Eosinophilic annular erythema is clinically characterized by central pigmentation reflecting basal melanosis: a clinicopathological study of 10 cases.

BACKGROUND: Eosinophilic annular erythema (EAE) has been proposed as a clinical entity to describe annular skin lesions associated with tissue eosinophilia. However, systematic investigations on the histopathology of EAE have not been performed, and useful histopathological findings for diagnosis of EAE remain unknown. OBJECTIVE: The aim of this study was to investigate the clinicopathological features of EAE. METHODS: We retrospectively studied 10 patients at our hospital during a 5-year span who clinically showed annular or figurate lesions and histopathologically exhibited eosinophilic infiltration in the dermis. RESULTS: Nine of the 10 cases had annular lesions with pigmentation on the interior side. Blood eosinophilia was found in only one patient. Histopathologically, basal melanosis was observed in nine cases. Infiltration of eosinophils was confined to the dermis in nine cases. Patients treated with systemic corticosteroid tended to show less recurrence than those treated with topical corticosteroid. LIMITATIONS: The main limitation of our study is the small number of patients. CONCLUSION: Skin biopsy should be performed when EAE is suspected, even in cases without blood eosinophilia. Basal melanosis and tissue eosinophilia confined to the dermis suggest the diagnosis of EAE. We recommend topical corticosteroids as the initial treatment for EAE.

A notable case report of May-Hegglin anomaly with immune complex-related nephropathy: a genetic and histological analysis.

May-Hegglin anomaly (MHA) is a rare autosomal dominant disease characterized by macrothrombocytopenia and leukocyte inclusions with microfilaments in the ribosomes. Mutations in the MYH9 gene, encoding non-muscle myosin heavy chain IIA (NMMHC-IIA) have been identified in patients with MHA and other MYH9-related diseases. Two young males (an older and younger brother) presented with macrothrombocytopenia and leukocyte inclusion bodies. Electron microscopy (EM) revealed parallel filaments in leukocyte inclusion bodies characteristic of MHA. Immunofluorescence microscopy (IF) showed NMMHC-IIA antibodies in 1 - 2 leukocyte inclusion bodies. These findings were consistent with MHA and they were identified to express the MYH9 mutation, D1424H. The older brother underwent a renal biopsy because of persistent proteinuria. Histology revealed mesangial proliferative glomerulonephritis with granular deposits of IgG and C1q. EM showed that the dense deposits were located in subendothelial cells, mesangial cells and Bowman's capsule. Immunocytochemistry revealed that NMMHC-IIA antibodies were localized in podocyte and endothelial cells in the glomerulus. Moreover, the expression of nephrin and podocin, slit diagram protein, was normal. An inflammatory mechanism may occur separately from MYH9-related disease. This report presents a case of MHA with immune complex-related nephropathy.

Gene structure of rat testicular cell adhesion molecule 1 (TCAM-1), and its physical linkage to genes coding for the growth hormone and BAF60b, a component of SWI/SNF complexes.

In the +9.7 to +21.7kb downstream region from the transcriptional start site of the rat growth hormone (GH) gene, a gene specifically expressed in the testis was found to have reverse transcriptional orientation to the GH gene. Its exon comprised 2693 bases encoded a protein having 548 amino acids (60479Da). The amino acid sequence of the testis-specific protein resembled that of the intercellular adhesion molecules (ICAM) 1 and 3. The gene was thus given the name testicular adhesion molecule (TCAM) 1 gene. The TCAM-1 gene was found to be 12041 bases with eight exons. Although exon 1 was noncoding, the remaining seven exons corresponded to the domains coding for the signal sequence, five immunoglobulin (Ig) domains, and the transmembrane plus cytoplasmic domain. The organization of TCAM-1 gene was shown to be the same as that of the ICAM-1 gene. The polyadenylation site of TCAM-1 gene was located 7.6kb downstream of that of the GH gene, whereas the 5' end of TCAM-1 gene was separated 5.9kb from that of the gene coding for BAF60b, a component of SWI/SNF complexes known as the chromatin remodeling factor. Six genes were thus mapped in the following order in the 88kb region of the rat GH locus: Na-channel (5' to 3')-B29/Ig-beta (5' to 3')-GH (5' to 3')-TCAM-1 (3' to 5')-BAF60b (5' to 3')-SUG/p45 (3' to 5').

Physical linkage of the B29/Ig-beta (CD79B) gene to the skeletal muscle, sodium-channel, and growth hormone genes in rat and human.

In the region between the polyadenylation site of the rat skeletal muscle (SkM) Na-channel gene and the 5' end of the growth hormone (GH) gene, a gene coding for B-cell-specific membrane protein B29/Ig-beta was found and noted to have the same orientation as the Na-channel and GH genes. Rat B29/Ig-beta gene was 3.1 kb in length with six exons and was separated by 3.3 and 9.3 kb from Na-channel and GH genes, respectively. Rat B29/Ig-beta protein comprised 228 amino acids, and its amino acid sequence was 85 and 69% identical with the mouse and human counterparts, respectively. With the long-area PCR method, genomic DNA connecting human SkM Na-channel (SCN4A) and B29/Ig-beta (CD79B) genes and CD79B and GH (GH1) genes was amplified, and the physical linkage of SCN4A/CD79B/ GH1 genes in the human genome was established. The human CD79B gene was separated by 6.3 and 10.5 kb from the SCN4A and GH1 genes, respectively.

Gene coding for the transcription factor, SUG/proteasome, p45 is located nearly 40 kb downstream from the rat growth hormone gene.

About 40 kilobases (kb) downstream of the rat growth hormone gene, a gene was found to be expressed in the liver and placenta as 1.5 kb poly(A)-rich RNA. Using the genomic DNA fragment as a probe, the corresponding cDNA clone containing a 1.3 kb insert was isolated from the rat liver cDNA library. The deduced amino acid sequence having 406 residues was identical with that of the mouse transcription factor, SUG and human proteasome subunit, p45. The gene was thus identified as the rat SUG/p45 (rSUG/p45) gene. The 5' end of the gene was determined by the primer-extension analysis and the exon was noted to comprise 1409 bases. The rSUG/p45 gene, 6.0 kb in length and possessing 12 exons, started at 42.8 and ended at 36.8 kb downstream from the transcription start site of the GH gene. From exon 2 to 11, the size of each rSUG/p45 exon was identical with the corresponding exon of the 4.4 kb pig gene. Rat SUG/p45 mRNA was similarly expressed in seven different tissues and one cell line.

Refractory anaemia with ringed sideroblasts concurrent with multiple myeloma--a brief review of the recent literature.

The report describes a patient in whom myelodysplastic syndrome and multiple myeloma (MM) were simultaneously present. This patient manifested an IgA-lambda type of MM concurrent with a refractory anaemia with ringed sideroblasts (RARS) without prior therapy. His bicytopenia could not be improved by vitamin B6 regardless of a reduced serum vitamin B6 concentration. A review of the literature suggests that myelodysplastic syndrome (MDS), chronic neutrophilic leukaemia (CNL) and idiopathic myelofibrosis (IMF) are the most frequent disorders associated with MM. The IgA type seems to be associated more commonly with these disorders. The mechanisms responsible for the development of plasma cell proliferation are diverse; the neoplastic transformation of a common progenitor, the involvement of the lymphoplasmacytic system and/or chronic reticuloendothelial stimulation may play a role in the occurrence of such hybrid haematological disorders.

Gene structure of rat BAF60b, a component of mammalian SW1/SNF complexes, and its physical linkage to the growth hormone gene and transcription factor SUG/proteasome p45 gene.

In the +27.6 to +36.7 kb downstream region from the transcriptional start site of the rat growth hormone (GH) gene, a gene encoding BAF60b, a component of mammalian SWI/SNF complexes, was found to have the same transcriptional orientation as the GH gene. The 5' end of the BAF60b gene was heterogeneous and the longest gene was 9060 bp long with 13 exons. The largest of all exons was estimated to be 2774 bases. Deduced rat BAF60b protein was made of 531 amino acids and its amino acid sequence was 97% identical with the human counterpart. No TATA box was found up to the -100 bp region but five GC boxes corresponding to the Sp1 binding site were observed up to 640 bp upstream from the transcriptional start site. Sixty-three bases downstream from the BAF60b gene, the polyadenylation site of the gene encoding transcription factor SUG/proteasome p45, whose expression is constant in many tissues, was identified. The BAF60b gene was expressed as 3.0 kb poly(A)-rich RNA in seven tissues and one cell line from rat but its expression varied considerably according to the tissue.

Differences in immune functions between human T-lymphotropic virus type I carriers and patients with adult T-cell leukemia/lymphoma.

A variety of immunologic tests were compared between human T-lymphotropic virus type I (HTLV-I) carriers and patients with adult T-cell leukemia/lymphoma (ATL). The mitogenic responses of the lymphocytes to concanavalin A and phytohemagglutinin were depressed in the ATL patients but not in the carriers, while the response to pokeweed mitogen in the carriers and ATL patients was depressed compared to the HTLV-I-seronegative controls. A positive tuberculin reaction in the carriers was as frequent as in the controls, but less frequent in the ATL patients. A marked inhibition of the intracellular multiplication of Legionella pneumophila was observed within the monocytes isolated from the carriers, but not in the ATL patients or the normal controls. These results indicate that the ATL patients have severe immunodeficiency, while the HTLV-I carriers have some activation of anti-microbial activity in monocytes although they are somewhat immunosuppressed.

Emission of extremely strong magnetic fields from the head and whole body during oriental breathing exercises.

This article reports the result of an experiment that was designed to measure the biomagnetic field emanating from two individuals who were practising traditional Oriental Qi Gong breathing exercises. The biomagnetic field was measured with differential coils wound 80,000 turns, a magnetic needle compass and a digital electromagnetic wave detection device. It was found that an extremely strong magnetic field was emitted from the two individuals. One subject emitted a magnetic field at the level of 200-300 mT (2-3 mGauss) and the other at 0.13 mT (1.3 mGauss). In both cases, moreover, the magnetic needle compass rotated 30 degrees (this was tested 32 times). When the rotation of the needle occurred, a reproducible magnetic field of 800-1500 mT (8-15 mGauss) was indicated on the digital measuring device (this was tested 12 times). It is concluded that traditional Oriental Qi Gong breathing appears to stimulate an unusually large biomagnetic field emission.

[Factors related to ADL of stroke patients three months after discharge].

Focusing on the ADL of stroke patients, 94 hemiplegia cases in rehabilitation units of acute care general hospitals were studied to identify factors affecting ADL, measured using the Barthel Index, 3 months after discharge. The main results were as follows: 1. The 8 independent variables: age, gender, length of hospitalization, degree of paralysis of lower extremities at discharge, motivation toward rehabilitation, sight, Barthel scores at discharge, and the expression of one's intentions emerged as the 8 reliable predictive factors (R = .904, R2 = .817). 2. It may be deduced that in older patients in particular, a status of amyotrophy has a significant influence on ADL after discharge. The 94 stroke patients were divided into two age groups-44 patients aged 64 years or younger, and 50 patients aged 65 years or older. Barthel scores at 3 months after discharge were analyzed by multiple regression analysis in both groups. The result was that ADL, after discharge, of the aged 65 years or older group, was more affected by their status at discharge, than was it with the 64 years or younger group (64 years or younger; R = .871, R2 = .758, 65 years or older; R = .934, R2 = .873). 3. For patients in the 64 years or younger group, Barthel scores at 3 months after discharge were significantly related to age, length of hospitalization, sight, Barthel scores at discharge and expression of one's intentions. For patients in the 65 years or older group, the results were significantly related to sight, Barthel scores at discharge, and motivation for rehabilitation.

Misexpression of Hoxa-13 induces cartilage homeotic transformation and changes cell adhesiveness in chick limb buds.

During chick limb development, the Abd-B subfamily of genes in the HoxA cluster are expressed in a region-specific manner along the proximodistal axis. To elucidate the function of Hoxa-13 that is expressed in the autopod during normal limb development, Hoxa-13 was misexpressed in the entire limb bud with a replication-competent retroviral system. Misexpression of Hoxa-13 resulted in a remarkable size reduction of the zeugopodal cartilages as a result of the arrest of cartilage cell growth and differentiation restricted in the zeugopod. This size reduction seems to be attributable to homeotic transformation of the cartilages in the zeugopod to the more distal cartilage, that of the carpus/tarsus. This transformation was specific to Hoxa-13 and was not observed by overexpression of other Hox genes. These results indicate that Hoxa-13 is responsible for switching the genetic code from long bone formation to short bone formation during normal development. When the limb mesenchymal cells were dissociated and cultured in vitro, Hoxa-13-expressing limb mesenchymal cells reassociated and were sorted out from nonexpressing cells. Forced expression of Hoxa-13 at the stage that endogenous Hoxa-13 was not expressed as of yet altered the homophilic cell adhesive property. These findings indicate the involvement of Hoxa-13 in determining homophilic cell-to-cell adhesiveness that is supposed to be crucial for the cartilage pattern formation.

[Immunoblastic lymphadenopathy (IBL)-like T cell lymphoma associated with granulocytopenia and autoimmune hemolytic anemia].

A 65 year-old woman was admitted in May 1990, because of fever, generalized lymphadenopathy and eruption. Laboratory examination showed granulocytopenia, polyclonal hypergammaglobulinemia, positive Coombs' test, positive cold antibody and positive anti-E erythrocyte antibody. 7 days after admission autoimmune hemolytic anemia developed. The appearance of the anti neutrophil leukocyte antibody was suspected because of the absence of the segmented neutrophils in marrow specimen. Histological findings of the biopsied lymph node and the marker study of the tumor cell disclosed IBL-like T cell lymphoma. The patient was treated with G-CSF and VEPA chemotherapy. Granulocytes increased and she showed marked symptomatic improvement with complete remission. Our case showed various clinical pictures with hemolytic anemia and granulocytopenia, which could be based on the autoimmune mechanisms.

Development of continuous recirculating peritoneal dialysis using a double lumen catheter.

Continuous recirculating peritoneal dialysis (CRPD) was newly introduced to improve solute removal efficiency in conventional dialysis therapies such as hemodialysis (HD) and continuous ambulatory peritoneal dialysis (CAPD). In CRPD, a part of the dialysate in the peritoneal cavity was drained through a double-lumen catheter and purified by an extracorporeal dialyzer. Urea removal characteristics in CRPD were examined in a canine study. In this study, a recirculation-dialysis experiment using a dog weighing 9.0 kg was carried out under 100 and 200 ml/min of flow for recirculating and delivered dialysates, respectively. An FB-50H (Nipro Medical Industries, Ltd., Osaka, Japan) composed of cellulose diacetate membrane with 0.5 m2 of surface area and Dianeal-1.5 (Baxter Limited Laboratories, Tokyo, Japan) containing urea were used as the extracorporeal dialyzer and dialysate. Urea peritoneal and dialyzer dialysances (DBP and DBD) were 3.05 and 33.3 ml/min by computer simulation using a compartment model for CRPD. This DBP value can be estimated as 20.3 ml/min for a 60 kg human. From this result, time-averaged value for BUN over an 8 hr/day CRPD, combined with three exchanges/day as CAPD is estimated to be 34.3 mg/dl, which is much lower than 45.2 mg/dl for a 12 hr/week HD, or 53.0 mg/dl for conventional CAPD.