence”. This syndrome is characterized by presenting a variety of oral manifestations such as dental agenesis, alteration in dental morphology, retained and/or impacted teeth as well as alteration and delay in dental eruption. At a systemic level, this syndrome has lesions that affect the skin, hair, vision, central nervous system, among others.