Von Recklinghausen osteitis fibrosa cystica. A clinical case [Osteite fibroso cistica di Von Recklinghausen. Caso clinico.]

In Recklinghausen's disease the skeleton lesions are often the first signal of the pathology. The main clinical manifestations are represented by bony lesions which appear as multicystic lesions with loss of the hard lamina and skull malformation and asymmetry. In this disease there is a relevant osteoclastic activity which prevails over the osteoblastic one associated to the fibrous substitution of the marrow, sometimes producing micro or macro cysts. The typical alteration consists of an increase along the endosteal and trabecular surfaces in the number of osteoclasts which can be found in small reabsorption gaps. This is the cause of a cortical and trabecular reduction which can appear as interrupted. Histologic lesions consist in the replacement of bone tissue with fibrous and osteoid tissue. These bony lesions are not characteristic of the disease but to be distinguished from other pathologies such as for example Paget's disease and other forms of fibrous dysplasia (Gardner's syndrome, Leontiasis ossea). A case personally observed is described: a women, 29 years old, suffering from Recklinghausen's disease with face and skull asymmetry, condyles and glenoid cavity deformation, abnormal face reduction. Observing the planigraphy on the right side of the temporomandibular articulation, flattened glenoid cavity and condyles with irregular outlines can be noticed, aplastic coronoid cuts, altered jaw. The patient was submitted to surgery for dental extraction followed by a biopsy which showed some regressive alterations on cellular level of the bony structure