[Show abstract][Hide abstract] ABSTRACT: Uncontrolled chronic hyperglycaemia including type 2 diabetes mellitus (DM) induces many skin problems related to chronic impaired skin barrier state. However, little is known about the skin barrier state of chronic hyperglycaemia patients, the dysfunction of which may be a major cause of their skin problems. In this study, we investigated whether a long-standing hyperglycaemic condition including type 2 DM impairs skin barrier homoeostasis in proportion to the duration and its pathomechanism. We utilized the Otsuka Long-Evans Tokushima Fatty (OLETF) rats as an animal model of long-standing hyperglycaemia and Long-Evans Tokushima Otsuka rats as a control strain. We confirmed that a long-standing hyperglycaemia delayed skin barrier homoeostasis, which correlated with haemoglobin A1c levels. OLETF rats as a long-standing hyperglycaemia model exhibited decreased epidermal lipid synthesis and antimicrobial peptide expression with increasing age. Decreased epidermal lipid synthesis accounted for decreased lamellar body production. In addition, OLETF rats had significantly higher serum levels of advanced glycation end products (AGEs) and elevated levels of the receptor for AGE in the epidermis. A long-standing hyperglycaemic condition impairs skin barrier function including permeability and antimicrobial barriers by accelerating skin ageing process in proportion to the duration of hyperglycaemia, which could be a major pathophysiology underlying cutaneous complications of DM.
[Show abstract][Hide abstract] ABSTRACT: Nevus sebaceous (NS) is a type of classical nevus or congenital malformation that is often present at birth and commonly involves the scalp or face. The lesion usually presents as a linear, yellow, hairless, and verrucous plaque. It has been well-established that several benign and malignant tumors can develop from the NS; however, there have been no reports about ectopic fat cells in the dermis, and cornoid lamella arising from the NS. We report a case of NS on the scalp with accompanying unusual histopathologic findings.
Annals of Dermatology 10/2011; 23(Suppl 2):S231-4. · 0.61 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A mucocele is a common, benign lesion of the oral cavity that develops following the extravasation or retention of mucous from a major or minor salivary gland. A pyogenic granuloma (PG) is also a common, benign condition characterized by proliferating capillaries that affect the skin and mucous membranes. The concurrent occurrence of a mucocele and a PG lesion has rarely been reported in the medical literature. This case study reports such a finding in a 16-year-old patient who presented to our department.
[Show abstract][Hide abstract] ABSTRACT: Epidermolysis bullosa (EB) is a rare hereditary disorder characterized by formation of blisters following minor trauma. It has been traditionally categorized by the level of basement membrane zone separation into EB simplex (EBS), junctional EB (JEB), and dystrophic EB (DEB). Recently, hemidesmosomal EB has been proposed as a fourth category, which includes EB with muscular dystrophy and EB with pyloric atresia. We report here on a case of concomitant occurrence of EB and pyloric atresia, a rare form of EB.
[Show abstract][Hide abstract] ABSTRACT: Atrophoderma of Pasini and Pierini is a form of dermal atrophy that manifests as either single or multiple, sharply demarcated, hyperpigmented, non-indurated patches. These patches are marked by a slight depression of the skin, with an abrupt edge (i.e., the "cliff-drop" borders), usually located on the backs of adolescents or young adults. The pathophysiology of the disease is unknown, but some authors have suggested a role of Borrelia burgdorferi infection. A 35-year-old woman visited our department because of asymptomatic, hypopigmented, depressed patches on her chest and back lasting for three months. Laboratory evaluations were normal, except for positive serum antibodies to Borrelia burgdorferi. Histologic examination revealed a significantly decreased thickness of the dermis. The patient underwent treatment with oral doxycycline 200 mg/day for six weeks, after which the depth of depression was improved. Herein, we report a case of atrophoderma of Pasini and Pierini, associated with Borrelia burgdorferi infection, successfully treated with oral doxycycline.
Annals of Dermatology 08/2011; 23(3):352-6. · 0.61 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Recently, we reported on the anti-ageing effects of K6PC-5. This compound induced keratinocyte differentiation and fibroblast proliferation by increasing sphingosine-1 phosphate synthesis. We performed this study to confirm the anti-ageing effects of new synthetic products (the K6EAA series) derived from K6PC-5 through an amino group induction. Cellular responses such as differentiation, proliferation and calcium mobilization were investigated using cultured human keratinocytes and fibroblasts. Also, we measured the expressions of collagen mRNA and protein using real time RT-PCR and ELISA, respectively. The K6EAA-L12 product, selected by in vitro screening, was evaluated for anti-ageing effects on intrinsically and extrinsically (photo) aged models of hairless mice. In the intrinsically aged murine skin, K6EAA-L12 showed anti-ageing effects by activating collagen synthesis, eventually causing dermal thickening. Also, in the photo-aged skin, the dermal collagen density and dermal thickness were increased. In photo-aged murine skin, K6EAA-L12 increased stratum corneum integrity by increasing corneodesmosome density and improved the barrier recovery rate. However, there were no changes in the expressions of epidermal differentiation maker proteins. In conclusion, topical K6EAA-L12, a new synthetic K6PC-5 derivative, improves intrinsically and extrinsically (photo) aged skin by increasing the collagen density and improving the skin barrier function.
[Show abstract][Hide abstract] ABSTRACT: Cherry angiomas are a common cutaneous vascular proliferation which manifests as single or multiple spots and occurs predominantly on the upper trunk and arms. They typically appear as round-to-oval, bright, red, dome-shaped papules and pinpoint macules measuring up to several millimeters in diameter. The histopathologic findings of a cherry angioma are consistent with a true capillary hemangioma, which is formed by numerous, newly developed capillaries with narrow lumens and prominent endothelial cells arranged in a lobular fashion in the papillary dermis. Herein, we report a case of multiple cherry angiomas on the scalp, an uncommon location for cherry angiomas.
[Show abstract][Hide abstract] ABSTRACT: Sphingosine-1-phosphate (S1P), which is formed by phosphorylation of sphingosine through a process catalysed by sphingosine kinase (SK), is a multifunctional mediator of a variety of cellular responses including proliferation, differentiation, motility, and survival. K6PC-5, which was recently synthesized as a novel SK activator, is expected to increase S1P levels. Indeed studies have already demonstrated that K6PC-5 exhibits anti-aging effects on intrinsic aged murine skin by increasing fibroblasts, collagen synthesis, dermal thickness, and epidermal differentiation. However, photoaging and intrinsic aging have highly different clinical and histopathological properties. In this study, we developed a photoaged murine model by exposing mice that were 56 weeks old to ultraviolet (UV)B and UVA radiation for 8 weeks. We then investigated whether K6PC-5, as an SK activator, had anti-aging effects on photoaged murine skin in addition to its effects on intrinsic aged murine skin and determined the mechanism. K6PC-5 increased dermal collagen density in photoaged skin through increases in fibroblasts and collagen production. Photoaged murine skin treated with K6PC-5 showed an increase in stratum corneum (SC) integrity with increased corneodesmosome density and an improvement in barrier recovery rate. Matrix metalloproteinase 13 remained unchanged. These results indicate that topical application of K6PC-5 improves photoaged skin by improving skin barrier and increasing fibroblast count and function. In conclusion, K6PC-5, as an S1P activator, improves long-term UV-exposed aged skin as well as intrinsic aged skin.
[Show abstract][Hide abstract] ABSTRACT: Glycogen storage disease type I (GSD-I) is a group of autosomal recessive disorders with an incidence of 1 in 100,000. The two major subtypes are GSD-Ia, caused by a deficiency of glucose-6-phosphatase (G6Pase), and GSD-Ib, caused by a deficiency of glucose-6-phosphate transporter (G6PT). We report that a substantial improvement was achieved following several infusions of hepatocytes in a patient with GSD-Ib. Hepatocytes were isolated from the unused cadaveric whole livers of two donors. At the first transplantation, approximately 2 x 10(9) cells (2% of the estimated recipient's total hepatocytes) were infused. Seven days later 1 x 10(9) (1% of liver mass) cryopreserved hepatocytes from the same donor were infused, and an additional 3 x 10(9) (3% of liver mass) cells from the second donor were infused 1 month after the second transplantation. After the hepatocyte transplantation, the patient showed no hypoglycemic symptoms despite the discontinuation of cornstarch meals. Liver biopsies on posttransplantation days 20 and 250 showed a normal level of glucose-6-phosphatase activity in presolubilization assay that was very low before transplantation. This was the first and successful clinical hepatocyte transplantation in Korea. In this study, hepatocyte transplantation allowed a normal diet in a patient with GSD-Ib, with substantial improvement in their quality of life. Hepatocyte transplantation might be an alternative to liver transplantation and dietary therapy in GSD-Ib.