Dermatology: Practical and Conceptual Image Letter | Dermatol Pract Concept. 2023;13(2):e2023108 1 A Midline Maxillary Tusk-Shaped Incisor in an Infant Mario Cutrone1, Vincenzo Piccolo2, Alejandro Ontiveros-Holguín3, Ramon Grimalt4 1 Department of Pediatric Dermatology, Ospedale dell’Angelo, Venice, Italy 2 Dermatology Unit, University of Campania Vanvitelli, Naples, Italy 3 School of Medicine, Universidad Autónoma de Durango Campus Chihuahua, Chihuahua, Mexico 4 Department of Dermatology, Universitat Internacional de Catalunya, Barcelona, Spain Citation: Cutrone M, Piccolo V, Ontiveros-Holguín A, Grimalt R. A Midline Maxillary Tusk-Shaped Incisor in an Infant. Dermatol Pract Concept. 2023;13(2):e2023108. DOI: https://doi.org/10.5826/dpc.1302a108 Accepted: September 21, 2022; Published: April 2023 Copyright: ©2023 Cutrone et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited. Funding: None. Competing Interests: None. Authorship: All authors have contributed significantly to this publication. Corresponding Author: Vincenzo Piccolo, MD, c/o II Policlinico, Edificio 9, Primo piano, Via Pansini 5 - 80131 Napoli, Italy. Tel: +39-0815666834 FAX: +39-0815468759 E-mail: piccolo.vincenzo@gmail.com Case Presentation A 4-month-old boy, who previously presented for a sizable gingival hemangioma on the lower gingiva two months ear- lier and was being treated with propranolol and monthly follow-up, now presented with a sudden onset of a white le- sion in the upper gingiva that caused great discomfort during breastfeeding to his mother one morning. Examination of the oral cavity revealed in the midline of the upper gingiva, a primary dental eruption reminiscent of a tusk or a fang due to its incurved crown and sharp vertex (Figure 1A). While waiting dental and maxillofacial surgery consulta- tions, the lesion abruptly disappeared. A small central muco- sal swelling with a slightly hemorrhagic pedicle at the site of detachment remained visible. Teaching Point Strictly speaking, this is not a neonatal tooth nor is it a na- tal tooth. That said, its early decay suggests a somewhat premature dentition with different structural characteristics than the transitional teeth that persist until the time of de- finitive dentition. Solitary median maxillary central incisor syndrome is a mild manifestation of the holoprosencephaly spectrum, typ- ically exhibiting an eruption of normal morphology in the midline of the maxillary alveolus at age of 8 months; pres- ent in both primary and permanent dentition [1,2]. An ini- tial head ultrasonography was performed with no anomaly being found. Considering the absence of other malformative elements, genetic evaluation was postponed to a later period of life. At age of 6 months, the boy developed an eruption of the upper left canine and lower central incisor teeth of normal morphology (Figure 1B). Acknowledgement: Patient parents in this manuscript has given written informed consent to the publication of her case details. 2 Image Letter | Dermatol Pract Concept. 2023;13(2):e2023108 References 1. Hall RK. Solitary median maxillary central incisor (SMMCI) syndrome. Orphanet J Rare Dis. 2006;1:12. DOI: 10.1186/1750 -1172-1-12. PMID: 16722608. PMCID: PMC1464380. 2. Garcia Rodriguez R, Garcia Cruz L, Novoa Medina Y, et al. The solitary median maxillary central incisor (SMMCI) syn- drome: Associations, prenatal diagnosis, and outcomes. Prenat Diagn. 2019;39(6):415-419. DOI: 10.1002/pd.5451. PMID: 30900264. Figure 1. (A) Tusk-shaped primary dental eruption in the midline of the upper gingiva at age 4 months. (B) Primary eruption of the upper left canine and lower central incisor teeth at age of 6 months.