Oral pigmented lesions in syndromic individuals: A systematic review

Abstract

Objective: To systematically integrate the available data published in the literature on oral pigmented lesions (OPL) associated with syndromes, summarizing the clinical and demographic features of the individuals. Materials and Methods:An electronic search was undertaken in six databases.Eligibility criteria were articles in English, Spanish, and Portuguese describing case reports or case series of OPL associated with syndromes. Data were aggregated and statistically evaluated. Results: About 108 articles reporting 149 cases of individuals with syndromes were identified. Among the affected individuals, nine syndromes were reported. The mean age at diagnosis was 35.93 years (0.41 to 83 years), with a predilection for white (n = 85/85.86%) female (n = 102/68.46%) individuals. As regards the number of lesions, 109 (73.15%) were multiple and 40 (26.85%) were single. Lip represented the anatomical location more affected (122 cases/38.01%), followed by the buccal mucosa (100 cases/31.15%). Brownish lesions accounted for 82 (69.49%) cases. The mean time of evolution was 10.52 years (0.16 to 56 years). OPL preceding diagnosis of the syndrome was observed in 111 (74.50%) cases. Conclusions: Although these syndromes are uncommon, dentists should be able to recognize their manifestations, since oral manifestations can represent an important aspect in early diagnosis.

Oral Mucosal Lesions in Childhood

“…Hamartomatous polyps in the gastrointestinal tract can cause abdominal pain, chronic bleeding, anemia, and obstruction of the intestines [ 6 , 42 , 52 , 53 , 54 , 55 ], whereas 2 to 3% of the polyps show a tendency towards malignant transformation [ 1 , 6 ]. Polyps in the gastrointestinal tract may develop at any age, but pigmentations usually occur in early childhood [ 6 , 54 , 56 ]. Skin lesions are most commonly found around the eyes, on the fingers, and around the mouth, while intraorally, they are typically localized on the buccal mucosa and inner side of the lips [ 1 , 6 , 52 , 54 , 55 , 56 ].…”

“…Polyps in the gastrointestinal tract may develop at any age, but pigmentations usually occur in early childhood [ 6 , 54 , 56 ]. Skin lesions are most commonly found around the eyes, on the fingers, and around the mouth, while intraorally, they are typically localized on the buccal mucosa and inner side of the lips [ 1 , 6 , 52 , 54 , 55 , 56 ]. Lesions are round or oval, 2–5 mm in diameter [ 1 , 6 , 25 ].…”

“…Lesions are round or oval, 2–5 mm in diameter [ 1 , 6 , 25 ]. Their color varies from dark brown to black [ 6 , 25 , 56 ]. Lesions are asymptomatic, and the majority of intraoral ones fade before the first decade of life [ 52 , 54 ].…”

Peutz‑Jeghers syndrome: Skin manifestations and endocrine anomalies (Review)

“…So-called familial lentiginosis dermato-endocrine syndromes include PJS, Carney complex, Cowden disease and Noonan syndrome (43). A study published in 2021 that analyzed the published papers focusing on oral pigmented lesions (OPL) introduced 9 different syndromes in individuals with a mean age at diagnosis of 35 years and female predominance (68%) (44). Multiple lesions were more frequent than single (73.15% vs. 26.85%); lip followed by buccal mucosa were the more affected sites, in 75% of cases, OPL preceded the recognition of the syndrome (44).…”

Lingua villosa nigra in a child after COVID: Review and differential diagnosis with oral pigmentations of childhood

“…9 Laugier-Hunziker syndrome and Peutz-Jeghers syndrome are both characterized by multiple diffuse macules of oral mucosa, with the Laugier-Hunziker syndrome showing a tendency to involve the perioral skin. 10 They can be easily recognized for the positive family history and the extracutaneous findings. Another disregarded cause of paediatric oral pigmentation may be the accumulation of fabric fibres on dorsal surface of tongue of newborns who use to suck their cloths (Figure 2e).…”