KINDLER-SYNDROME - A RARE CAUSE OF DESQUAMATIVE LESIONS OF THE GINGIVA

Kindler syndrome is a rare syndrome with cutaneous and intraoral manif estations. It has been suggested that there is an overlap between this syndrome and another called Weary syndrome. Only 68 cases of Weary an d Kindler syndromes have been reported, with fewer solely attributed t o Kindler syndrome. The salient cutaneous features are neonatal bullae , poikilodermal photosensitivity, and acral atrophy. This article pres ents the clinical intraoral findings of two siblings of consanguineous descent diagnosed as having Kindler syndrome. Both had an erythematou s and erosive appearance of the gingiva; one sibling had poor oral hyg iene and a rapidly progressive form of periodontal disease; the other, whose oral hygiene was acceptable, had no detectable bone loss.