Clinical Features and an Ultrastructural Study of Ehlers‐danlos Syndrome Type Ii

Histopathological and ultrastructural studies were carried out on a case of a 4‐year‐old boy with type II Ehlers‐Danlos Syndrome. The patient showed velvety skin, hyperelasticity of the skin, and mild hypermobility of the joints. Histopathologically, numerous very fine collagen fibers were observed in the dermis, and no recognisable group substance was found. Ultrastructurally, abnormal collagen fibrils with irregular profiles were interspersed in the reticular dermis. However, their periodic band pattern was that of normal skin collagen. It is supposed that these abnormalities of fibrils are due to abnormalities of collagen fibrillogenesis.