PROGRESSIVE DIAPHYSEAL DYSPLASIA (CAMURATI-ENGELMANNS DISEASE) - IMPROVEMENT OF CLINICAL SIGNS AND OF BONE-SCINTIGRAPHY DURING PREGNANCY

The case of a woman suffering from progressive diaphyseal dysplasia is presented. Characteristic symptoms of crippling pain in both legs, se vere aching in both forearms, and episodic temporofrontal and occipita l headache were only partially regulated by corticosteroid treatment. However, pregnancy resulted in a progressive disappearance of these sy mptoms, allowing withdrawal of steroid treatment. Tc-99m MDP scintigra phy performed immediately after delivery showed a decrease of the inte nse uptake in the forearms, tibiae, and skull, which had been document ed prior to pregnancy. However, widespread pain recurred within 6 week s after delivery, accompanied by a recurrence of multiple severely hyp eractive foci on bone scintigraphy. Alterations of immune modulated pr ocesses and changes in bone mineral homeostasis and in endogenous cort isol metabolism during pregnancy can be considered as possible explana tions for the temporary improvement in clinical and scintigraphic sign s of progressive diaphyseal dysplasia in this patient.