• Imaging Science in Dentistry
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• 제33권3호
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• pp.195-198
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• 2003

We present two cases of multiple jaw cysts not associated with basal cell nevus syndrome. Case 1: a nine year-old boy visited CNU Hospital for orthodontic treatment and his radiographs showed cystic lesions surrounding the crowns of teeth #13 and #17 respectively, which were diagnosed as dentigerous cysts. Subsequently, two more cysts were found on his follow-up radiographs in 12 and 15 months. The two cysts were determined to be odontogenic keratocysts. The boy had no skeletal abnormalities and no skin lesions associated with basal cell nevus syndrome. Case 2: a fifty-eight year old man had three impacted third molars with pericoronal radiolucencies, which were diagnosed as dentigerous cysts. He had no additional abnormalities associated with basal cell nevus syndrome. Multiple jaw cysts can occur at any age, and periodic radiographic surveillance may be needed for any cases of impacted tooth.

• Journal of Chest Surgery
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• 제21권4호
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• pp.711-715
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• 1988

Pulmonary arteriovenous fistula is a congenital vascular malformation originated from abnormalities of capillary development. Fistulas may develop only in the lung or may be associated with similar abnormalities of the skin, mucous membrane, and the other organs. It may occur with hereditary hemorrhagic telangietasia[Rendu-Osier-Weber syndrome]. Recently we have experienced a case of the pulmonary arteriovenous fistula associated with Schwannoma in 20 year old male man. Tennis ball sized bright reddish mass which was composed of variable sized vascular channels filled with blood clots was located in the just subpleural portion of left lower lobe. Left lower lobectomy was done. Microscopic findings showed variable sized numerous blood vessels embedding in the parenchyma. There was a thumb-tip sized brown nodular mass which was turned out to be Schwannoma at the left 7th thoracic paravertebral area.

• Journal of The Korean Society of Inherited Metabolic disease
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• 제22권2호
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• pp.53-57
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• 2022

Congenital metabolic disorders are rare inherited disorders resulting from a defect in biochemical and metabolic pathways affecting proteins, fats, carbohydrates metabolism or impaired organelle function. Depending on the abnormality of biochemical metabolism, various precursors and their abnormal metabolites can accumulate in the body and the final products which are critical in normal physiology can be deficient, resulting in disease. Congenital metabolic disorders present complicated medical conditions involving several human organ systems, including nervous system, eyes, liver, and kidneys. Various proteins and lipids are involved in the development and homeostasis of the skin, so many congenital metabolic disorders present abnormal changes in skin and hair. In this review, congenital metabolic diseases related to amino acid and lipid metabolism accompanying skin abnormalities will be discussed.

• Journal of Veterinary Clinics
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• 제30권4호
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• pp.283-287
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• 2013

Canine skin is a highly dynamic organ that is constantly adapted to changes in its environment. It provides structural, sensory, immunologic, and physiologic functions and contributes an essential barrier function against potential environmental insults. We assessed the changes of canine skin hydration, pH, and protein contents by noninvasive method according to the frequency of application with canine commercial shampoo. Canine commercial shampoo was applied topically every other day, 5 days, and 8 days on different sites, respectively in 8 dogs. Saline-applied site was as a control. Skin hydration was increased and skin surface pH was decreased significantly in canine commercial shampoo-applied site every other day (p < 0.05). Also, skin protein concentration measured by D-squame$^{(R)}$ tape stripping method was increased significantly in canine commercial shampoo-applied site every other day (p < 0.05). There were alterations on every 5 days and 8 days, but not significantly. These results may be recognized that the frequent use of canine commercial shampoo cause abnormalities of the skin barrier function and alteration of stratum corneum integrity/cohesion. Consequently it was suggested that bathing the dog with canine commercial shampoo was appropriate every 5 or 8 days.

• Korean Journal of Head & Neck Oncology
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• 제13권1호
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• pp.86-89
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• 1997

Neurofibromatosis, now termed neurofibromatosis type I, is known as a congenital and familial disease presenting abnormalities of the skin, nervous system, bones, and soft tissue. We experienced a case of extremely large neurofibromatosis which developed on the orbital and temporal region of a 24-year-old man. The tumor was widely excised including normal skin margin, outer table of cranium, a part of zygoma and maxilla. Bony defect was reconstructed by rib bone graft and secondary cosmetic correction of blepharoptosis was performed using supratarsal fixation in postoperative 6 months.

• Journal of the Korean Applied Science and Technology
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• 제40권6호
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• pp.1330-1339
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• 2023

In order to verify whether the serum using the medicinal plant Coptis japonica is effective in anti-aging of the skin, the improvement rate was analyzed by measuring the skin elasticity and radiance of the subjects, and the satisfaction was evaluated through a questionnaire. Twenty-two female subjects were given a serum containing 3% Coptis Japonica extract on one cheek for 4 weeks, and the test site was measured, and the skin elasticity increased by 5.73% from 0.8505 ± 0.0395 R2 (mm) before use to 0.8993 ± 0.0234 R2 (mm) after 4 weeks. Skin glow increased by 4.74% from 55.94 ± 5.49 (a.u.) to 58.59 ± 4.66 (a.u.), and the probability of significance was p<0.001, indicating a significant increase in the improvement rate of both items. It was found that all subjects were 100% satisfied with the improvement of elasticity and 95.5% satisfied with the improvement of radiance, and no skin abnormalities were found during the trial period. These results are expected to be used as basic data for future research and product development of anti-aging functional cosmetics using Coptis Japonica extract.

• Clinical and Experimental Pediatrics
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• 제46권11호
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• pp.1135-1138
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• 2003

KID syndrome was named after the initials of the major three symptoms of the disease; keratitis, ichthyosis, and deafness. The syndrome was first introduced by Dr. Burns in 1915 who described one of his patients with those symptoms. In 1981, Dr. Skinner and his colleagues reported 17 patients who had keratitis, ichthyosis, and deafness. They also called the disease KID syndrome for the first time. After that, there have been only 60 cases of KID syndrome reported. KID syndrome may be presented with neurosensory deafness, vascularizing keratitis and such skin disorders as ichthyosis, ichthyosiform skin eruptions, and alopecia. Also, those with KID syndrome may suffer from repetitive infections, dental affections, hypohidrosis, growth delay and hepatic and renal abnormalities. The authors report the first case of KID syndrome in Korea with some references. The patient presented with keratitis, neurosensory deafness, and such characteristic skin disorders as ichthyosis and hyperkeratosis.

• Clinical and Experimental Pediatrics
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• 제46권6호
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• pp.606-609
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• 2003

Goltz syndrome(focal dermal hypoplasia) is a rare disorder characterized by ectodermal and mesodermal dysplasia described in 1962 by Goltz. In Korea, one case of Goltz syndrome was reported in 1994. The inheritance mode is mostly X-linked dominant. Skin abnormality is the most common manifestation including hypoplasia of the dermis. Skeletal involvement such as syndactyly, polydactyly, scoliosis, kyphosis and spina bifida occulta may be present, also ocular and dental abnormalities are reported. Radiologic findings are the osteopathy and striation of the long bone. We experienced a case of Goltz syndrome in a 9-year old female who was presented with right side hypotrophy, focal dermal hypoplasia, ocular(anidria, microcornea), dental(oligodontia, amelogenesis) and skeletal(syndactyly) abnormalities. Skin biopsy was performed and showed decreased expression of type I collagen gene with Northern blotting.

• Journal of Veterinary Clinics
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• 제22권2호
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• pp.157-159
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• 2005

Two-month old male Shih Tzu weighing 2.1 kg was presented with alopecia in black-haired areas of the skin. The hair loss had been present since five weeks of age. There was no history of pruritus on any part of the body. No other symptoms had been recognized. Physical examination found no abnormalities other than hair loss. Skin examination showed marked alopecia of black-haired area of the body. The white areas appeared to have hair growth of normal density and texture. Affected pigmented areas showed no evidence of skin lesions. Skin scraping and fungal culture were negative. Microscopic examination of plucked black hairs showed marked pigment clumping in the remnants of the follicles and were mainly in telogen phase. But white hairs were normal in various stages of hair growth. The diagnosis of black hair follicular dysplasia was made based on the history, alopecia of the pigmented areas, the confinement of abnormalities to dark areas, and the normal unpigmented areas.

• Imaging Science in Dentistry
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• 제41권4호
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• pp.177-181
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• 2011

Pyknodysostosis is a rare autosomal recessive disorder characterized by the post natal onset of short limbs, short stature, and generalized hyperostosis along with acro-osteolysis with sclerosis of the terminal phalanges, a feature that is considered essentially pathognomonic. Other features include persistence of fontanelles, delayed closure of sutures, wormian bones, absence of frontal sinuses, and obtuse mandibular gonial angle with relative mandibular prognathism. We report a case of 17-year-old girl who presented with a chief complaint of retention of deciduous teeth. General physical examination demonstrated short stature, frontal and parietal bossing, depressed nasal bridge, beaked nose, hypoplastic midface, wrinkled skin over the finger tips, and nail abnormalities. Radiographs showed multiple impacted permanent and supernumerary teeth, hypoplastic paranasal sinuses with acro-osteolysis of terminal phalanges, and open fontanelles, and sutures along with wormian bones in the lambdoidal region.