A Case of Sjögren-Larsson Syndrome

Sjögren-Larsson syndrome(SLS) is a rare hereditable disease characterized by congenital ichthyosis, spastic diplegia and mental retardation. Along with the typical triad of symptoms, many patients with this disease have short stature, kyphosis and glistening dots in the retina of the eye. The pathogenesism is unknown but recent studies suggest that SLS might be, at least in part, a disorder of fatty acid metabolism. We describe a patient with a pathognomonic finding in the fundus and with the classic features of SLS.

Chemical Peeling-A Histological Study on The Skin of Guinea Pigs

BACKGROUND: It is a recent trend in Korea that the uses of trichloroacetic acid(TCA) have been increased as the usual chemical peeling agent for treating abnormal pigmentation like solar lentigo, freckle, melasma, or actinic keratoses, rhytides, acne scar and other facial problems. We made an experiment to see how the different concentrations of TCA affect the guinea pig skin histologically. OBJECTIVE: The purpose of this study was to evaluate the histologic changes using various concentrations(35%, 50%, 90%) of TCA. METHODS: At first, 35%, 50% or 90% of TCA was applied to the each right ear of guinea pigs. After the application, we checked these specimens in intervals - one hour after (D1hr), the following day(D1), the third(D3), fourth(D4), fifth(D5) day, one week(D1wk), two weeks(D2wks), six-teen weeks(D16wks) later. The specimens were processed and stained with hematoxylin and eosin (H & E), Verhoeff elasic, and alcian blue stain. RESULTS: 1. In Do1hr of 35% and 50% TCA treated groups, some keratinocytes with eosinophilic cytoplasm and pyknotic nuclei were scattered in the epidermis. The higher the concentration of TCA, the more evidence of homogenization of the epidermis. The homogenization of epidermis and dermis was most prominent in 90%. 2. In D1 and D3, necrosis of the epidermis was proportional to the concentrations of TCA. We could see infiltrates of inflammatory cells in the whole dermis-when 90% of TCA was tried. 3. Desquamation of the necrotic epidermis appeared in each group between 3 and 7 days after the treatment. 4. In groups treated with 50% and 90% TCA, reepithelialization arising from skin appendages was prominent. 5. Hyperplasia of epidermis appeared in every group between the second and sixteenth weeks but dermal hyperplasia was prominent only in the group of 90% TCA at sixteen weeks. CONCLUSION: While thirty-five per cent TCA solution produced partial destruction of the epidermis, 50% and 90% of TCA destroyed the entire epidermis and upper dermis. Regeneration of epidermis from skin appendage is a signficant base of deep chemical peeling. Epidermal hyperplasia was found in 50% and 90% of TCA, but dermal hyperplasia was only found in 90% of TCA.

Three Cases of Nevus Lipomatosus Cutaneous Superficialis / 대한피부과학회지

Nevus lipomatosus cutaneous superficialis(NLCS) is a rare nevoiri anomaly characterized by ectopic adipose tissue in the dermis without predilection. In this disease two clinical types are distinguished: The classic type consists of asymptomatic linear or zosteriform coliection of soft, flesh colored to yellowish, papules or nodules that often coalesce into plaque. It is usually located in the pelvic girdle and lumbar area but has been repeted on the abdomen, thorax, thigh, and scalp. The solitary form usually appears later in life and can occur at any site. There is no sexurl irredilection or hereditary trend. There are no assoeiated abnor malities. The authors experienced three cases of typical NLCS including thia silitary type that had several solitary nodules irregularly. The first case was a 24-year-old female which has had multiple, flesh to dark brown-colored, confluent nodules over the left upper thigh for 5-years. The second case is the solitary type that a 56-year-old female has had discrete bean sized to what sized nodules on the right buttock for 13-years. The third case was a 23-year-old female who had soft, yellowish skin colored, cerebriform tumor on the right buttock for 10-yesrs. Diagnos was confirmed by clinical and histopathologic findings.

Atypical Dermatoglyphics in Trisomy 18 (Edwards Syndrome)

A 19 month old girl with trisomy 18 is described. She showed loose folds of skin about the neck, a prominent occiput, a simian crease on both palms, epicanthal folds, acrocephaly, micrognathia, and unusual dermatologic features including total alopecia and no finger prints on either hands. Because of the simian crease on both palms, dermatoglyphics of both hands and total alopecia, a skin biopsy from the scalp and a chromosomal study were established at age 19-months, and an absence of hair follicles was observed, while peripheral blood lymphocytes demonstrated 47, XX, 4-18. To our knowledge, this would be the first recorded report on the dermatoglyphic pattern of Edwards syndrome in a Korean journal of dermatology.

Atypical Dermatoglyphics in Trisomy 18 (Edwards Syndrome)

A 19 month old girl with trisomy 18 is described. She showed loose folds of skin about the neck, a prominent occiput, a simian crease on both palms, epicanthal folds, acrocephaly, micrognathia, and unusual dermatologic features including total alopecia and no finger prints on either hands. Because of the simian crease on both palms, dermatoglyphics of both hands and total alopecia, a skin biopsy from the scalp and a chromosomal study were established at age 19-months, and an absence of hair follicles was observed, while peripheral blood lymphocytes demonstrated 47, XX, 4-18. To our knowledge, this would be the first recorded report on the dermatoglyphic pattern of Edwards syndrome in a Korean journal of dermatology.

A case of lymphangioma circumscriptum with cystic hygroma / 대한피부과학회지

Lymphangiomas and cystic hygromas are congenital malformatiors of the lymphatic system. They are composed of lymph-containing endothelium-lined spaces which very in size from channels of capillary dimensions to cysts several centimeters in diameter. Both types of lesions are rarely present, in the same patients. We report herein a case of lymphangioma circumscriptum with cystic hygroma on the tongue and the right side of the neck in a 5 year-old boy. The patient had a grop of deep-seated, small, crystal-line vesicles resembling frogs spawn that contained a viscous liquid with hemorrhage in the tongue and a mobile non-tender cystic swelling in the right side of the neck.