Fig. 3: Lack of attached gingiva is pathognomonic for periodontal Ehlers–Danlos syndrome (pEDS) in young children.

In all affected children the attached gingiva is missing, and the thin and fragile mucosa extends to the free gingival margin and the interdental papillae. The free gingival margin and the papillae are normally keratinized and “thick.” In most individuals the generalized lack of attached gingiva is very clear on the photographs due to the extraordinary thin and translucent mucosa with increased vascular visibility (a, c, d, e, h, j, k); in one child A1 (i) it could only be diagnosed clinically by rolling the mucosa with a probe gently to the gingival margin. Gingival recession with exposed dental roots (= receding gums) was present in children C2 and F1 (a, b). Gingival recession, likely attributable to physiological dentition change, was seen near primary teeth of individuals D2, C2, E1, A1 (d, f, h, i). Gingival inflammation recognizable as red and edematous tissues due to insufficient oral hygiene and a hyperinflammatory reaction typical for pEDS was a frequent finding (b, c, d, e, f, h, j, l).