Tokihiko Shimada

Three cases of pityriasis rubra pilaris successfully treated with cyclosporin A.

Background: Pityriasis rubra pilaris (PRP) is reported to respond poorly to cyclosporin A (CsA). Objective: We attempted to determine the efficacy of CsA in the treatment of classic adult-type PRP. Methods: Three patients with classic adult-type PRP were treated with 5 mg/kg/day CsA. Results: A sustained clinical response was achieved within 2–4 weeks of therapy. Relapses were noted when the CsA dose was decreased to 1.2 mg/kg/day. Conclusion: CsA should be considered in the treatment of classical adult-type PRP.

Correction of ornithine transcarbamylase (OTC) deficiency in spf-ash mice by introduction of rat OTC gene

We introduced rat ornithine transcarbumylase (OTC) gene into OTC‐deficient spf‐ash mice by mating spf‐ash heterozygotes with transgenic mice which carried recombinant DNA composed of 1.3 kb of the 5′ flanking region of the gene fused onto rat OTC cDNA. The liver OTC activity of hemizygous spf‐ash mice which carried the transgene was about twice that of nontransgenic spf‐ash mice, and the small intestinal OTC activity was 6 times higher; the values being 12% and 27% of the control levels, respectively. The transgenic spf‐ash mice showed normal hair growth without sparse, fur, nearly normalized urinary orotic acid excretion and normalized serum citrulline concentration.

A Case of Localized Pemphigus Foliaceus

Pemphigus foliaceus (PF) is most commonly observed on the face, scalp, chest and back at the onset of the condition. The case described here is that of an 81‐year‐old female with a single PF lesion localized to the right cheek. A review of the literature published in English and Japanese disclosed only 3 cases of PF in which the patient presented with a single lesion, and 2 of these cases were referred to as “localized pemphigus foliaceus”.

A Case of Chromomycosis with Tumor-like Growth

A 66‐year‐old man who lived on Tokunoshima Island, a small and remote southern island of the Japanese archipelago, had suffered from chromomycosis for more than 30 years and presented with a tumor‐like growth on the posterior crural region of his right leg. Fonsecaea pedrosoi was identified as the pathogen from its growth pattern and micromorphological characteristics. The patient was successfully treated with 5‐fluorocytosine, itoraconazole, and topical thermotherapy.

Simultaneous detection of mutant gene and transgene in ornithine carbamoyltransferase-deficient spf-ash mice with rat OCT gene

Ornithine carbamoyltransferase (OCT; EC 2.1.3.3) is a mitochondrial matrix enzyme which functions in the urea cycle in the liver and in the synthesis of citrulline in the small intestine. In both human beings and mice, the OCT gene is located on the X chromosome and is composed of 10 exons spread over 70-80 kb. Two mouse strains with partial OCT deficiency serve as useful animal models: sparse-fur (spf), and sparse-fur with abnormal skin and hair (spf-ash). As their names imply, both these strains have delayed hair growth and, as in human OCT deficiency (McKusick 311250), they show hyperammonaemia, orotic aciduria and low concentration of serum citrulline. Their molecular and genetic defects have been analysed. The spf mouse phenotype is caused by a missense mutation, changing His to Asn at amino acid 117 (Veres et al 1987). The spf-ash mutation is a G to A transition of the last nucleotide of the fourth exon of the OCT gene (Hodges and Rosenberg 1989). These mice have served as experimental subjects for gene therapy (Cavard et al 1988; Jones et al 1990; Shimada et a l 1991). Murakami et al (1989) introduced recombinant DNA carrying 1.3 kb of the Y-flanking region of rat OCT gene fused to rat OCT cDNA into fertilized mouse eggs and established transgenic mice. Shimada et al (1991) introduced rat OCT cDNA into spf-ash mice by mating splash heterozygotes with transgenic mice. The liver OCT activity of the hemizygous spf-ash mice which carried the transgene was about twice that of non-transgenic splash mice, and the small intestinal OCT activity was 6 times higher. These values are 12% and 27% of the control levels, respectively. The spf-ash mice with the transgene showed normal skin and hair growth without sparse fur, nearly normalized urinary orotic acid excretion and serum citrulline concentration. At that time, Shimada et al (1991) used Southern blot analysis to detect the transgene and measured the enzyme activity to detect the spf-ash gene, because spf-ash mice with transgene, which have normalized skin and hair, could not be distinguished from normal mice.

Cutaneous Arterial Fibromuscular Dysplasia: A Case Report and Electron-microscopic Study

A case of a 39‐year‐old female with fibromuscular dysplasia (FMD) manifesting subcutaneous pulsatile nodules on the right side of her forehead and on her right wrist is reported here. These nodules proved to be FMD aneurysms of the frontal ramus of the right superficial temporal artery and the right radial artery. The patient had also suffered a stroke with subarachnoidal bleeding as a result of this disease.

Normalization of hair growth in sparse fur-abnormal skin and hair (SPF-ASH) mice by introduction of the rat ornithine transcarbamylase (OTC) gene

The sparse fur-abnormal skin and hair (SPF-ASH) mouse is a model for the human X-linked hereditary disease, ornithine transcarbamylase (OTC) deficiency. This condition is characterized by abnormal skin and delayed hair growth, hyperammonemia, orotic aciduria and low levels of serum citrulline and arginine. Murakami et al. [1] established a line of transgenic mice, by introducing the recombinant rat OTC (rOTC) gene into fertilized C57BL mouse eggs. We introduced the rOTC gene into SPF-ASH mice by mating SPF-ASH heterozygotes and transgenic mice, which carried this gene. The hemizygous SPF-ASH mice bearing the rOTC gene showed normal hair growth without sparse fur, normal urinary orotic acid excretion and normal serum citrulline and arginine levels. These mice showed OTC activities 2 and 6 times higher in the liver and small intestine, respectively, than the SPF-ASH mice but about 12% and 27% those of the controls [2].

On MRI images of skin cancer.

Growth patterns of malignant cutaneous tumors in 21 patients obtained by magneti resonance imaging (MRI) were assessed in contrast with those in histological examinations.Degree of intradermal invasion in MRI pictures of the lesions was well corresponded with that in histological examinations. In general, intensit of tumor images was higher in T2 weighted images than that of T1 weighted images. It is very likely that cellularity, inflammation, edema and degeneration of the lesions may cause the phenomena.MRI seems to be better to analyze tumor invasion arising in head and neck region than other imaging method such as CT scan, xerography and FCR, because MRI enables us to select slice freely, when the lesion occurs in unevenly surfaced region.