Perforating pilomatricoma with anetodermic epidermis in an adolescent with lymphoma.

Pilomatricoma is a benign neoplasm originating from primitive cells of the hair matrix. Occasionally the skin overlying pilomatricomas assumes peculiar clinical features, and anetodermic change of the skin overlying pilomatricomas is sometimes seen, but perforation in pilomatricoma is a rare event. We report a case of perforating pilomatricoma with anetoderma in an adolescent with lymphoma.

Eruptive molluscum contagiosums in a patient with rheumatoid arthritis and lung cancer.

A 67-year-old woman with rheumatoid arthritis (RA) treated with systemic prednisolone and methotrexate over 20 years developed eruptive molluscum contagiosums on the trunk and extremities. Investigation revealed lung cancer 2 years later. Newly development of molluscum contagiosums ceased after the surgical operation of lung cancer. Immunologic dysfunctions have been shown in RA, and especially patients under long-term methotrexate therapy are susceptible to miscellaneous skin conditions. Eruptive molluscum contagiosums are induced in association with hematologic malignancies such as lymphoma, leukemia, and HIV infection; however, it is important to investigate internal malignancies, not only hematologic malignancies but also solid cancers, when patients with RA under immunosuppressive therapies presented eruptive or disseminated molluscum contagiosums.

Construction and commissioning of mid-infrared self-amplified spontaneous emission free-electron laser at compact energy recovery linac.

The mid-infrared range is an important spectrum range where materials exhibit a characteristic response corresponding to their molecular structure. A free-electron laser (FEL) is a promising candidate for a high-power light source with wavelength tunability to investigate the nonlinear response of materials. Although the self-amplification spontaneous emission (SASE) scheme is not usually adopted in the mid-infrared wavelength range, it may have advantages such as layout simplicity, the possibility of producing a single pulse, and scalability to a short-wavelength facility. To demonstrate the operation of a mid-infrared SASE FEL system in an energy recovery linac (ERL) layout, we constructed an SASE FEL setup in cERL, a test facility of the superconducting linac with the ERL configuration. Despite the adverse circumstance of space charge effects due to the given boundary condition of the facility, we successfully established the beam condition at the undulators and observed FEL emission at a wavelength of 20 µm. The results show that the layout of cERL has the potential for serving as a mid-infrared light source.

Efficacy of tocilizumab and evaluation of clinical remission as determined by CDAI and MMP-3 level.

Tocilizumab, a biological agent developed in Japan, is a human anti-interleukin-6 (anti-IL-6) receptor antibody. Rheumatoid arthritis improves with its use. A remission rate of 59% is attainable, as measured by disease activity score 28 (DAS28) in the SAMURAI study. However, in tocilizumab treatment, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) levels drop to negative values; therefore we sought to utilize a different index for measuring its efficacy. In order to evaluate the effects of tocilizumab we carried out this study using clinical disease activity index (CDAI), as it is not reliant on blood data and would also allow us to determine which markers are present in remission. Twenty-two patients under treatment with tocilizumab participated in this study. Effects of treatment as well as the remission rate were measured by CDAI and DAS28 3 months after initiation of treatment. IL-6 and matrix metalloproteinase-3 (MMP-3) levels were measured at the same time. We studied the clinical efficacy of tocilizumab using DAS28 after treatment; remission as measured by DAS28 was 57.1% at 1 year. However, the remission rate as measured by CDAI was only 19.1% at 1 year. CDAI was not only correlated with DAS28, but also other clinical variables, MMP-3, and IL-6. We conclude that CDAI is effective in measuring clinical response to tocilizumab treatment, and that MMP-3 level is as useful as IL-6 level as an indicator.

[Intra-arterial infusion chemotherapy for advanced breast cancer--5 cases of marked response].

In order to obtain less severe toxic reactions, while attaining maximal therapeutic effects, CPA, 5-FU and ADM or EPI have been administered through the catheter inserted into the internal thoracic artery with the IVR technique and the implantable infusion port system. Of the 181 patients with the unresectable or recurrent breast cancers attempted with intra- arterial infusion chemotherapy for the past 19 years, more than half of the patients showed a good response to this modality of the chemotherapy. In 4 out of the 5 patients with unresectable breast cancer, radical mastectomy became feasible following the marked response with the intra-arterial infusion chemotherapy. However, a distant metastasis proved to be a limiting factor for improvement of survival.

[Switching to direct intrahepatic arterial infusion of 5-FU after a progression of liver metastases from colorectal carcinoma treated with systemic infusion of FOLFOX].

Direct intrahepatic arterial infusion of 5-FU produced a significantly higher response rate than systemic infusion of FOLFOX in the treatment of hepatic metastases from colorectal carcinoma. Fourteen patients switched over from systemic FOLFOX therapy to intrahepatic protracted 5-FU infusion after a progression of liver metastases treated with systemic therapy. Of the 14 patients whose tumors had initially failed to respond to systemic FOLFOX therapy, 12 (85%) had a partial response, and 13 (93%) had a reduction in their tumor marker (CEA, CA19-9, TPA) when the treatment was switched to intrahepatic 5-FU therapy. Traditional chemotherapy toxicity, such as myelosuppression, nausea, vomiting and neurotoxicity did not occur in the intrahepatic group. Three out of 14 patients survived more than a year, and the longest was 18 months. A better survival rate can be achieved with the use of hepatic artery infusion therapy.

Clinical characteristics, differential diagnosis, and treatment outcome of subcutaneous panniculitis-like T-cell lymphoma: a literature review of published Japanese cases.

BACKGROUND: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of non-Hodgkin lymphoma that shows phenotypic features of cytotoxic T cells and preferentially involves subcutaneous tissue. SPTCL is believed to show an indolent clinical course, unless patients develop haemophagocytic syndrome. Various reported therapies include corticosteroids, immunosuppressive drugs, and chemotherapies. The use of chemotherapy as a first-line treatment remains controversial, and treatment approaches for SPTCL have not been established yet. OBJECTIVES: To investigate the clinicopathological features, treatment modalities, and outcomes of Japanese patients with SPTCL. MATERIALS & METHODS: We performed a literature review of Japanese cases of SPTCL. RESULTS: Twenty-two cases have been reported in the English and Japanese literature. Six cases were excluded due to a lack of sufficient clinical and immunohistological data, and treatment modalities and outcome were available in 16 cases. Clinical characteristics of the Japanese cases were generally similar to those of Western countries. Approximately half of the Japanese patients were treated initially with corticosteroids. Among them, more than half subsequently received chemotherapies due to a lack of response or recurrence of the disease. Overall, chemotherapy was used for approximately 80% of reported cases as a primary or secondary therapy. CONCLUSION: Our findings suggest that corticosteroids may be beneficial for some SPTCL patients, but most patients required chemotherapy during the course of their disease. Further investigations are needed to both establish appropriate treatment strategies for SPTCL and clarify predictive factors in order to identify patients who may benefit from corticosteroid therapy as a primary treatment.

Ichthyosiform sarcoidosis: report of three cases from Japan and literature review.

Cutaneous lesions of sarcoidosis present with various manifestations including specific and non-specific cutaneous lesions. Ichthyosiform sarcoidosis is a rare form of cutaneous sarcoidosis, presenting with asymptomatic, adherent, polygonal scales, mainly appearing on the lower limbs.  Ichthyosiform sarcoidosis has a predilection for dark-skinned races, and cases affecting Japanese patients have rarely been reported in English literature.  We herein describe three Japanese cases of ichthyosiform sarcoidosis on the lower limbs. All of the patients were female, with an age range of 57-69 years old.  Histologically, sarcoidal granulomas were located in the mid- to lower dermis.  All cases had scar sarcoidosis on the knees.  Furthermore, Case 1 presented with papular sarcoidosis on the back, and Case 3 presented with subcutaneous nodules on the buttock as well as erythema nodosum-like lesions on the lower legs.  All patients had lung sarcoidosis, but ocular sarcoidosis was seen in only Case 2. Case 3 showed Heerfordt syndrome with facial nerve paralysis. Histological features showed that the granular layers were scarcely detected in the overlying epidermis; however, filaggrin expression was not decreased.  Sarcoidal granulomas accumulated around the sweat glands in one case, whereas those features were not detected in the other two cases. In conclusion, ichthyosiform cutaneous sarcoidosis may be overlooked or misdiagnosed as xerotic dry skin which is frequently found in elderly people, and ichthyosiform cutaneous lesions may be more prevalent than previously estimated.