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We report the case of a 71-year-old woman with acute hepatitis C infection and persistent viremia since 2 years. Her clinical course was characterized by general fatigue and prolonged jaundice with unusually high serum bilirubin levels. Liver histology showed lymphocyte infiltration, marked fibrosis, and severe cholestasis in the periportal zone, findings mimicking fibrosing cholestatic hepatitis (FCH). Fibrosing cholestatic hepatitis is a life-threatening form of recurrent hepatitis C infection that typically occurs in immunosuppressed patients. Here we report the rare case of an immunocompetent patient who developed this condition.

Ependymomas can relapse at the primary site and disseminate to the spinal cord. Furthermore, extraneural metastases are rare. We present a case of anaplastic ependymoma with extraneural metastasis, possibly caused by surgical seeding. An 18-year-old female was referred to us after experiencing frequent convulsive attacks. At age 9 years, she underwent partial resection of a left frontal anaplastic ependymoma, followed by 45 Gy radiation. At age 15 years, a recurrent tumor was totally removed. Three years later, magnetic resonance images revealed a left frontal recurrent tumor and a new parietal lesion. Our preoperative diagnosis of the parietal tumor was a radiation-induced meningioma. However, intraoperative pathological examinations revealed the lesion to be an anaplastic ependymoma. The tumor, which was located in the epidural space and had invaded extensively into the cranial bone, was gross totally removed. Although extremely rare, surgical seeding of anaplastic ependymomas should be considered as a differential diagnosis for recurrent tumors in the surgical field.

Biologics such as tumor necrosis factor (TNF) inhibitors are highly effective for treating severe psoriasis, and an increasing number of patients benefit from them. However, subsidiary subjects have arisen in the course of treatment which may directly impact the continued use of these drugs in a given case. In this article, we report two cases of psoriasis vulgaris refractory to treatment with both adalimumab and infliximab. We measured the serum drug concentration and absorbance value of antidrug antibodies using the sandwich enzyme-linked immunoassay method. Antibodies to adalimumab and infliximab were detected in both patients. The low drug concentration and adverse clinical response observed corroborated these findings. Dosage and concomitant medication are considered to be important factors influencing the development of antibody formation. Therapeutic drug monitoring and measurement of antidrug antibodies will assist in maintaining a clinical response and in optimizing the response of patients being treated with biological therapies. The use of concomitant immunosuppressive medication should be considered for patients who will be treated with their second TNF inhibitor after having developed antidrug antibodies against their first TNF inhibitor., (© 2013 Japanese Dermatological Association.)

We quantified the subarachnoid hemorrhage (SAH) volume in 64 patients on three-dimensional computed tomography (3D-CT) scans and studied the correlation between the SAH volume and the occurrence of symptomatic vasospasm (SVS). We studied 64 patients with SAH onset (day 0) and on days 1, 4, 7, and 14. We compared the hematoma volume by 3D-CT with 2D-CT on day 0 and examined the correlation between the hematoma volume and the occurrence of SVS. The hematoma volume, including the volume of normal structures, was automatically calculated (V1). The volume of normal structures manifesting identical CT numbers was previously calculated in patients without intracranial lesions (V2). The total hematoma volume was defined as V1 minus mean value of V2 (= 12 ml). The mean hematoma volume by 3D-CT was 48 ± 12 ml and by 2D-CT was 31 ± 45 ml (mean ± SD, n = 64). The hematoma volume was significantly larger by 3D-CT than by 2D-CT (p < 0.05). At all time points, the hematoma volumes were significantly larger in patients with than without SVS. We developed a new method for the quantitative determination of the SAH volume by 3D-CT. This method may allow us to quantify the volume of SAH in clinical studies of cerebral vasospasm.

A 37-year-old male presented with transorbitally penetrating bamboo fragments, which resulted in multiple brain abscesses during 30 years. Neurological examination revealed left blindness, oculomotor palsy, trigeminal nerve (V1) anesthesia, and cerebellar ataxia. Computed tomography demonstrated foreign bodies and two ring enhanced lesions in the cerebellum. Magnetic resonance imaging demonstrated a hypointense mass penetrating through the cavernous sinus into the posterior fossa, and another mass into the temporal lobe. Cerebral angiography showed that the sylvian vein drained to the pterygoid plexus. Two weeks after the drainage of two cerebellar abscesses using an endoscope, bamboo fragments were removed using a zygomatic anterior petrosal approach. Occlusion of the draining vein to the pterygoid plexus might cause temporal venous infarction. Using indocyanine green intraoperative angiography, we confirmed the collateral venous flow of the sylvian vein after temporary occlusion of the draining vein to the pterygoid plexus. Postoperative course was uneventful. Foreign bodies must be removed even after 30 years to prevent repetitive brain abscesses.

Nephrogenic systemic fibrosis is a fibrosing disorder that affects patients with advanced renal dysfunction and is associated with gadolinium-based contrast media. As the number of reports increase, it is becoming clear that its clinical course and symptoms are various. We describe a 14-year-old boy and 71-year-old man with nephrogenic systemic fibrosis and review the Japanese cases documented thus far. In Japan, there are only 10 cases definitely associated with gadolinium, whereas over 500 cases have been recorded worldwide. We found a remarkable difference in clinical signs among Japanese cases. Some cases showed keratotic papules or plaques on the extremities. This group tended to develop symptoms after a shorter interval following gadolinium exposure. The remainder of the cases presented glossy and smooth surfaces, with symptoms tending to develop after a longer interval following their last gadolinium exposure. The discrepancies between the actual and the estimated number of patients, and the various clinical manifestations can be explained by the comparatively smaller dosage of gadolinium-based contrast agents commonly administrated in Japan, in contrast to the higher dosages administrated in the USA and Europe., (© 2011 Japanese Dermatological Association.)

Patients with metastatic spinal tumor are the largest in number among the patients with bone tumor. It causes a severe bone pain, pathological fracture and spinal cord compression. Thus it harshly hampers patient's quality of life. We report 3 patients with lung cancer whose initial manifestation was metastatic spinal tumor. We treated the 3 patients with palliative radiotherapy and medication. Although the severe pain has improved on a numerical rating scale(NRS), but performance status(PS)and activity of daily living(ADL)of the 3 patients got worse because the disease was progressed and complicated. Generally, PS of cancer patients found by bone matastasis is low. However, it is difficult to take an effective treatment, which leads to ADL improvement. There are many choices for treating metastatic bone tumors including pain control, bisphosphonate administration, radiation therapy, strontium radiotherapy, bone cement, palliative surgery and orthotics. In addition, a development of molecular target drugs, such as Denosmab, is expected as future modality of palliative care. In conclusion, we should detect a bone metastasis in the patient with lung cancer as early as possible, and select an appropriate treatment in collaboration with each specialist for achieving the ADL and PS improvement.

We report a case of cutaneous infection due to Scedosporium apiospermum in a 75-year-old immunocompromised male patient who had received long-term corticosteroid and immunosuppressant therapy for the treatment of nephrotic syndrome. The patient came to our department complaining of erythema with a number of pustules on the dorsal surface of the right hand. S. apiospermum was identified from a culture taken from the pus. After unsuccessful treatment with topical ketoconazole, oral itraconazole and oral terbinafine, the lesion quickly resolved with the daily administration of 400 mg voriconazole. No recurrence was observed despite discontinuation of voriconazole due to drug-induced hepatitis. Voriconazole holds out the promise of an effective treatment for invasive Scedosporium infection.

A 73-year-old man presented with a two year history of multiple nodules and follicular papules accompanied by slight itching on the face and the forearm. A physical examination showed multiple, soft, erythematous nodules on the forehead, cheek, and jaw, contributing to a generally leonine appearance of the face. Histopathological examination from the forehead revealed dense, massive concentrations of atypical lymphocytes in the dermis, and the forearm showed infiltration of atypical lymphocytes predominantly around the follicles. We diagnosed this condition as folliculotropic cutaneous T cell lymphoma (CTCL). EPOCH therapy was very effective and the lesions of the forehead and forearm showed a decrease in tumor elevation; the histology showed a precipitous decrease in the number of the atypical lymphocytes.

We report a case of acute generalized exanthematous pustulosis (AGEP) induced by ampicillin/cloxacillin sodium (ABPC/MCIPC) in a pregnant woman. AGEP is caused mostly by drugs. Among them, beta-lactam antibiotics account for a high proportion of the cases, predominantly by amoxicillin. To our knowledge, this is only the second case ever reported in the Japanese language published work of AGEP induced by ABPC/MCIPC.

Stimulation of L929 cells with tumor necrosis factor-alpha (TNFalpha) caused cell death accompanied by a release of arachidonic acid (AA). Although the inhibition of caspases has been shown to cause necrosis in TNFalpha-treated L929 cells, its role in the TNFalpha-induced release of AA has not been elucidated. The release of AA is tightly regulated by phospholipase A(2) (PLA(2)). To find out the mechanisms underlying the TNFalpha-induced release of AA, we investigated the relationship between TNFalpha stimulation and PLA(2) regulation with and without zVAD, an inhibitor of caspases. In the present study, we found that treatment with TNFalpha and zVAD stimulated release of AA and cell death in C12 cells (a variant of L929 cells lacking alpha type of cytosolic PLA(2) (cPLA(2)alpha)). Stimulation with TNFalpha/zVAD also caused the release of AA from L929-cPLA(2)alpha-siRNA cells. Treatment with pyrrophenone (a selective inhibitor of cPLA(2)alpha) completely inhibited the TNFalpha-induced release of AA, but only partially inhibited the TNFalpha/zVAD-induced response in L929 cells. The TNFalpha/zVAD-induced release of AA from C12 and L929-cPLA(2)alpha-siRNA cells was pyrrophenone-insensitive, but inhibited by treatment with butylated hydroxyanisole (BHA, an antioxidant). Treatment with dithiothreitol, which inactivates secretory PLA(2) activity, decreased the amount of AA released by TNFalpha/zVAD. TNFalpha/zVAD appears to stimulate release of AA from C12 cells in a cPLA(2)alpha-independent, BHA-sensitive manner. The possible roles of secretory PLA(2) and reactive oxygen species from different pools in the release of AA and cell death were discussed.

Lichen sclerosus is a chronic inflammatory skin disorder that has a predilection for the anogenital area. Topical corticosteroid is occasionally effective; however, continuous treatment is often required and recurrence after its stoppage is frequent. Herein, we report a case of vulvar lichen sclerosus in a 5-year-old girl, which was refractory to topical corticosteroids. After 14 weeks of treatment with 0.03% tacrolimus ointment once daily, the lesions completely resolved without side-effects. Of interest, the number of milia within the plaque of lichen sclerosus was reduced in tandem with the improvement of lichen sclerosus. This is the first report of topical low-concentration tacrolimus treatment showing a dramatic effect in the treatment of childhood vulvar lichen sclerosus.