Increased CD27 expression in the skins and sera of patients with systemic sclerosis.

Systemic sclerosis (SSc) is a kind of collagen disease and has an acquired autoimmune activation as represented by the production of autoantibodies. CD27 is a type I glycoprotein and a member of the tumor necrosis factor receptor family. It binds to the CD70 ligand, CD27-CD70 signaling is implicated in the development of various autoimmune diseases, but its role in the regulation of extracellular matrix expression and its contribution to the phenotype of SSc both remain to be elucidated. This study aimed to investigate the associations between CD27 and SSc in the skins and sera. Immunohistochemistry were performed to determine the expression of CD27 in the skin. Enzyme-linked immunosorbent assays were done to the sera of the 54 patients with SSc and 23 normal healthy controls. CD27 expression was significantly increased in the affected regions of the skin and the sera of patients of SSc. Thereafter, we evaluated the correlation between the serum soluble CD27 (sCD27) levels and the clinical symptoms. The study subjects with increased sCD27 levels had a significantly higher ratio of dcSSc and to showed higher modified Rodnan's total skin thickness scores (mRSS) than those with normal sCD27 levels. These results suggest that sCD27 levels might be useful for diagnosis of SSc and its severity.

Case Report: Concurrent Sympatric Scrub Typhus and Japanese Spotted Fever in Japan.

Scrub typhus and Japanese spotted fever-both rickettsial diseases-are endemic and notifiable in Japan and may cause a fatal outcome without prompt treatment. Here we present the first case of a concurrent sympatric infection of both diseases with grade II evidence. A 67-year-old woman, after a single event of potential exposure to the pathogens, presented with a 12-day history of fever, pharyngeal pain, papulo-erythematous rash, and pronounced fatigue. Her erythematous rash was distributed on her trunk and extremities, palms, and soles and eventually progressed to purpura. Fever persisted until doxycycline was administered on day 12. A significant > 4-fold increase in immunoglobulin G and immunoglobulin M titers against multiple serotypes of Orientia tsutsugamushi and Rickettsia japonica were revealed by indirect immunoperoxidase assays. These clinical and serological data, even in the absence of molecular or isolation evidence, provided grade II evidence that this was a concurrent infection of sympatric scrub typhus and Japanese spotted fever.

Decreased miR-7 expression in the skin and sera of patients with dermatomyositis.

Expression of microRNA (miRNA) in the skin in dermatomyositis has not previously been studied in detail. In this study, we performed miRNA array analysis using miRNAs purified from dermatomyositis-involved skin and normal skin, and found that several miRNAs were up- or down-regulated in dermatomyositis skin. Among them, we focused on miR-7, one of the most down-regulated miRNAs in dermatomyositis skin. Total miRNAs were purified from serum, and hsa-miR-7 levels were measured with quantitative real-time PCR using the specific primer. Serum levels of miR-7 were significantly decreased in patients with dermatomyositis compared with normal subjects or patients with other autoimmune diseases. Thus, serum miR-7 levels might be a possible diagnostic marker for dermatomyositis. Clarifying the up- or down-stream events of down-regulated miR-7 in patients with dermatomyositis may lead to further understanding of the disease and a new therapeutic approach.

Photosensitivity and acute liver insufficiency in late-onset erythropoietic protoporphyria with a chromosome 18q abnormality.

Late-onset erythropoietic protoporphyria (EPP) is rare, and it is usually associated with an acquired somatic mutation of the ferrochelatase gene secondary to hematological malignancy such as myelodysplastic syndrome or myeloproliferative disorder. In 0.5-1% of patients with EPP, deposition of protoporphyrin in the liver leads to progressive liver insufficiency. Herein, we report the case of a 67-year-old female who developed EPP with typical photosensitivity and hemolytic anemia. Six months later, she was admitted with acute liver damage with a rapidly progressing course, and developed liver insufficiency. She recovered from the liver insufficiency after undergoing plasmapheresis and red blood cell exchange transfusion. A bone marrow examination revealed normal features; however, a cytogenetic analysis identified an abnormal clone of cells with a translocation between chromosomes 13q12 and 18q21.1. This is the first report of a patient who recovered from liver insufficiency. The results of this report suggest that plasmapheresis and red blood cell exchange transfusion are effective for treating liver insufficiency in patients with late-onset EPP.