Background: Inherited epidermolysis bullosa (EB) is a genetic disorder characterized by skin fragility and unique oral features.Aims: To provide (a) a complete review of the oral manifestations in those living with each type of inherited EB, (b) the current best practices for managing oral health care of people living with EB, (c) the current best practices on dental implant‐based oral rehabilitation for patients with recessive dystrophic EB (RDEB), and (d) the current best practice for managing local anesthesia, principles of sedation, and general anesthesia for children and adults with EB undergoing dental treatment.Methods: Systematic literature search, panel discussion including clinical experts and patient representatives from different centers around the world, external review, and guideline piloting.Results: This article has been divided into five chapters: (i) general information on EB for the oral health care professional, (ii) systematic literature review on the oral manifestations of EB, (iii) oral health care and dental treatment for children and adults living with EB—clinical practice guidelines, (iv) dental implants in patients with RDEB—clinical practice guidelines, and (v) sedation and anesthesia for adults and children with EB undergoing dental treatment—clinical practice guidelines.Each chapter provides recommendations on the management of the different clinical procedures within dental practice, highlighting the importance of patient‐clinician partnership, impact on quality of life, and the importance of follow‐up appointments. Guidance on the use on nonadhesive wound care products and emollients to reduce friction during patient care is provided.Conclusions: Oral soft and hard tissue manifestations of inherited EB have unique patterns of involvement associated with each subtype of the condition. Understanding each subtype individually will help the professionals plan long‐term treatment approaches.

The available literature on the orthodontic treatment of patients with rare disorders is extremely scarce. The aim of this study was to analyze the diagnosis and orthodontic treatment of a group of 94 individuals with rare diseases, referred for orthodontic evaluation to a university special care dentistry center (University of Santiago de Compostela, Spain). We created a control group of 94 systemically healthy individuals, paired by sex and age range. For all participants, we recorded their dental and skeletal abnormalities, oromotor dysfunctions and the characteristics of their orthodontic treatment. Some of the morphological and functional abnormalities were more prevalent in the rare disorders group than in the control group, including dental agenesis, microdontia, enamel defects, maxillary hypoplasia, overbite, cleft lip/palate, mouth breathing, atypical swallowing, lingual/labial interposition, labial incompetence, modified consistency diet, bruxism, and muscle tone abnormalities. Compared with the control group, the 56 patients with rare disorders who underwent orthodontic treatment required more desensitization sessions, used mixed appliances (fixed and removable) more often and for longer periods and had more frequent complications, such as gingivitis, caries, mucosal ulcers and recurrent debonding of the device. In conclusion, for selected patients with rare disorders, it is feasible to perform orthodontic treatment, whose planning will be determined by the dental-skeletal abnormalities and oromotor dysfunctions. Although complications are more frequent, they can typically be solved without having to stop treatment.

An 8-year-old girl diagnosed with cardiofaciocutaneous syndrome presented to our department with gingival pain, inflammation, and bleeding. Her medical history included hypoplasia of the corpus callosum, intellectual disability, trichothiodystrophy, global developmental delay, myopia, laryngomalacia, hypothyroidism, and osteoporosis. A diagnosis was reached of “periodontitis as a direct manifestation of systemic diseases”. During 9 years of follow-up, there were exacerbation episodes with spontaneous gum bleeding, ulcers in the interdental papilla, tooth mobility, and progressive tooth loss. Some of these exacerbation episodes resolved clinically with the administration of amoxicillin and metronidazole. We therefore proposed an oral microbiome study (subgingival and saliva samples) before and after antibiotic therapy. The most abundant genera at the subgingival level before administering antibiotics were Prevotella, Streptococcus, Fusobacterium, Leptotrichia, and Aggregatibacter. Of the 94 genera sequenced, 57 were less abundant in the post-treatment state than at baseline, particularly certain Gram-negative periodontal pathogens such as Porphyromonas, Treponema, Aggregatibacter, Fusobacterium, and Campylobacter. In contrast, other genera related to oral health, such as Haemophilus, Granulicatella, and Abiotrophia, showed an increase after administering the antibiotic. In conclusion, periodontitis exacerbations as a direct manifestation of systemic disease can occasionally be controlled exclusively with systemic antibiotics, without the need for performing mechanical periodontal therapy. This clinical recovery is correlated to substantial changes in the oral microbiome, which lead to the recovery of eubiosis of the microbiota.

Introduction and aim of the study: Childhood cancer is a rare disease that affects children and adolescents under 19 years of age and is one of the leading causes of mortality worldwide. Increased survival has led to the emergence of sequelae resulting from cancer treatment. The objective of this review was to describe the complications in dental development in this group of individuals after receiving cancer treatment. Methodology: An electronic database search and manual review were conducted to identify relevant studies. Cohort, cross-sectional, and case-control studies published in the last 20 years in English, Spanish, and Portuguese were included. The search strategy combined MeSH terms and free text. According to the PICO strategy, articles were selected that evaluated patients aged 0–19 years with childhood cancer treated with chemotherapy, radiotherapy, surgery, hematopoietic cell transplantation, and combination therapies. The outcome evaluated was the presence of long-term manifestations in dental development after cancer treatment. Results: Out of 116 studies, 21 were selected. The methodological designs were equitable (33.3% each). Leukemia was the most common childhood cancer (40.3%). Most patients received chemotherapy alone (71.6%), and the main dental abnormalities were root malformations (26.1%), enamel hypoplasia (24.8%), microdontia (24.6%), and tooth agenesis (17.8%). Conclusion: Antineoplastic therapies in pediatric oncology patients are associated with dental abnormalities. Integrating the dentist into the interdisciplinary team is key to preventing, diagnosing, and treating these manifestations.

Antecedentes: El Síndrome Cornelia de Lange (SCdL) es un síndrome que presenta características congénitas, físicas y conductuales específicas, y que varían entre cada persona. Es una enfermedad rara, que presenta una prevalencia de 1 entre 10.000 a 60.000 individuos. Descripción del caso: El presente artículo compara dos casos clínicos de personas con SCdL, atendidas en la la Clínica de Cuidados Especiales (CCE) de la Facultad de Odontología de la Universidad de Chile (FOUCh), en el cual se muestran distintos estados de salud bucal y por lo tanto diferentes aproximaciones terapéuticas, incluyendo al equipo multidisciplinario en la atención de ambos. Conclusión: Es importante que el odontólogo y el equipo de salud en general se adapten a las necesidades y condiciones de los pacientes, para realizar un tratamiento odontológico integral e individualizado para cada persona.