Idiopathic gingival papillokeratosis with crypt formation: a report of 7 additional cases of an uncommon entity

Introduction

Idiopathic gingival papillokeratosis with crypt formation (IGPCF) is an uncommon condition of unknown etiology, characterized by keratotic plaques in the upper labial attached gingiva of young patients. No treatment is required, although periodic follow-up is recommended as some lesions persist. Despite the limited number of published cases, the diagnosis of IGPCF is highly suggestive based on the distinctive clinical features, and biopsy is usually unnecessary. We aim to report seven additional patients with IGPCF from Brazil and the United States.

Material and Methods

Seven IGPCF cases were retrieved from the archives of the Oral Pathology Laboratories from the Federal University of Rio de Janeiro, Brazil, and Texas A&M University, USA, between 2017 and 2023.

Results

Six patients were male, and one patient was female, with an average age of 15.8 years (ranging from 12 to 21). All patients presented with asymptomatic bilateral papillary white plaques located exclusively in the attached anterior gingiva with a duration ranging from 4 to 12 months. Six cases presented in the maxillary labial gingiva, whereas a single case was located in the mandibular labial gingiva. Clinically, the lesions appeared as well-demarcated symmetric white plaques with an irregular surface, stopping abruptly at the mucogingival junction. Incisional biopsies were performed on six patients with exuberant lesions, while an exclusively clinical diagnosis was established in a single patient with discrete plaques. Microscopic analysis revealed gingival mucosa showing overlying parakeratosis with papillary architecture and multifocal epithelial crypt-like invaginations with parakeratin plugging. Mild pseudoepitheliomatous hyperplasia was noticed, with no signs of epithelial atypia.

Conclusion

Clinicians should be aware of IGPCF and differentiate it from other papillary keratotic oral lesions. Reporting additional cases may provide further characterization of this unusual entity and improve understanding as it relates to etiology and prognosis.