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Background The long-term prognosis of Henoch-Schönlein purpura ( HSP) is determined by the severity of renal involvement, known as HSP nephritis, which varies considerably from patient to patient. There is now increasing evidence that dysregulated cytokine production plays a crucial role in human autoimmune and inflammatory processes. Aim To explore the possible contributions of serum antistreptolysin O, C-reactive protein, Ig A, interleukin ( IL)-6, IL-8, tumour necrosis factor ( TNF)-α, anticardiolipin antibody (a CL) and antiphosphatidylserine-prothrombin complex antibody (anti- PSPT) in the pathogenesis of HSP, and to evaluate correlations between those biological parameters and the clinical features. Methods Records were reviewed of 58 patients with HSP who presented initially with palpable purpura between 2003 and 2009. Serum IL-6 levels were determined by chemiluminescent enzyme immunoassay, IL-8 levels by ELISA and TNF-α levels by quantitative sandwich enzyme immunoassay. Serum a CL and anti- PSPT levels were measured according to our previously published procedures. Results There was a significant correlation between the serum IL-6 and Ig A anti- PSPT levels, and also between the serum IL-8 and Ig A anti- PSPT levels. Serum IL-8 and Ig A a CL levels were both significantly higher in patients with renal involvement than in those without. Serum IL-6 and IgM anti- PSPT levels were also significantly higher in patients with gastrointestinal symptoms than in those without. Conclusions We suggest that serum IL-6 and IL-8 associated with antiphospholipid antibodies play a pivotal role in the induction of HSP. Based on our results, IL-8 and Ig A a CL levels could be useful as markers to monitor the development of HSP nephritis, and IL-6 and Ig M anti- PSPT levels could be used as markers to monitor the development of gastrointestinal involvement. [ABSTRACT FROM AUTHOR]

A 79-year-old Japanese woman presented with severe recalcitrant erosions on her oral mucosa, resembling paraneoplastic pemphigus. Using indirect immunofluorescence, we detected Ig A antibodies against the cell surface, and both Ig G and Ig A antibodies against the basement membrane zone. Immunoblotting showed that the Ig G antibodies reacted weakly with bullous pemphigoid 230 and periplakin, whereas the Ig A antibodies did not react with any antigen. Ig A antibodies to both desmoglein (Dsg)1 and Dsg3 were detected by ELISA. Ig A antibodies to desmocollin (Dsc)3 were also detected by using cDNAs for human Dsc1-3 transfected into COS-7 cells. Despite treatment with oral prednisolone, high-dose intravenous immunoglobulin and double-filtration plasmapheresis, the skin lesions remained active, and the patient died from bronchiolitis obliterans-like respiratory failure. Despite extensive investigations and postmortem examination, no underlying neoplasms were found. The complex immunopathological findings probably played an important role in the development of the patient's unusual clinical features. [ABSTRACT FROM AUTHOR]

Background Henoch-Schönlein purpura (HSP) is characterized by IgA-containing immune complexes within leucocytoclastic vasculitis. Lysosomal-associated membrane protein-2 (LAMP-2) was first identified as part of a systematic search for antineutrophil cytoplasmic antibody (ANCA) antigens expressed on neutrophils and endothelial cells. Objectives To investigate the presence of ANCA in patients with adult HSP and microscopic polyangiitis (MPA), and to measure serum LAMP-2 antibody levels in these patients. Methods Twenty-four adult patients with HSP, eight with MPA and 24 normal healthy controls were examined. ANCA detection was performed using indirect immunofluorescence (IIF), a direct enzyme-linked immunosorbent assay (ELISA) and a capture ELISA specific for myeloperoxidase (MPO) and proteinase 3 (PR3). We measured other ANCA-associated antibodies including anti-LAMP-2 antibody in serum using ELISA. Immunohistochemical (IHC) staining was used for anti-LAMP-2 antibody expression in patient skin biopsies. To determine the cut-off value of the serum anti-LAMP-2 antibody, a receiver operating characteristic (ROC) curve was constructed using statistical analysis software (JMP 8·0·2; SAS Institute Inc., Cary, NC, U.S.A.). Results The sera of all patients with HSP were negative for MPO-ANCA and PR3-ANCA by direct ELISA and by capture ELISA. However, ANCA was present in 17 (71%) of the 24 patients with HSP based on IIF. In contrast, we found MPO-ANCA in all eight patients with MPA using both ELISA methods. We found serum anti-LAMP-2 antibody levels in HSP significantly higher than in MPA and in healthy individuals ( P = 0·002 and P = 0·00167, respectively). The area under the curve of serum anti-LAMP-2 antibody between HSP and MPA was 0·8698 by ROC analysis. The optimal cut-off point was 0·267 U mL−1 (sensitivity 1·000, specificity 0·583). We found a significant positive correlation between serum anti-LAMP-2 antibody levels and serum IgA levels in HSP ( rs = 0·731, P = 0·00226). Anti-LAMP-2 antibody overexpression in IHC staining was present in 20 (83%) of the patients with HSP. The overexpression was observed within the neutrophils and endothelial cells of leucocytoclastic vasculitis. There was a significant positive correlation between IHC staining score and positive serum anti-LAMP-2 antibody. The 24 patients with HSP and the eight patients with MPA were negative for antiazurocidin antibodies, antibactericidal permeability increasing protein antibodies, anticathepsin G antibodies, antielastase antibodies, antilactoferrin antibodies and antilysozyme antibodies. Conclusions We suggest that anti-LAMP-2 antibody could play some role in the pathogenesis of adult HSP, and have excluded a role for MPO-ANCA and PR3-ANCA. We propose that measuring serum anti-LAMP-2 antibody could be a feasible method of differential diagnosis between HSP and MPA. [ABSTRACT FROM AUTHOR]

Most QTL detection studies in pigs have been carried out in experimental F2 populations. However, segregation of a QTL must be confirmed within a purebred population for successful implementation of marker-assisted selection. Previously, QTL for meat quality and carcass traits were detected on SSC 7 in a Duroc purebred population. The objectives of the present study were to carry out a whole-genome QTL analysis (except for SSC 7) for meat production, meat quality, and carcass traits and to confirm the presence of segregating QTL in a Duroc purebred population. One thousand and four Duroc pigs were studied from base to seventh generation; the pigs comprised 1 closed population of a complex multigenerational pedigree such that all individuals were related. The pigs were evaluated for 6 growth traits, 7 body size traits, 8 carcass traits, 2 physiological traits, and 11 meat quality traits, and the number of pigs with phenotypes ranged from 421 to 953. A total of 119 markers were genotyped and then used for QTL analysis. We utilized a pedigreebased, multipoint variance components approach to test for linkage between QTL and the phenotypic values using a maximum likelihood method; the logarithm of odds score and QTL genotypic heritability were estimated. A total of 42 QTL with suggestive linkages and 3 QTL with significant linkages for 26 traits were detected. These included selection traits such as daily BW gain, backfat thickness, loin eye muscle area, and intramuscular fat content as well as correlated traits such as body size and meat quality traits. The present study disclosed QTL affecting growth, body size, and carcass, physiological, and meat quality traits in a Duroc purebred population. [ABSTRACT FROM AUTHOR]

Background Matrilysin (MMP-7), a member of the matrix metalloproteinase (MMP) family of proteins, is expressed in various types of malignant tumours. There have been no previous studies of the correlation between matrilysin expression and melanoma. Objectives Protein expression of matrilysin was evaluated in human cutaneous melanomas, metastatic melanomas, acquired common melanocytic naevi and Spitz naevi, and the data were corrected with the clinicopathological factors. Methods We retrospectively investigated 18 primary melanomas, 15 metastatic melanomas, 10 common melanocytic naevi and five Spitz naevi samples at our clinic using immunohistochemistry (IHC). Both promatrilysin and active matrilysin were found in the melanoma tissue extracts by Western immunoblotting. In situ hybridization demonstrated that melanoma cells selectively express matrilysin mRNA. Results Of the melanoma samples, 29 of 33 (87·9%) were positive for matrilysin, including 14 of 18 (77·8%) primary cutaneous melanomas and 15 of 15 (100%) metastatic melanomas. In contrast, matrilysin was not expressed in common naevi or Spitz naevi. The matrilysin IHC staining score in primary melanomas was associated with the presence of metastases, tumour thickness and TNM staging ( P = 0·001, 0·025 and 0·021, respectively). The 5-year overall survival was 26·3% for matrilysin-positive cases and 100% for matrilysin-negative cases among melanoma specimen. Conclusions We found matrilysin expression in primary melanomas and in metastatic melanomas. We further demonstrated that the matrilysin IHC staining score was associated with invasive depth of primary melanoma lesions and metastases. Our observations indicate that matrilysin may be associated with melanoma progression, and may enhance melanoma tumour cell invasion. Therefore, matrilysin may be potentially valuable as a prognostic indicator to predict the clinical behaviour of melanoma. [ABSTRACT FROM AUTHOR]

Background Henoch–Schönlein purpura (HSP) is a small-vessel vasculitis characterized by palpable purpura on the lower extremities and IgA-dominant immune complex deposition within the wall and lumen of dermal vessels in the lesions. This disorder is associated, to varying degrees, with joint, gastrointestinal and renal involvement. Antiphospholipid antibodies, including anticardiolipin antibodies (aCL Abs), are a heterogeneous group of circulating autoantibodies found in patients with autoimmune and infectious diseases. Objectives To investigate the possible role of aCL Abs in adult HSP, we measured levels of serum IgA, C-reactive protein (CRP), aCL Abs of the IgG, IgM and IgA isotypes and anti- β2-glycoprotein I ( β2GPI)-dependent aCL Abs in adult patients with HSP. We evaluated the correlation between these biological parameters and the clinical manifestations. Methods Adult patients with HSP with an initial cutaneous manifestation of palpable purpura on their lower extremities seen between 2001 and 2005 in our department were retrospectively reviewed. Patients with known connective tissue diseases were not included in the study. Histological examination of all patient skin biopsy specimens revealed leucocytoclastic vasculitis in the upper and mid-dermis. Direct immunofluorescence analysis showed prominent deposits of IgA in the capillary walls of all patients. Blood samples were taken at the time that the patient presented. Serum levels of aCL Abs and anti- β2GPI-dependent aCL Abs were measured by enzyme-linked immunosorbent assay. Results Twenty adult patients with HSP (12 men and eight women), mean age 62·2 years (range 23–81) were enrolled. IgA aCL Abs were found in 15 of the 20 patients (75%). All were negative for IgG aCL Abs, IgM aCL Abs and anti- β2GPI-dependent aCL Abs. The elevation of serum IgA aCL Abs in the 15 patients showed a significant correlation with serum IgA and CRP levels ( rs = 0·91, P = 0·0007; rs = 0·80, P = 0·0026, respectively). Levels of serum IgA aCL Abs were also significantly associated with arthralgia ( P = 0·022) and proteinuria according to urinalysis ( P = 0·013). Conclusions Serum levels of IgA aCL Abs are elevated in the initial active stage of adult HSP, suggesting that serum IgA aCL Abs may play some role in the onset of adult HSP. We believe that serum IgA aCL Abs might be an indicator of adult HSP activity. [ABSTRACT FROM AUTHOR]

Acquired symmetrical dermal melanocytosis (ASDM) is a pigmentary disorder characterized by blue–brown macules most frequently seen on the face of young and middle-aged Asian women. ASDM developing after other skin diseases has not been previously reported.To characterize the clinical and histopathological features of ASDM associated with atopic dermatitis (AD) and to elucidate the differences between AD-associated ASDM and idiopathic ASDM.Sixteen patients with ASDM associated with AD were examined clinically and histopathologically and were compared with 69 patients with idiopathic ASDM.The AD patients associated with ASDM consisted of four men and 12 women with a mean age of 32·8 ± 13·1 years. Most patients remembered that the pigmented macules appeared in places where refractory eczema had existed for a long time. The marked preponderance in females and the appearance in the early reproductive period were common features of AD-associated ASDM and idiopathic ASDM. AD-associated ASDM was most commonly observed on the forehead (68·8%) and on the backs of the hands (50·0%), whereas 89·9% of idiopathic ASDM was seen on the cheeks. There was no significant difference in the number of dermal melanocytes between AD-associated ASDM and idiopathic ASDM. Electron microscopic studies demonstrated many mature melanocytes and smaller numbers of immature melanocytes in the dermis. Some melanocytes were seen adjacent to mast cells.AD-associated ASDM does not appear to be rare in Japan. ASDM may be triggered in AD patients by sunlight exposure, some alterations in sex hormones and/or persistent cutaneous inflammation. Histamine and stem cell factor produced by mast cells may play crucial roles in the pathogenesis of AD-associated ASDM. [ABSTRACT FROM AUTHOR]

Summary Background The mechanism by which a low dose of methotrexate (MTX) works to treat psoriasis is not clear. The overexpression of cell adhesion molecules on dermal vessels is important in the pathogenesis of psoriasis and is probably induced by upregulation of tumour necrosis factor (TNF)-α. Objectives To determine the effects of MTX at concentrations comparable with in vivo levels after the administration of low-dose MTX to human umbilical vein endothelial cells (HUVEC) on the growth and expression of intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1). Methods Cell proliferation assay, immunostaining, immunoblotting, cell enzyme-linked immunosorbent assay and reverse transcriptase–polymerase chain reaction (RT–PCR) were performed to examine the effects of MTX on HUVEC. Results MTX inhibited the proliferation of HUVEC at 10-7 mol L-1 and 10-6 mol L-1 without showing cytotoxic effects. It also inhibited TNF-α-induced ICAM-1 and VCAM-1 expression by HUVEC at 10-6 mol L-1 . The inhibitory effect of MTX was more pronounced on ICAM-1 expression than on VCAM-1 expression. RT–PCR analysis revealed that TNF-α-induced ICAM-1 gene expression was strongly downregulated by MTX. Conclusions Low-dose MTX may act on psoriasis by suppressing the TNF-α-induced expression of ICAM-1 and VCAM-1 by vascular endothelial cells. Inhibition of neovascularization may be another mechanism of action of MTX. [ABSTRACT FROM AUTHOR]

Background Bowen's disease is a well-established in situ malignancy of the epidermis. The keratin expression in Bowen's disease has been studied in many reports. However, the patterns of keratin (K) 14 expression in each case have not been closely examined. Objectives To investigate if the pattern of expression of K14 has a relationship with tumour progression, we analysed the expression patterns of K14 in relation to the nature of tumour cells, comparing tumour cells in direct contact with the dermis, tumour cells separated from the dermis, and tumour cells invading into the dermis. Methods Twenty-seven tissue sections from 22 patients were stained with anti-K14 antibody, as well as with antilaminin and periodic acid-Schiff (PAS) staining to evaluate the conditions of the basement membrane. Staining patterns of K10 and integrin β1, and their relationships with proliferating cell nuclear antigen (PCNA) and Ki-67 staining patterns, were also examined. Results Tumour cells with no, or with obscured, basement membranes always showed positive staining for K14, while those with continuous (intact) basement membranes usually did not. Of 10 sections showing dermal involvement of Bowen's disease, five were K14 positive and five were K14 negative. All of these K14-positive sections with dermal involvement showed negative or obscured laminin and PAS staining. Most of the sections having K14-negative tumour cells with dermal involvement showed K14-positive lining cells with continuous staining with laminin and PAS-positive basement membranes. K10 was reciprocally expressed with K14 in most of the sections. Integrin β1 was expressed in the basal layers of non-tumour epidermal cells, but not in tumour cells. Ki-67 and PCNA were expressed at high frequencies in tumour cells, clearly demarcating tumour cells from non-tumour cells. Conclusions Tumour cells separated from the dermis by lining cells were K14 negative with PAS- and laminin-positive basement membranes... [ABSTRACT FROM AUTHOR]

Background Mast cell infiltration in skin lesions of atopic dermatitis (AD) is considered to play an important role in the pathogenesis of the disease. The most common factor that stimulates mast cell growth, migration and differentiation is stem cell factor (SCF), and the interaction of SCF and its receptor, KIT (tyrosine kinase transmembrane receptor), appears to be the key event in the recruitment and proliferation of mast cells. Objectives To determine whether any altered metabolism of SCF and/or KIT is present in patients with AD. Methods We measured serum levels of soluble SCF (sSCF) and soluble KIT (sKIT) using enzyme-linked immunosorbent assay in 54 patients with AD, five patients with erythrodermic psoriasis vulgaris and 64 healthy individuals. Results Serum levels of both peptides in AD patients were significantly higher than those in healthy individuals, whereas patients with psoriasis vulgaris did not show any difference from healthy controls. Both sSCF and sKIT levels were positively correlated with the disease severity in AD patients, and decreased after effective treatment with topical corticosteroids. Conclusion Serum levels of sSCF and sKIT may be useful indicators for evaluation of the activity and severity of AD. [ABSTRACT FROM AUTHOR]

Reports issues related to dermatology. Expression of transforming growth factor in systemic sclerosis; Correlation between antinuclear antibodies and T-helper cells in lupus erythematosus; Incidence of infantile-type digital fibromatosis tumor in an adult.

Background Elevated levels of soluble vascular cell adhesion molecule-1 (sVCAM-1) and soluble E-selectin (sE-selectin) have previously been reported in patients with various inflammatory diseases, but not in patients with polymyositis/dermatomyositis (PM/DM). Objectives To determine serum levels and significance of sVCAM-1 and sE-selectin in patients with PM/DM. Patients and methods Serum samples from 36 PM/DM patients, 30 patients with systemic sclerosis and 25 healthy control subjects were examined using specific enzyme-linked immunosorbent assay systems. Results The serum levels of sVCAM-1 in the PM/DM patients were significantly higher than those in the healthy controls. The elevated serum sVCAM-1 levels were correlated with the values of elevated erythrocyte sedimentation rate and elevated serum hyaluronate levels in the PM/DM patients. The serum sE-selectin levels in the PM/DM patients were also significantly higher than those in the healthy controls. The elevated serum sE-selectin levels were correlated with the incidence of elevated creatine kinase activities. The concentrations of serum sE-selectin were correlated with the serum tissue inhibitor of metalloproteinase-1 concentrations in the PM/DM patients (r = 0·53). Conclusions These results suggest that serum sVCAM-1 and sE-selectin levels might be useful for detecting disease activity in patients with PM/DM. [ABSTRACT FROM AUTHOR]

We describe a 51-year-old Japanese woman with generalized pustular psoriasis treated with PUVA who subsequently developed a bullous disease. Throughout the disease course, there was no phase of psoriasis vulgaris. Although several reports describe coexistence of psoriasis vulgaris and bullous disease such as bullous periphigoid, coexistence of generalized pustular psoriasis without any phase of psoriasis vulgaris and bullous disease is rare. As for the bullous disease, direct immunofluorescence study showed IgG and C3 deposition along the basement membrane zone. Indirect immunofluorescence disclosed IgG anti-basement membrane zone antibodies. Indirect immunofluorescence on 1 mol/l sodium chloride-split skin demonstrated linear IgG staining almost exclusively on the dermal side of the split. Western immunoblot analysis revealed that the antibody was directed to neither epidermolysis bullosa acquisita antigen nor bullous pemphigoid antigens. Considering the unusual clinical course, we suspect the possibility of a novel autoimmune blistering disease. [ABSTRACT FROM AUTHOR]

This study investigated the prevalence and clinical significance of anti-ribosomal P protein (anti-P) antibodies in patients with systemic sclerosis (SSc). Serum samples from 150 patients with SSc were examined by indirect immunofluorescence, ELISA and immunoblotting. Anti-P antibodies were detected in four (3%) patients with SSc. Three of the four patients showed SSc/SLE (systemic lupus erythematosus) overlap syndrome, but psychiatric disorders were not observed in these patients. By longitudinal immunoblotting analysis one patient, who was initially diagnosed with SSc, later developed anti-P antibodies along with clinical manifestations of SLE. Our data suggest that anti-P antibodies are uncommon in SSc and that the presence of anti-P antibodies in patients with SSc indicates an overlap with SLE. [ABSTRACT FROM PUBLISHER]

Sclerotic skin change in systemic sclerosis (SSc) usually accompanies increased skin thickness. In order to quantify the cutaneous changes and to clarify the changes in the ‘uninvolved’ skin in systemic sclerosis (SSc), we measured the skin thickness on the chest, the forearms and the hands of 79 patients with SSc and 81 healthy controls with a B-mode ultrasound (30 MHz) apparatus. The thickness of the ‘uninvolved’, as well as the ‘involved’ skin in patients with SSc was significantly greater than that of healthy controls. Increased skin thickness on the forearms and/or the hands showed a 64.6% sensitivity and a 100% specificity for SSc. These results indicated that the skin which appears to be ‘uninvolved’ in patients with SSc is already pathologic, as shown by increased thickness. Moreover, measurement of skin thickness may be beneficial in the diagnosis of this disease at an early stage. [ABSTRACT FROM PUBLISHER]

Anti-p80-coilin antibody produces a unique pattern of immunofluorescence staining called nuclear dots characterized by the presence of up to six discrete nuclear bodies in interphase cell nuclei. The distribution of this antibody and its clinical relevance have not been established. We studied the prevalence of anti-p80-coilin antibody in the sera from 810 patients with collagen diseases and various skin diseases. Five sera showed the nuclear dot pattern by indirect immunofluorescence on an HEp-2 cell substrate, and reacted by immunoblotting with 80-kDa protein in a nuclear extract from HeLa cells. Pour of these sera were from patients with localized scleroderma, and the other was from a patient with primary Raynaud's phenomenon. The patients with localized scleroderma who were positive for anti-p80-coilin antibody had all been classified as having linear scleroderma. They had only one or two lesions, and were negative for antihistone and anti-single-stranded DNA antibodies. Our data indicate that anti-p80-coilin antibody is uncommon in skin diseases; however, this antibody is present in patients with a milder form of linear scleroderma, although the incidence of positivity may not he high. [ABSTRACT FROM AUTHOR]

In order to determine the prevalence and clinical significance of β2-GPI-dependent anticardiolipin antibodies (β2-GPI/aCL) in patients with systemic sclerosis (SSc), serum samples from 80 patients with SSc, 20 patients with systemic lupus erythematosus (SLE), and 120 healthy control subjects were examined by EL1SA using purified β2-GPI. IgG isotype β2-GPI/aCL was present in eight of 80 patients with SSc (10%), and the presence of β2-GPI/aCL IgG was significantly correlated with the presence of isolated pulmonary hypertension (P1-I). Furthermore, levels of β2-GPI/aCL lgG were significantly correlated with levels of mean pulmonary arterial pressure. These data suggest that EgG isotype β2-GPI/aCL might be a serological indicator of the severity of PH in patients with SSc. [ABSTRACT FROM AUTHOR]

Sera from patients with localized scleroderma frequently produce cytoplasmic staining by indirect immunofluorescence, although the antigen remains to be determined. We studied the prevalence, antigen specificity and associated clinical characteristics of anti-cytoplasmic antibodies in localized scleroderma. Serum samples from 60 patients with localized scleroderma were examined by indirect Immunofluorescence analysis and immunoblotting. By immunofluorescence analysis on HEp-2 cell substrate, seven of 60 (12%) patients were shown to be positive for anti-cytoplasmic antibodies. Among these, six patients with generalized morphea had anti-mitochondrial antibodies as shown by immunoblotting: they showed reactivity with the E2 component of pyruvate dehydrogenase complex (PDC), with protein X, and with the E2 component of α-oxo-glutarate dehydrogenase complex, while two of them showed reactivity with PDC-E1α. One of these patients who was positive for anti-PDC-E1α antibody showed laboratory abnormalities, suggesting the presence of primary biliary cirrhosis. The age of disease onset was significantly higher in these six patients than in those without anti-mitochondrial antibodies. Furthermore, five of them were classified into generalized morphea with multiple plaque lesions but without linear lesions (multiple plaque type). These observations suggest that major antigens for anti-cytoplasmic antibodies in patients with localized scleroderma are mitochondrial enzymes, 2-oxo-acid dehydrogenase complexes. Patients with anti-mitochondrial antibodies may comprise a unique subset of localized scleroderma designated multiple plaque type of generalized morphea of older onset. [ABSTRACT FROM AUTHOR]

This study was designed to explore the attitudes of dermatologists towards the Chapel Hill Consensus Conference (CHCC) nomenclature and classification. We developed a questionnaire to determine the views of chief assistant dermatological professors at 61 Japanese university hospitals. A χ2 analysis of the responses found a close relationship between dermatological facilities that based their evaluations on the CHCC and their likelihood of taking confirmatory skin biopsies from patients with suspected microscopic polyangiitis with cutaneous features. In those facilities, the physicians and pathologists tended to consider cutaneous polyarteritis nodosa and cutaneous leucocytoclastic angiitis as independent disease conditions. We believe that it would be beneficial for dermatologists to take advantage of the CHCC, through which an appropriate early diagnosis of vasculitis can be realized. The present investigation provides a picture of current practices of Japanese dermatologists with reference to the management of vasculitis, including the extent to which biopsies are used to establish the diagnosis. [ABSTRACT FROM AUTHOR]

Dermatomyositis (DM) often has a poor prognosis, due to complications associated with malignancy or interstitial pneumonia (IP). It is uncommon to find histopathological small-vessel vasculitis within cutaneous lesions and pulmonary capillaritis in patients with DM. A 64-year-old woman was diagnosed with DM based on the presence of quadriparesis, typical heliotropic rash, Gottron's papules, increased serum levels of muscle enzymes and typical muscle biopsy findings. She also had associated IP, which subsequently developed into a rapidly progressive condition. We found a high titre of Krebs von den Lunge (KL)-6 in her serum. Measurement of serum KL-6 level is widely accepted as a diagnostic test to monitor the activity of interstitial lung diseases. Histopathological examinations of the cutaneous and lung-associated features showed small-vessel vasculitis. We suggest that investigation of KL-6 levels in patients with DM and associated conditions should be carried out to determine if raised KL-6 levels are useful at predicting outcome or severity of various features. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article about tuberous sclerosis, which appeared in the previous issue.

A letter to the editor is presented in response to the article concerning the therapeutic effect of lipoprostaglandin E1 on livedoid vasculitis associated with essential cryoglobulinemia.

Presents a letter to the editor about anaplastic large cell lymphoma in a patient with hepatitis C virus infection.

Summary We describe a 20-year-old woman with trisomy 18 mosaicism, who presented with skeletal anomalies, epilepsy, mental retardation, and linear and whorled naevoid hypermelanosis. [ABSTRACT FROM AUTHOR]

Focuses on the immunohistochemical analysis of transforming growth factor beta3 expression in solitary morphoea profunda with histological membranocystic changes. Characteristics of morphoea profunda; Reference on the case of 29-year old Japanese woman with skin lesion; Diagnosis of the disease.

Investigates the clinical relevance of serum soluble CD30 levels in patients with dermatomyositis (DM)/polymyositis. Clinical and serological features; High frequency of elevated sCD30 levels in patients with DM.

Examines the immunohistochemical analysis of transforming growth factor (TGF)-β3 expression in acquired reactive perforating collagenosis (ARPC). Mediator of dermal-epidermal interactions; Overexpression of immunoreactive TGF-β3 in ARPC patients; Details on the transepidermal elimination of the necrotic substance.

Details the case of 3-year-old Japanese boy diagnosed with benign cephalic histiocytosis, a rare, benign, self-healing form of non-X histiocytosis. Small brown papules scattered on the face, neck, upper arms, buttocks and thighs; Histopathological features; Nature and course of the disease.

The article presents a case study of an 18-year-old Japanese girl with a two-year history of asymptomatic and hyperpigmented spots on her face and upper part of her back without causal relation to medication or inflammatory skin disease. Histopathological findings show that the patient has idiopathic eruptive macular pigmentation. The patient was treated with a Q-switched ruby laser, leading to partial resolution of the pigmentation.