Oral manifestations of Chediak-Higashi syndrome: A systematic review

Chediak-Higashi syndrome (CHS) is an autosomal recessive disorder characterized by leukocytes with giant secretory granules and a myriad of clinical features. However, it is unknown whether oral lesions are part of the syndrome or are refractory to systemic treatment. Herein, we integrated the available data published in the literature on the oral manifestations of individuals with CHS. Searches on PubMed, Web of Science, Embase, Scopus, and LILACS were conducted to identify studies published up to March/2022. The Joanna Briggs Institute tool was used for the critical appraisal of studies. Fourteen articles (21 cases) were detected. The mean age of individuals was 15.9 & PLUSMN;8.8 years. There was a slight predominance of males (52.4%). The major manifestation was periodontal disease (81%), although ulceration of the oral mucosa (14.3%), gingival/labial abscess (4.8%), and periodontal abscess (4.8%) were also reported. Oral rehabilitation including dental implants (9.5%) was performed after tooth losses due to the poor prognosis of periodontal therapy. CHS is usually diagnosed in an early stage due to its systemic manifestations such as classic oculocutaneous albinism, recurrent infections, and a propensity for bleeding. Oral health providers should be aware of the manifestations of individuals with CHS. Special care, including oral prophylaxis, is indispensable. & COPY; 2022 Elsevier Inc. All rights reserved.

Automated summary

This article summarizes the oral manifestations of individuals with Chediak-Higashi syndrome (CHS), including periodontal disease, oral mucosal ulceration, gingival/labial abscess, and periodontal abscess. Oral rehabilitation such as dental implants may be needed due to the poor prognosis of periodontal therapy. Oral health providers should be aware of these manifestations and provide special care and prophylaxis.