Down syndrome is the most, well known autosomal trisomy and e large number of abnormal skin features have been reported to occur in patients with Down syndromir. including syringoma. Syringomas occur with increased frequency in patients with Down syringoma, usually limited to the region around the eyes. but few cases of eruptive syringoma with Down syndrome have been reported. We reported the case of familial eruptive syringoma in a 13 years old patient with Down syndrome.
Adolescent ; Down Syndrome* ; Humans ; Livedo Reticularis ; Skin ; Syringoma* ; Trisomy

Skin necrosis is a rare complication of heparin administration that is usually localized to injection sites. We report a case of skin necrosis that was caused by minidose intraarterial infusion of porcine heparin which had been used in a touch to prevent coagulation in percutaneous intraarterial cannula. The skin necrosis appeared 35 days after starting heparin use.
Catheters ; Heparin* ; Infusions, Intra-Arterial ; Necrosis* ; Skin

Livedo vasculitis clinically shows purpuric papules and recurrent ulcers in the lower extremities, mainly on the ankles, leaving characteristic scars called atrophie blanche after the healing of the ulcers. Its characteristic histopathologic features and clinical evolution indicate that the common pathologic event is occlusion of vessels in the middle and deep dermis. In Korean literature, seven cases of this disease have been reported but the response of the treatment was not, satisfactory. We report three cases of livedo vasculitis cleared by combined ther by of acetylsalicylic acid, dipyridamole and nifedipine, which has not been reported in Korean literatur.
Ankle ; Aspirin* ; Cicatrix ; Dermis ; Dipyridamole* ; Lower Extremity ; Nifedipine* ; Ulcer ; Vasculitis*

Hydroa Vacciniforme is a rare, chronic, photosensitivity disorder witli onset in childhood that is characterized by recurrent, discrete vesiculation, necrosis and vacciniform scarring limited to sun exposed skin. We report a case of hydroa vacciniforme associated with ocular inwlvment in which vesicles could be induced only with single MED exposure to UVA.
Cicatrix ; Hydroa Vacciniforme* ; Necrosis ; Photosensitivity Disorders ; Skin ; Solar System

A 38-year-old male patient visited our clinic complaining of three skin lesions on the scalp. There were yellowish to brownish, waxy, non-tender, walnut-sized nodules. Hematoxylin and eosin staining revealed amorphous pinkish material deposits in the dermis. The Congo red stain and Dylon stain under polarizing microscopy showed yellow-green birefringence and the immunoglobulin-lambda light chain stain showed a positive reaction. An electron microscopic examination revealed filaments with uniform diameter(6 to 10nm) that were straight and neither branched nor anastomosed. Based on the clinical, histopathological, immunohistochemical and electron microscopical findings, the skin lesions were diagnosed as nodular amyloidosis.
Adult ; Amyloidosis* ; Birefringence ; Congo Red ; Dermis ; Eosine Yellowish-(YS) ; Hematoxylin ; Humans ; Male ; Microscopy ; Scalp ; Skin

Trichilemmal carcinoma is a rare tumor that is located predominantly on sun-exposed skin of the elderly. It is thought to be related to the external root sheath of the hair follicle and the malignant counterpart of a trichilemmoma. We report a case of trichilemmal carcinoma in a 88-year-old woman who had a 10 year history of a single, dark brown tumor on the right preauricular region. In her past medical history, it had been excised twice at a private clinic without a histopathological evaluation and recurred 2 years later. Histopathological findings showed atypical clear cells resembling those of the outer root sheath. Tumor cells showed PAS-positive cytoplasm. Immunohistochemistry revealed positive high molecular weight cytokeratin expression. To our knowledge, this is the second case of trichilemmal carcinoma in the Korean literature. (Ann Dermatol 100) 175178, 1998).
Aged ; Aged, 80 and over ; Cytoplasm ; Female ; Hair Follicle ; Humans ; Immunohistochemistry ; Keratins ; Molecular Weight ; Skin

BACKGROUND: The high level of susceptibility of patients with liver cirrhosis (LC) to septicemic infection is thought to be due to decreased reticuloendothelial function and irnpairment of several components of cell mediated and humoral immunity. It is well known that LC may cause many characteristic skin changes including jaundice, spider angioma, palmar erythema, xanthelasrna and nail changes such as clubbing and white nails. OBJECTIVE AND METHODS: During a 7 month period frorn April to October 1995, 100 patients who had been diagnosed as having LC were evaluated for the duration of their LC, the associated diseases they had apart from LC and various skin manifestations. Also, we performed the tuberculin test in 20 patients with LC and evaluated the correlation of the tuberculin test with severity of LC divided into 3 groups according to the Childs classification. RESULTS: The results are summarized as follows ; 1. The most frequent duration of LC was under 5 years (74%). 2. The most common associated disease of patients with LC was upper GI bleeding including esophageal varix (24 cases). Also, diabetes mellitus (13 cases) and hepatic coma (11 cases) were observed. 3. Jaundice (82 cases) was the most frequent skin rnanifestation of patients with LC. In addition, vascular changes (81 cases) and various nail changes (69 cases) were observed. Onychomychosis was the most common nail change of patients with LC. 4. 60% of the patients who were given the tuberculin test showed anergy, and the frequency of anergy increased in proport on to the severity of LC. However, it was not significant statistically (p> 0.05 ). CONCLUSIONS: Some of the skin manifestations help in the diagnosis of LC. Also, increased frequency of tuberculin test anergy in proportion to the severity of LC is thought to be related to decreased cell mediated immunity, although statistically it is not significant.
Child ; Classification ; Diabetes Mellitus ; Diagnosis ; Erythema ; Esophageal and Gastric Varices ; Hemangioma ; Hemorrhage ; Hepatic Encephalopathy ; Humans ; Immunity, Cellular ; Immunity, Humoral ; Jaundice ; Liver Cirrhosis* ; Liver* ; Skin Manifestations* ; Skin* ; Spiders ; Tuberculin Test

No abstract available.

BACKGROUND: To determine whether N-acetylcysteine(NAC) reduces methemoglobin. METHOD: We carried out an in vivo experiment in rats, weighed about 300g. Each rat was ingested 200mg of dapsone(4,4'diaminodiphenyl sulphone) to induce methemo- globinemia. After 1 hour, 30 rats were received NAC 160mg(2ml) and another 30 rats, served as control, were received 2ml of normal saline orally,4 times hourly. Serum methemoglobin concentrations were checked 1,6, and 24 hours after dapsone ingestion. RESULT: The methemoglobin concentrations on each time were 27.1+/-5.8%,23.5+/-8.8%, 17.1+/-6.4%(mean+/-SD) in control group and 25.5+/-6.3%, 25.5+/-8.8%, 65.5+/-31.2%(mean+/-SD) in NAC group. There were no differences on methemoglobin concentrations at 1 and 6 hours between two groups(P>.05). At 24 hours, the methemoglobin concentrations of NAC group was significantly higher than those of control group(P<.01). CONCLUSION: NAC had no therapeutic effects on dapsone induced methemoglobinemia in this experimental setting.
Acetylcysteine* ; Animals ; Dapsone ; Eating ; Methemoglobin ; Methemoglobinemia* ; Rats

Infantile myofibromatosis is an uncornmon, benign, self-limiting, localized or generalized process, probably of hamartomatous origin, which consists to a large degree of cells having the characteristics of myofibroblasts and sometimes of pericytes. Both solitary and multicentric forms occur. Most lesions are present at birth or in early infancy, and some are familial in origin. A female newborn presented with a firm, round, red colored, 3 x 3 cm sized tumor with central necrosis on the left chest. Histological examination revealed well-circumscribed nodules consisting of short bundles of plump, spindle shaped cells displaying staining characteristics intermediate between fibroblasts and smooth muscle cells. The tumor was immunoreactive for actin but did not stain for desmin. A Follow-up examination at the age of 3 months revealed a moderate degree of spontaneous regressior of the lesion.
Actins ; Desmin ; Female ; Fibroblasts ; Follow-Up Studies ; Humans ; Infant, Newborn ; Myocytes, Smooth Muscle ; Myofibroblasts ; Myofibromatosis* ; Necrosis ; Parturition ; Pericytes ; Thorax