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

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

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

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.

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: 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

Sweet's syndrome is an important cutaneous sign of underlying myeloproliferative disorder. The majority of cases have occurred with acute leukemia, primarily of the myelogenous type. We described a case of Sweet's syndrome in a patient with myelodysplastic syndrome that preceded acute myelogenous leukemia by 9 months.
Humans ; Leukemia ; Leukemia, Myeloid, Acute* ; Myelodysplastic Syndromes* ; Myeloproliferative Disorders ; Sweet Syndrome*

BACKGROUND: Generally, there are few problems in the diagnosis of Paget's disease(PD) using the H&E stain. However, the differentiation of PD from the clonal type of Bowens disease and superficial spreading melanoma in situ that shows pagetoid spreading of tumor cells, may present diagnostic difficulties. In addition, the specia1 stains used for demonstrating the presenee of Pagets cells, such as PAS and mucicarmim, are non-specific and not always sensitive. So, inenunohistochemical stains with monoclonal antibodies against various antigens may be helpful for differentiating PD from ather morphologically similar skin lesions. OBJECTIVE: The purpose of this study was to investigate the diagnostic accuracy of immunohisto- chemical staining for diagnostic use in PD. METHODS: Immunohistochemical stains used in the biotin streptavidin amplificxl technique with monoclonal antibodies to several low rnolecular weight cytokeratin(CK)s, EMA and CEA, were performed on formalin-fixed, paraffin-embedded tissue. Twelve cases of PD(10 cases of extranmmmary PD and 2 cases of mammary PD), five cases of superficial spreading melanoma in situ and five cases of Bowens disease were investigated. RESULTS: The results were as follows. 1. Positive reactions with variable intensity using CK7, CKS, CK19 were seen in all cases(100%) of PD and the. staining intensity tor CK7 or CK19 was stronger than that of CKS. 2. Of the 12 cases of PD, both CK18 and CAM5.2 staining showed positivity in 11 cases(92%). 3. EMA and CEA staining showed positivity in 10(83%) and 9(75%) of 12 cases, respectively. 4. Some Pagets cells were negative for CK8, CK18 and EMA, although other positive cells were observed in the same sections. 5. All antigens were consistently negative in all cases of Bowens disease and superficial spreading melanoma in situ. CONCLUSION: The results show that moaoclonal antibodies to low molecular weight CKs are more sensitive than EMA or CEA in the demonstration of Pagets cells. Moreover, among the low molecular weight CK series, CK7 and CK19 are most useful for their high sensitivity and intensity.
Antibodies ; Antibodies, Monoclonal ; Biotin ; Bowen's Disease ; Coloring Agents ; Diagnosis ; Melanoma ; Molecular Weight ; Skin ; Streptavidin