Our data support the idea that this disorder represents a reactive process. The modified term 'papular angiolymphoid hyperplasia' would define this disorder more appropriately.
We report the first case of 68-year-old Japanese woman with metastatic HER2-positive extramammary Paget's disease that showed the validity of trastuzumab monotherapy. We administered trastuzumab at a loading dose of 8 mg/kg i.v., followed by a 6 mg/kg maintenance dose every three weeks according to a protocol for HER2-positive metastatic breast cancers and a near-complete response was achieved after the tenth infusion. The patient experienced a moderate headache and flushing during the first infusion, but had no advanced effects during subsequent infusions with ibuprofen and d-chlorpheniramine maleate. Given the dramatic response, the patient has had 17 infusions of trastuzumab with no disease progression. Thus, trastuzumab has few side effects and is well tolerated for elderly patients. It may become a new choice of the adjubant therapy of this disease.
A 56 year old female developed combined hepatocellular cholangiocarcinoma associated with dermatomyositis. Serum tumour markers except for carbohydrate antigen (CA 19-9; 6400 ng/ml) were within normal range. Despite extensive chemotherapy, no clinical response was obtained and the patient's course deteriorated after 4 months. Macroscopically, the liver was mainly occupied by hepatocellular carcinoma but cholangiocarcinoma was found in the hilum. This is the first case of a rare association of combined hepatocellular-cholangiocarcinoma and dermatomyositis.
A 26-year-old woman developed 249 discrete papules on her lower extremities. Twenty-three of them had keratotic plugs. Histologic examination demonstrated granulomatous foci consisting of epithelioid cells and a epidermal invagination which was disrupted at the base and lateral sites. This invagination was considered to represent transepithelial elimination.
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