PMC3147132_01
Photograph of the skin of the forehead (a) and pathological finding of the skin biopsy (b). Noncaseating granuloma composed of epithelioid cells and multinucleated giant cells were present.
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Axillar and pubic hair were lost and there was palpable erythematous rush on his forehead (Figure 1(a)). A biopsy from the skin of the forehead was performed, which revealed noncaseating granuloma composed of epithelioid cells (Figure 1(b)).
Hypertonic saline test before (a) and after (b) glucocorticoid therapy. Before therapy, although plasma AVP was undetectable, urine became concentrated. After therapy, the hypertonic saline test failed to concentrate the urine and AVP was undetectable.
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Although plasma AVP was undetectable before and after administration of hypertonic saline, urine osmolality (391 mOsm/kgH2O) increased above the plasma levels (296 mOsm/kgH2O) (Figure 2(a)). Soon after the replacement therapy was begun, moderate polyuria (3.5-4.0 liters daily) occurred (Figure 2(b)).
Hormonal provocative test results.
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Hormonal provocative tests revealed that growth hormone (GH) response to GH-releasing hormone was normal, TSH response to TSH-releasing hormone was low and blunted, and FSH and LH response to LH-releasing hormone were low and blunted (Figure 3).
T1-weighted MRI of the sella turnica after administration of gadolinium revealed enhancing lesion centrally in the pituitary stalk, which spread continuously to the bottom of the third ventricle, and high intensity of posterior lobe was lost.
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CDI (Figure 4). Considering the patient's age, we first suspected of neurohypophysial germinoma.
Clinical course.
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Plasma AVP was undetectable during the hypertonic saline test and urine osmolality was consistently below plasma levels, which was compatible with a diagnosis of CDI (Figure 5).