Abstract:

Kindler syndrome, as a rare subtype of Epidermolysis Bullosa, sets in motion a series of genetic conditions causing minor traumas and blisters on skin and making the skin susceptible to sunburn. The present study presented a case report of a 32-year-old female diagnosed with Kindler syndrome. Coronal and axial cone-beam computed tomography (CBCT) images clearly exhibited the agenesis of frontal sinuses. The condition was completely obvious in the images, which is a rare occurrence and has not been previously reported. The underdevelopment or aplasia of the paranasal sinuses is a rare phenomenon, which relates primarily to the frontal sinuses (12%) and secondarily to the maxillary sinuses (5-6%). Similarly, the agenesis of the sphenoid sinuses is an extremely rare condition. Therefore, raising our awareness about the paranasal sinus anomalies associated with the Kindler syndrome can lead to new discoveries about this syndrome. Further, with respect to the other patients suffering from Kindler syndrome, obtaining the basis of such knowledge together with evaluating CBCT or CT images in order to detect abnormalities can facilitate the management of the problems arising from paranasal sinus abnormalities.

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