We report a 48-year-old female who had eruptive vellus hair cysts associated with milia on the face. Histopathologic examination of lesions on the forehead and cheek showed typical findings of eruptive vellus hair cysts and milia. These findings suggested that two entities are in some way related. Eruptive vellus hair cysts and milia can be considered as subtypes of multiple pilosebaceous cysts that may all present overlapping histologic features.
Cheek ; Female ; Forehead ; Hair* ; Humans ; Middle Aged

Extramammary Paget's disease is a neoplastic disease, which usually manifests as an erythematous plaque on the genital area. Pigmented extramammary Paget's disease is a rare variant. It should be differentiated from malignant melanoma. We report a case of pigmented extramammary Paget's disease in a 62-year-old male who had a brown-to-black or erythematous-colored plaque on the scrotum and inguinal area. Histopathologic findings showed features typical to extramammary Paget's disease, with proliferation of epidermal melanocytes and dermal melanin pigments.
Humans ; Male ; Melanins ; Melanocytes ; Melanoma ; Middle Aged ; Paget Disease, Extramammary* ; Scrotum

Mauriac syndrome consists of a triad of poorly controlled diabetes, profound growth retardation and hepatomegaly. We experienced a case of Mauriac syndrome in an 18-year-old girl who had poorly controlled diabetes mellitus, short stature, hepatomegaly and central obesity. Also at the time of examination, she had complications of diabetic cataract and nephropathy. Fourteen years prior to admission, she was diagnosed as diabetes mellitus at a hospital. Thereafter, she had been managed with irregular insulin injection. On physical examination at admission, her height was 135cm(<3 percentile) and her weight was 39kg(<3 percetile). She was short and obese. The liver was 5 cm palpable below the right subcostal margin. Her sexual maturation was Tanner stage I. On ophthalmologic examination, the cataracts were observed on both eyes and diabetic retinopathy was absent. Diabetic nephropathy could not be confirrned by kidney biopsy due to her mother's refusal. We studied the hormonal, radiographic and histological abnormalities. The hormonal study was normal and the bone age was by delayed as much as 10 years. The liver biopsy revealed glycogen accumulation in hepatocyte. She was consistent with Mauriac syndrome. She was managed by strict diabetic control with insulin therapy, diabetic diet and intensive education. She was discharged with well controlled blood glucose. Five months later, growth acceleration and sexual maturation have not been observed, but hepatomegaly subsided. (J Korean Pediatr Soc 2000;43-837-841)
Acceleration ; Adolescent ; Biopsy ; Blood Glucose ; Cataract ; Diabetes Mellitus* ; Diet, Diabetic ; Diabetic Nephropathies ; Diabetic Retinopathy ; Disulfiram ; Education ; Female ; Glycogen ; Hepatocytes ; Hepatomegaly ; Humans ; Insulin ; Kidney ; Liver ; Obesity, Abdominal ; Physical Examination ; Sexual Maturation

BACKGROUND: Skin organ culture is widely used as a tool to investigate skin biology or skin disease. OBJECTIVE: The objective of the present study was to develop an ideal skin organ culture model for evaluation of melanin pigmentation. METHODS: An air-liquid interface and submerged method were used. The histology of the cultured skin was studied with H&E stain. To examine the epidermal pigmentation, Fontana-Masson stain and NKI/beteb stain were performed. Pigment modifiers (arbutin, LY294002) were applied to the culture medium for 3 days as an air-liquid interface culture. RESULTS: The general architecture of the skin was well maintained for 5 days. The melanin pigment decreased during culture without change of the number of melanocytes. As expected from previous reports, the effect of pigment modifiers (arbutin, LY294002) on cultured skin was demonstrated. CONCLUSION: The results indicate that this skin organ culture model is useful in evaluating the melanin pigmentation
Biology ; Melanins* ; Melanocytes ; Organ Culture Techniques* ; Pigmentation* ; Skin Diseases ; Skin*

Lichen planus and vitiligo are common skin disorders, which rarely coexist. We report a case of colocalization of lichen planus and vitiligo in a 42-year-old woman. Furthermore, herein, we discuss the possible mechanism of colocalization of lichen planus and vitiligo, including the role of actinic damage in the initiation of lymphocytic infiltrates of lichen planus in vitiliginous skin.
Actins ; Adult ; Female ; Humans ; Lichen Planus* ; Lichens* ; Skin ; Vitiligo*

BACKGROUND: There are evidences that uPA and its inhibitor play a key role in tumor spread. We studied whether uPA and PAI-1 expressions could serve as prognostic parameters along with clinical, gross and microscopic findings in gallbladder carcinomas. METHODS: We analyzed 42 cases of gallbladder carcinomas by immunohistochemical staining and clinicopathologic parameters. RESULTS: uPA and PAI-1 were more frequently expressed in the adenocarcinoma than in the normal or benign gallbladder tissue. The uPA expression in the glands of low grade adenocarcinoma was significantly correlated with both distant and lymph node metastases. The uPA expression in the stroma around the low grade adenocarcinoma was significantly correlated with either distant or lymph node metastasis. The PAI-1 expression was significantly correlated with lymph node metastasis only for both distant and lymph node metastases. In multivariate analysis, the lymphatic invasion was significantly related to poor survival (p= 0.0115). In univariate analysis, the cases without lymphatic invasion had prolonged survival. Positive expression of uPA in the glands of low-grade adenocarcinoma was significantly correlated with poor survival (p=0.0391). CONCLUSION: In conjunction with clinicopathologic findings, expressions of uPA and PAI-1 may be useful prognostic markers in gallbladder carcinomas.
Adenocarcinoma ; Gallbladder* ; Lymph Nodes ; Multivariate Analysis ; Neoplasm Metastasis ; Plasminogen Activator Inhibitor 1 ; Plasminogen Activators* ; Plasminogen* ; Prognosis ; Urokinase-Type Plasminogen Activator*

Cerebral aspergillosis is a rare condition, and like other opportunistic fungal infections, it most commonly occurs in immunocompromised patients. Because of the increasing use of chemotherapy in organ transplanta-tion, cases involving neoplasms, corticosteroid therapy, and cases of lymphoma and leukemia, the incidence of fungal infections in the brain has recently increased. Cerebral aspergillosis in an infant with normal immunity is a very rare condition, and has not been reported in Korea. We report the MR findings of this condition in an infant with normal immunity.
Aspergillosis* ; Brain ; Drug Therapy ; Humans ; Immunocompromised Host ; Incidence ; Infant* ; Korea ; Leukemia ; Lymphoma ; Magnetic Resonance Imaging*

Pediatric patients with dissecting aneurysms usually present with ischemia rather than bleeding. We report a case of a 15-year-old boy with a dissecting aneurysm of the posterior cerebral artery (PCA) presenting with hemorrhage. He was first treated with stent-assisted coil embolization, in an attempt to avoid trapping of the PCA and preserve the perforators. After the procedure, he recovered well from general anesthesia, but rebleeding occurred from the same lesion 6 hours after the procedure, therefore endovascular segmental occlusion of the parent artery was performed secondarily. Apparently, a reconstructive method of stent-assisted coiling is worth trying to preserve the parent vessel and perforators, but it is not always efficient and durable for dissecting aneurysms.
Adolescent ; Anesthesia, General ; Aneurysm, Dissecting ; Arteries ; Child ; Glycosaminoglycans ; Hemorrhage ; Humans ; Ischemia ; Parents ; Passive Cutaneous Anaphylaxis ; Posterior Cerebral Artery ; Subarachnoid Hemorrhage

When dysfunction of two or more endocrine glands occurs in association with circulating organ specific antibodies directed against the involved glands, the term polyglandular autoimmune (PGA) syndrome is applied. This syndrome is usually classified into three groups. The autoimmune nature of this disease has been based on the presence of lymphocytic infiltration of the affected glands, organ specific autoantibody in serum, cellular immune defects and association with HLA DR/DQ genes. A 12-year-old girl developed PGA syndrome, type III manifesting Grave's disease and insulin-dependent diabetes mellitus. The thyroid microsomal Ab, TSH receptor Ab and pancreatic islet cell Ab were positive. She should be observed for the possible development of adrenal insufficiency and/or other autoimmune disease.
Adrenal Insufficiency ; Antibodies ; Autoimmune Diseases ; Child ; Diabetes Mellitus, Type 1 ; Endocrine Glands ; Female ; Humans ; Islets of Langerhans ; Receptors, Thyrotropin ; Thyroid Gland