[Clinical variants of pachydermoperiostosis: complete, incomplete and atypical forms]

We describe two cases of pachydermoperiostosis, that, with the case that we have already published, allow us to present the complete clinical picture of the disease in the three forms: complete, incomplete and "forme fruste". It is interesting to note that the lack of an important symptom (digital clubbing or CVG) does not exclude the diagnosis but defines the clinical variant. The primitive hypertrophic osteoarthropathy is a common feature for the three patients, marked for I and III case, mild, but important from the diagnostic point of view, for the II case. The identification of a new marker of osteoblastic activity: osteocalcin blood level, is very important to determine the degree of activity of the disease. EM study, performed for the first time, shows an enhanced activity of the fibroblasts and a thickening of the basal membrane of the blood vessels. It is own opinion that the clinical picture observed, the vascular, the metabolic, the EM investigations give a further contribution to the understanding of this disease.