Systemic mastocytosis is an uncommon disease. We describe a boy with congenital systemic mastocytosis with involvement of both skin and internal organs, but without any sign of bone marrow disease. Treatment with interferon alpha-2b was effective.

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A 67-year-old patient was admitted to the clinic following post-menopausal bleeding. Secondary findings consisted of COPD and non-insulin dependent diabetes mellitus type 2. On examination, the vaginal sonogram found a hyper-dense structure a few millimetres thick in the uterine cavity, with otherwise inconspicuous internal genitals (l " Fig. 1). When asked, the patient reported that she has had a "gold spiral" in her uterus since approx. 1963. In the hysteroscopy, a slightly corroded metal spiral (l " Fig. 2) could be observed in the uterus, which could be removed completely and surprisingly problem-free given its almost 50 years retention in utero through a surgical hysteroscopy. A fractionated abrasion was then performed. The histological finding resulted in little endometrium without atypia or malignancy, no mucosal necrosis or pigment depositspossibly metallosis. The spiral was identified as a Gräfenberg ring and, based on the medical history, a micro X-ray fluorescence analysis was carried out in the Rathgen research laboratory of Berlin State Museum using an ArtTAX Pro device (formerly Röntec GmbH, now Bruker). It appeared that the Gräfenberg ring examined (l " Fig. 3) consists of approx. 97 % silver and approx. 3 % copper. The alloy corresponds to Britannia silver, a British silver standard (95.84 %).