Kindler syndrome: Absence of definite ultrastructural feature

Kindler Syndrome in Native Americans From Panama

“…Pyogenic skin infections were common and in 7 patients (patients 5,6,19,20,21,23, and 24) were complicated by cases of acute glomerulonephritis. Because no skin cultures were obtained, it was not known whether the precipitating agent in these cases was a glomerulonephritic strain of group A streptococcus or another pathogen.…”

“…We were not able to determine the level of bullae on electron microscopic studies, and, similar to other reports to date, we found no definitive ultrastructural feature. 6 However, several articles report a pattern regarding KiS and ultrastructural findings such as marked duplication of the lamina densa, 5,17 basal layer separation, 18 and blistering with trauma above the level of the lamina densa. 16 Several conditions can cause blistering, cutaneous atrophy, and/or poikilodermalike skin changes.…”

Kindler Syndrome

“…The most frequently encountered cleavage plane is through the lowermost portion of the basal keratinocyte layer of the epidermis. 10,12,13 This finding correlates with structural defects in the anchoring of the actin filaments, which are localized to the lower portion of the keratinocyte. Cleavage planes have also been reported within the basement membrane, both through the lamina lucida and beneath the lamina densa.…”

A Very Rare Case of Kindler Syndrome

“…Osteoma in the spectrum of the syndrome has not been reported so far. No diagnostic ultrastructural features of the syndrome have been described to date 17 . Several reports communicate marked disorganization of the basement membrane with reduplication of lamina densa and cleavage at or close to the dermo-epidermal junction in cases of Kindler syndrome 18,19 .…”