Características dentoesqueléticas de pacientes com Epidermólise Bolhosa Distrófica Recessiva: série de casos

Abstract

Patients with Recessive Dystrophic Epidermolysis Bullosa (RDEB) manifest systemic conditions conducive to dental and skeletal alterations, significantly affecting facial aesthetics and the functionality of the stomatognathic system. This study aimed to analyze the dentoskeletal characteristics of 11 patients diagnosed with RDEB. Anthropometric measurements were collected, and cephalometric analyses were conducted based on reconstructions of tomographic examinations, along with evaluating the maturation index of cervical vertebrae. Of the sample, 72.7% were male, aged between 7 and 28 years, with body mass index ranging from 10.2 kg/m2 to 19.3 kg/m2. Cephalometric analysis revealed that only 27.3% of patients exhibited harmonious facial growth, while 45.4% showed vertical growth and 27.3% horizontal growth. The ANB angle ranged from 2.4° to 8.6°, with a mean of 5.0°, significantly deviating from the recommended population mean of 2° (p<0.05). Linear CoA and CoGn measurements in all patients, adjusted for sex and age, demonstrated values below the population mean. Assessment of cervical vertebrae did not reveal skeletal development delays in most patients relative to age. In conclusion, patients with RDEB predominantly exhibit vertical facial growth, a class II skeletal pattern, and deficiencies in maxillary and mandibular length, strongly correlated with body height.