Akio Yamakage

Quantitative analysis of microchimerism in systemic sclerosis skin tissue

Abstract It has been reported that more male DNA of presumed fetal origin is present in the blood and skin of women with systemic sclerosis (SSc) as compared with healthy controls after delivery, but these findings are controversial. We sought to determine whether male cell DNA is present in SSc using a quantitative polymerase chain reaction for Y chromosome DNA. The study groups comprised 57 healthy women, 49 patients with SSc and 30 patients with connective tissue diseases other than SSc who had given birth to at least one son and/or had experienced fetal loss. The intensity of the PCR bands on negatives of gel photographs was quantified with a video densitometer linked to a computer analysis system. Positive Y chromosome DNA was found in 20 healthy women, 14 SSc patients and 6 patients with connective tissue diseases other than SSc. The occurrence rate of DNA equivalents of male cells among the three groups showed no significant differences. The number of male cell DNA equivalents per 80 ng tissue DNA in SSc patients (4.59 ± 9.63), however, was significantly higher than in healthy women (1.83 ± 4.96; P < 0.05) and in patients with connective tissue diseases other than SSc (0.27 ± 0.64; P < 0.01). The occurrence rate of fetal loss in male cell DNA-positive SSc (eight) was significantly higher than in male cell DNA-negative SSc patients (four; P < 0.01). No correlation was found between the number of male cell DNA equivalents and birth of sons or clinicolaboratory findings. These results indicate that the elevated amount of male cell DNA in SSc skin tissue may contribute to the pathogenesis of SSc.

The polymorphism of transforming growth factor‐β1 gene in Japanese patients with systemic sclerosis

Background Transforming growth factor (TGF)‐β has been shown to be a potent stimulator of collagen production by fibroblasts, and could play a role in the pathogenesis of systemic sclerosis (SSc).

Cutaneous localization of endothelin-1 in patients with systemic sclerosis: immunoelectron microscopic study

Background Endothelin‐1 (ET‐1) has some relation to the pathogenesis of systemic sclerosis (SSc) and Raynauds phenomenon. This study was performed to determine the localization of ET‐1 in patients with SSc.

Nailfold capillary abnormality and pulmonary hypertension in systemic sclerosis

Background

Digital ulcers and necroses: novel manifestations of angiocentric lymphoma.

We describe a patient with angiocentric lymphoma whose presenting features were multiple areas of digital ulceration and necrosis, including deep ulcers on both great toes. He lacked the lateral halves of both earlobes because of multiple ulcers. Skin biopsy revealed a patchy and diffuse infiltrate of lymphoid cells with nuclear atypia in the dermis and subcutaneous tissue. Angiocentric and angiodestructive features of the lymphoid cells, a prominent histiocytic infiltrate and some epithelioid cell granulomas were found. The results of immunohistochemical staining showed a T‐cell phenotype, and showed positive staining for apoptosis. He died in July 1999. Peripheral vascular disturbances including Raynaud’s phenomenon, digital skin ulcers and necroses are novel clinical symptoms in patients with angiocentric lymphoma, which should be added to the differential diagnosis in patients with peripheral vascular disturbances.

Electron-microscopic study of sclerodermatous chronic graft-versus-host disease

Background. Sclerodermatous chronic graft‐versus‐host disease (SC‐GVHD) resembles systemic scleroderma (SSD) closely, both clinically and histologically. Our purpose was to try to define the morphologic differences of collagen fibers between SC‐GVHD and SSD.

A Case of Lichen Myxedematosus with Clearly Exacerbated Skin Eruptions after UVB Irradiation

We report a 48‐year‐old male with typical lichen myxedematosus, liver dysfunction, and diabetes mellitus. His skin eruptions were clearly exacerbated after accidental over‐irradiation by UVB. These findings were clinically and histologically confirmed. The pathogenesis is still unknown, but we consider it to be due to Koebner phenomenon.

Correlation between diffuse pigmentation and keratinocyte-derived endothelin-1 in systemic sclerosis.

Abstract

The polymorphism of telomerase RNA component gene in patients with systemic sclerosis

Summary Background The molecular basis of senescence and immortalization is not still understood, but one hypothesis for which there has recently been much evidence involves the shortening of telomeres. It can be hypothesized that abnormalities of telomerase contribute to the emergence of abnormal fibroblast clones in systemic sclerosis (SSc).

Cutaneous electron microscopic study of sclerodermia diffusa in childhood

A 9‐year‐old Japanese boy presented in July 1994 with gradual skin sclerosis of the extremities and Raynauds phenomenon of 10 months duration. He had been treated with oral prednisolone after the diagnosis of juvenile rheumatoid arthritis at the pediatric clinic. He had skin sclerosis with luster on his trunk and extremities (Fig. 1A). His skin score was III and IV, with the exception of his face (I, normal; II, edema; III, edematous sclerosis; IV, skin sclerosis; V, skin atropy). His hands showed finger contracture and he had reticular pigmentation on the back of both hands (Fig. 1B). He had no symptoms of digestive and respiratory organs.