A case of neurocutaneous melanosis with systemic occupying lesion difficult to treat

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  • 全身に治療困難な占拠性病変を伴った神経皮膚黒色症の一例

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Abstract

<p> For the patient, Giant congenital melanocytic nevus（GCMN）was found in the whole body, and hemangioma and nevus sebaceous were found in head and neck region at birth. Magnetic resonance imaging（MRI）of the head was performed after hospitalization and the high signal intensity area（HSIA）which thought to be melanin deposition, was observed in the cerebellum and brainstem at T1-weighted images. As a result, she was diagnosed with neurocutaneous melanosis. Since anemia and thrombocytopenia were progressed, daily blood transfusions were required. The contrast-enhanced computed tomography（CT）scan, the contrast-enhanced MRI, and the abdominal ultrasonography（AUS）were undertaken to find the cause of pancytopenia. The results showed hepatosplenomegaly without hemangioma and the suspicion of lymphoma in multiple regions including bilateral neck region, mediastinum, retroperitoneum and mesentery. It was difficult to select the effective treatment due to the difficulty of identifying the cause, so it did not save lives. Considering autoimmune diseases, gamma globulin was given to the patient. While it did not work, steroid pulse therapy was effective temporarily. The clinical course of it will be described.</p>

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