Structure of the Dermis in Type VIIC Ehlers—Danlos Syndrome

This report is the first to study histological, immunopathological, ultrastructural, and morphometric aspects of the abnormal structure of the dermis in Ehlers-Danlos syndrome, type VIIc. This disease, resulting from a defect in procollagen peptidase, resembles dermatosparaxis in animals. Dermal cells were abnormal in many aspects, including their large number, their strong argent affinity, and the occasional presence of oligocilia. Factor XIIIa-positive dendrocytes were, however, few in number. The stroma had many tears resulting from the absence of collagen bundles replaced by a loose meshwork of cauliflower fibrils