Ehlers-Danlos Syndrome: Report of Two Cases with Hypophyseal Dysfunction

The Ehlers-Danlos syndrome is a welldefined and distinct clinical entity.¹ As its leading symptoms are cutaneous and its impediment appears at a time when children start to walk, most of the information on it appears in the dermatological and pediatric literature.^* Only a few reports have been published in journals of more general interest †; however, the disease is neither particularly a pediatric nor a dermatological one. It is thought of by most authors as a general developmental disturbance of the mesenchyme. The etiology is probably based on a germ plasma mutation, as emphasized by its hereditary and familial tendency and frequent coexistence with other congenital anomalies.¹⁰

The main constituent features or signs, not all of which need to be present in the same case, are in short¹⁰ as follows:

1. Hyperelasticity of the skin.

2. Hyperextensibility of joints.

3. Skin friability, especially over bony prominences as