Formation of defect-fluorite structured NdNiOxHy epitaxial thin films via a soft chemical route from NdNiO3 precursors.

A new phase of oxyhydride NdNiOxHy with a defect-fluorite structure was obtained by a soft chemical reaction of NdNiO3 epitaxial thin films on a substrate of SrTiO3 (100) with CaH2. The epitaxial relationship of this phase relative to SrTiO3 could be controlled by changing the reaction temperature. At 240 °C, NdNiOxHy grew with a [001] orientation, forming a thin layer of infinite-layer NdNiO2 at the interface between the NdNiOxHy and the substrate. Meanwhile, a high-temperature reaction at 400 °C formed [110]-oriented NdNiOxHy without NdNiO2.

Epidermolysis bullosa acquisita associated with psoriasis vulgaris.

We report a case of epidermolysis bullosa acquisita (EBA) associated with psoriasis vulgaris. A 71-year-old woman with psoriasis vulgaris developed subepidermal blisters on the extremities. Direct immunofluorescence demonstrated linear deposit of IgG at the basement membrane zone, which bound to the dermal side of normal human skin split with 1 mol/L NaCl. Immunoblot analysis using recombinant full-length type VII collagen detected a 290-kDa band, confirming the diagnosis of EBA. A literature search for previous reports found a few cases of EBA associated with psoriasis, and all cases, including our own, presented with widespread inflammatory vesicles and bullae, and responded to conventional therapy with corticosteroids and immunosuppressive agents. This study suggests that western blotting using recombinant full-length type VII collagen could be useful for diagnosis of EBA, and that EBA associated with psoriasis may have a tendency to be the inflammatory type.

Mutational analysis of the ATP2A2 gene in two Darier disease families with intrafamilial variability.

BACKGROUND: Darier disease (DD), an autosomal dominant genodermatosis characterized by warty papules and plaques over seborrhoeic areas, is caused by mutations in the ATP2A2 gene, which encodes the sarco/endoplasmic reticulum Ca(2+) ATPase type 2 isoform (SERCA2). While markedly different clinical severity within DD-affected family members is known, the pathomechanism has not been elucidated. OBJECTIVES: Based on the hypothesis that multiple ATP2A2 mutations might contribute to the pathomechanism, we have analysed two DD families in which the clinical severity differs markedly within a single pedigree, and, as controls, eight DD families without differing clinical severity. METHODS: All the exons and intron-exon borders of ATP2A2 were directly sequenced from the genomic DNA extracted from all the subjects. RESULTS: We identified the heterozygous mutations, G233R in pedigree 1 and C318R in pedigree 2, respectively, whereas no other ATP2A2 mutations in any of severely affected individuals were found. In eight DD pedigrees as control, we have found M1V, N39D, L180R, A838P and 2170 insertion G in each of five pedigrees, but no mutation was found in three DD pedigrees. CONCLUSIONS: Our results together with previous data indicate that the distribution of mutations is scattered over the entire ATP2A2 without any, as yet, discernible 'hotspots'. The mutations in pedigrees 1 and 2 with intrafamiliar clinical differences occurred around the Ca(2+)-binding sites on SERCA2, which might be associated with differences in clinical severity. These variations in ATP2A2 mutations alone cannot account for the clinical heterogeneity within DD pedigrees.

Penile hybrid verrucous-squamous carcinoma associated with a superficial inguinal lymph node metastasis.

A 62-year-old Japanese man who presented with penile carcinoma is reported. The initial exophytic neoplasm excised from the coronal sulcus and prepuce on the abdominal side of the penis was diagnosed histologically as verrucous carcinoma. Twenty-six months after the primary operation, an epithelial neoplasm recurred within the scar of the primary operation. The neoplasm histologically showed verrucous carcinoma and multiple invasive foci of conventional squamous cell carcinoma in the advancing edge of the tumor, as such representing a hybrid verrucous-squamous carcinoma. A lymph node metastasis in the left superficial inguinal lymph node occurred 4 months after the second operation. A total bilateral inguinal lymphadenectomy was performed and revealed no other lymph node metastases. The patient is alive without local recurrence or evidence of metastases in pelvic lymph nodes or visceral organs 2 years after the resection of the hybrid verrucous-squamous carcinoma. The initial verrucous carcinoma, recurrent hybrid verrucous-squamous carcinoma, and metastatic lymph node were negative for human papillomavirus DNA type 6, 11, 16, 18, and 33 sequences by dot blot hybridization of polymerase chain reaction products. The characteristics of hybrid verrucous-squamous carcinoma and importance of lymph node metastasis in penile carcinoma are discussed.

Domain configurations of the "incommensurate" structures of 2H-TaSe2 and Bi2Sr2(Ca1-xLn(x))Cu2O8+delta studied by HRTEM

High-resolution transmission electron microscopy (HRTEM) images of the "incommensurate" structures of 2H-TaSe2 and Bi2Sr2Ca1-xLnx Cu2O8+delta (Ln: rare earth metal) are shown. They were taken from a wide specimen area with homogeneous thickness. For the former, a configuration of two domains was found by a scrutiny of HRTEM images. For the latter, many configurations of two domains were extracted from the photometric density distribution in the one-dimensional contrast modulations in HRTEM images. One domain of the two in both configurations is created by a phase slip occurring in the primary atomic displacement longitudinal wave.

Atomic displacements in the modulated structure of Bi2Sr2(Ca(1-x)Pr(x))Cu2O(8+delta) and their effect on HRTEM reverse contrast image

The amplitude of transverse atomic displacement wave along the direction of incident electron beam exerts a significant effect on the brightness of spots in a high-resolution transmission electron microscope (HRTEM) reverse contrast image. The bright spot is associated with the projection of the modulated atomic chain along the beam direction. In general, the spot brightness is roughly inversely proportional to the amplitude of the displacement wave in an appropriate region of defocus, specimen thickness and amplitude. Monotonic decrease in the spot brightness with increasing amplitude can be attributed to the decrease in the degree of interference between scattered electrons. A complicated domain configuration has been found by the analysis of the spot brightness modulation observed in a [0 0 1] HRTEM reverse contrast image.

Paraneoplastic syndromes of leukocytosis, thrombocytosis, and hypercalcemia associated with squamous cell carcinoma.

Paraneoplastic syndromes including leukocytosis, thrombocytosis and hypercalcemia are occasionally seen in patients suffering from progressive malignant disorders. Recent studies have revealed the production of several humoral factors by tumor cells and normal splenic cells of tumor-bearing patients to be the major cause of these reactions. Granulocyte-macrophage colony-stimulating factor (GM-CSF), granulocyte-colony stimulating factor (G-CSF), parathyroid hormone-related peptide, interleukin (IL)-1, IL-6, and tumor necrosis factor (TNF) have been implicated. We describe a 58-year-old Japanese man with squamous cell carcinoma (SCC) on the left sole, which developed in a deep linear scar after a train crash. He developed pulmonary and lymph node metastases, then leukocytosis (57,110/mm3 with 95% neutrophilia), thrombocytosis (86.3 x 10(4)/mm3), and hypercalcemia (7.0 mEq/1), and finally cachexia, followed by death. Serum G-CSF, IL-1 alpha, IL-1 beta, and TNF-beta were determined; revealing G-CSF and IL-1 beta levels were above the upper limits of their normal ranges at 39.2 pg/ml and 4.63 pg/ml, respectively. It is probable that these humoral factors were partially responsible for the paraneoplastic syndromes induced by the cutaneous SCC with metastasis in the present case.

Nasal and nasal-type T/NK-cell lymphoma with cutaneous involvement.

Natural killer (NK) cells are a third lymphocyte lineage, in addition to B- and T-cells, that mediate cytotoxicity without prior sensitization. NK cells also have phenotypic and genotypic characteristics; they express the NK-related antigen CD56 and T-cell markers such as CD2 and CD3 epsilon, but their T-cell receptor (TCR) locus is not rearranged. Non-Hodgkin's lymphomas are divided into B- and T-cell neoplasms and NK-cell lymphomas. We describe 2 Japanese patients with nasal and nasal-type T/NK-cell lymphoma in which the skin, nasal/nasopharyngeal region, bone marrow, and lymph node were the sites of involvement. The clinical and histopathologic findings were recorded. In addition, immunophenotyping, TCR gene rearrangement, and the existence of Epstein-Barr virus (EBV) DNA by polymerase chain reaction amplification were determined. Clinically, the cutaneous eruptions were purplish, hard, multiple nodules. Histologically, angiocentric proliferation of small-to medium-sized, pleomorphic, lymphoid cells were observed. They revealed hand-mirror-shaped lymphocytes with azurophilic granules with the use of Giemsa staining by touch smear. These lymphocytes were found to be positive to immunophenotyping for CD2 (Leu5b), CD3 epsilon (DAKO), CD4 (Leu3a), and CD56 (Leu 19). No clonal rearrangement of TCR-beta, -gamma, and -delta genes and immunoglobulin gene markers were found, and no positive results of identification of EBV DNA were shown. The patients underwent cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with complete remission; however, both had recurrence of disease. Because NK-cell lymphomas express some T-cell markers, they may be mistakenly diagnosed as peripheral T-cell lymphomas if they are not investigated for the NK-cell-specific marker, CD56. Therefore the importance of immunophenotypic investigations of CD56 should be stressed. Also, the importance of clinical investigation of nasal/nasopharyngeal lymphomas should be stressed when NK-cell lymphoma is diagnosed involving the skin, because NK-cell lymphomas are often associated with the nasal and nasopharyngeal region.

Primary cutaneous adenoid cystic carcinoma with lymph node metastasis.

We report on a case of primary cutaneous adenoid cystic carcinoma with local recurrence and lymph node metastasis. The patient was a 52-year-old Japanese woman. The initial cutaneous lesion was a skin-colored, centrally elevated, thumb-sized nodule with hair loss on the scalp in the right side of the parietal region. Three and half years after the initial combined treatment consisting of surgery and irradiation, a recurrent nodule on the left side of the parietal region of the scalp and a lymph node metastasis with perinodal tissue involvement in the left retroauricular area were discovered. In the literature on primary cutaneous adenoid cystic carcinoma, the average age in the 37 cases reported, including the present case, is 58.1 years, and 23 of the patients are women. Thirty-two percent of the carcinomas (12 cases) occur on the scalp and 16% (6 cases) on the breast. The average size of the tumors in the 24 documented cases is 3.2 cm. The local recurrence rate is 51% (19 cases). Distant metastasis to the lungs and pleura are recorded in three cases. Lymph node metastasis is recorded only in two cases including the present case. Multiple modalities including surgery, irradiation, and chemotherapy should be used for this locally aggressive and potentially metastasizing carcinoma.