A case of 49 year-old male with dermatomyositis associated with stomach cancer and Sjogren's syndrome is reported. The skin changes were characterized by the presence of Gottron's sign and dusky erythematous and finely scaling macular eruptions with telangiectasias on the scalp, forehead, butterfly area of the face, both elbows and knees. Dryness of eyes and mouth, nuchaI rigidity, numbness on extremities and epigastric hunger pain were also noted. The family history and past history were not contributory. After that weakness on proximal muscles, nuchal rigidity, dysphagia and walking difficulty were more aggravated. On dental and ophthalmologic examinations, shiny appearance of tongue and oral mucosa, burning and dry sensation in the mouth was noted and diminished tear and saliva production was also discovered. On laboratory findings, ESR, serum enzymes, especially CPK 3 and LDH, urinary creatine excretion were increased. LE cell was not found and RA test was also negative. Electromyographic and nerve conduction studies revealed myopathic EMG and normal nerve conduetion in both motor and sensory. There are gastric ulcer, positive vallecular sign on esophagus and thread like narrowing on almost all main and minor ducts of parotid gland. Histologic findings revealed ulcerative adenocarcinoma of stomach, a mild focal infiltration of lymphocytes and degenerative changes in left deltoid muscle and diffuse and extensive dermal edema associated with inflammatory infiltrates and hydropic degeneration and atrophy on the facial skin. He was treated with prednisolone, aspirin and intravenous methotraxate but no favorable effect was noted. Authors reviewed relevant literature.
Adenocarcinoma ; Aspirin ; Atrophy ; Burns ; Butterflies ; Creatine ; Deglutition Disorders ; Deltoid Muscle ; Dermatomyositis* ; Edema ; Elbow ; Esophagus ; Extremities ; Forehead ; Humans ; Hunger ; Hypesthesia ; Knee ; Lymphocytes ; Male ; Middle Aged ; Mouth ; Mouth Mucosa ; Muscle Rigidity ; Muscles ; Neural Conduction ; Neutrophils ; Parotid Gland ; Prednisolone ; Saliva ; Scalp ; Sensation ; Sjogren's Syndrome* ; Skin ; Stomach Neoplasms* ; Stomach Ulcer ; Stomach* ; Tears ; Telangiectasis ; Tongue ; Ulcer ; Walking

Primary leiomyosarcomas are extremely rare. Its clinical configuration is a round to oval, more or less hemispherical elevation of the skin, but the appearance of these lesion is not distinctive so that the diagnosis is established by the histopathologic findings. Microscopically the cutaneous leiornyosarcomas consist of a poorly delineated proliferation of spindle shaped atypical myomatous cells arranged in interlacing fascicles which merge into collagenous stroma. We experienced a case of primary cutaneous leiornyosarcoma which developed as a hen egg sized tumor on the left shin in a 71-year-old woman and present this case with the review of literatures.
Aged ; Collagen ; Diagnosis ; Female ; Humans ; Leiomyosarcoma* ; Ovum ; Skin

Angiotensin-converting enzyme(ACE) is a ipeptidyl carboxypeptidase that is a membrane bound mainly on the luminal surface of pulmonary endothelial capillary cells. It functions to inactivate bradykinin, and also converts angiotensin 1 to angiotensin Activity of ACE was first identified in plasma by Skeggs and co-workers in ]956 In 1974 Lieberman discovered that elevated levels of serum ACE were associated with active sarcoidosis and that this assay would be usei to assist a diagnosis of sarcoidosis. The association of sarcoidosis and enhanced ACE activity was subsequently supported by data from other investigators. Increased serum ACE levels have also been observed in patients with nongranulomatous diseases and granulomatous diseases including leprosy. The author studied the serum ACE levels in leprosy patients(fourty-three with tuberculoid type and eighty-nine with lepromatous type) and twenty normal healthy controls by the spectrophotometric method described by Lieberman. Comparative studies of ACE levels in these two types of leprosy with normal healthy controls and relationship among the duration of treatment, age, and sex were also conducted. The results were summarized as follows: Ages of the selected patients were between 3Q to 77 years in tuberculoid leprosy (average 54 1), 23 to 75 years in lepromatous leprosy(average 53. 8) and 14 to 49 years in the control group(average 28 4) The duration of treatment in tuberculoid leprosy was between 1 and 39 years and average was 2p 7 years. Of lepromatous leprosy, duration of treatment was between 2 and 50 years and the average was 25. 4 years.
Angiotensins* ; Bradykinin ; Capillaries ; Diagnosis ; Humans ; Leprosy* ; Leprosy, Lepromatous ; Leprosy, Tuberculoid ; Membranes ; Peptidyl-Dipeptidase A* ; Phenobarbital ; Plasma ; Research Personnel ; Sarcoidosis

BACKGROUND: Epidermal growth factor receptors (EGFR) have been reported to be absent in melanomas. But recently, the presence of EGFR on melanocytic cells was reported to be a marker of malignant transformation. OBJECTIVE: Our purpose was to investigate the presence of EGFF in melanocytic lesions and to determine whether EGFR presence correlates with the potential or malignant transformation of melanocytic cells. METHODS: We performed the immunohistochemical studies to reveal immunoreactivity of EGFR in 7 compound nevi, 10 intradermal nevi, and four melanomas using the Vectastain ABC immunoperoxidase stain system. RESULTS: Although the intensity of staining was slightly variable, all melanocytic cell types in the studied lesions of compound nevi, intradermal nevi, and melanoms had immunoreactive EGFR. Intense staining far EGFR of all nucleated layers of keratinocytes overlying a melanocytic lesion was also seen. But in the melanoma cells, the staining intensity was modarately deereased. CONCLUSION: Although we found no correlation of EGFR with the potential for malignancy in melanocytic lesions, the high level of expression within nevocytes and lesional keratinocytes suggests EGFR or transforming growth factor a, by acting through the EGFR, plays a role in the pathogenesis, maintenance, or evolution or these lesions.
Epidermal Growth Factor* ; Keratinocytes ; Melanoma ; Nevus ; Nevus, Intradermal ; Receptor, Epidermal Growth Factor* ; Transforming Growth Factors

BACKGROUND: Epidermal growth factor(EGF) usually stimulate she growth and proliferation of a variety of cell types in vitro and in vivo through binding to a peific cell surface receptor, a 170- kilodalton glycoprotein. The EGF receptor (EGFR) may be respansi ile for deranged keratinocyte proliferation and differentiation. OBJECTIVE: Our purpose was to investigate the pattern of EGFR expression in malignant epidermal tumors. METHODS: We performecl immunohistochemical studies to reveal immunoreactivity of EGFR in 7 basal cell carcinomas, 6 squamous cell carcinomas, and five nomal control skin using the Uectastain ABC immunoperoxidase stain system. RESULTS: In normal skin, EGFR showed strong staining of basal cells and lower keratinocytes of the stratum malpighii. As squaous cells matured, staining gradually beame weaker. In all cases of basal cell carcinoma studied, there was loss of membrane labelling of the tumor cells and but in half the cases there was little or no siaining of the lesional cells. In squamous cell carcinomas, variable patterns were seen. The better differentiated tumors showed an essentially no mal pattern of EGFR expression. However, less well differentiated areas showed loss of membrane staining, cytoplasmic accumulation of receptor, and a heterogeneiy of staining intensity. CONCLUSION: Dysregulation of the EGFR may be important in the levelopment, of cutaneous epithelial malignancies but that giossly abnormal forms of the receptor do not occur. The quantitative and qualitative changes in EGFR that we have demonstrated may well be of importance in the pathogenesis of these keratinocyte tumors.
Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Cytoplasm ; Epidermal Growth Factor* ; Glycoproteins ; Keratinocytes ; Membranes ; Receptor, Epidermal Growth Factor* ; Skin

BACKGROUND: Mutations in the p53 gene are the most frecjuent genetic alterations found in human cancers to date. Bvidense suggests that wild-type p53 is a tumor suppressor protein, crucial for the negative regulative of cell cycling, and requiring loss of function mutations for tumorigenesis. OBJECTIVE: Our purposr is to investigate the expression pattern of the p53 protein in the squamous cell carcinomas(SCCs) of the human skin. METHODS: We studied p53 protein expression, using DO7 mnoclonal antibody immunohi-stochemistry, in 29 SCCs of the skin. Also, we compared the p53 expression depending upon with or without a history of UV exposure. RESULTS: p53 immunoreactivity was observed in 48% (14 of 29) of SCCs and was not seen in normal skin. In 56% (16/29) SCCs the tumors were developed on UV-light exposure area. SCCs were divided on histopatal biological criteria in to three categories, well, moderately, or poorly differentiated. Although no significant differenie in the prevalence of p53 immunoreactivity was obierved between these groups, positive, strong staining was observed more frequently in poorly differentiated than in well-differe: treated tumors. CONCLUSION: Accumulat,i,on of p53 protein, suggestive in nessary cases of p53 gene mutation and hence loss of tumors upperesor function, may play a role in the tumorigenesis of SCCs.
Amoxicillin ; Carcinogenesis ; Carcinoma, Squamous Cell* ; Genes, p53 ; Humans* ; Prevalence ; Skin*

A Case of prurigo nodularis in 47-year-old female was presented with brief review of literatures. This is one of relatively rare dermatoses and still has many disputes in etiology and classification. Multiple erythematous brownish hyperkeratotic lichenified nodules were scattered on the back and extremities associated with intolerable itching trouble. Histopathologically, severe hyperkeratosis with focal neutrophile infiltration and pseudoapitheliomatous hyperplasia of acanthotic lesion in the epidermis is presented. Diagnosis was confirmed by characteristic clinical pictures and histopathological features. Treatment with corticosteroids, sediatives and tranquilizers revealed of favorable results.
Adrenal Cortex Hormones ; Classification ; Diagnosis ; Dissent and Disputes ; Epidermis ; Extremities ; Female ; Humans ; Hyperplasia ; Middle Aged ; Neutrophils ; Prurigo* ; Pruritus ; Skin Diseases

A case of incontinentia pigmenti without any developmental defect has been presented. And literatures have been reviewed. The patient was a 20 year old female and had been affected with numerous dark brownish linear streaks of pigmentation on the entirebody. No familiar traits were noted. Diagnosis was confirmed by characteristic clinical picture, laboratory and histopathologic findings.
Diagnosis ; Female ; Humans ; Incontinentia Pigmenti* ; Pigmentation ; Young Adult

PURPOSE: The purpose of this study was to evaluate clinical manifestation, etiology and prognosis of hypocalcemic infants who were admitted with seizure. METHODS: We reviewed medical records of 32 infants admitted at the Asan Medical Center with hypocalcemic seizure retrospectively. We classified patients into vitamin D deficiency group(n=7, 21.9%), transient hypoparathyroidism group(n=4, 12.5%), relative hypoparathyroidism with hyperphosphatemia group(n=16, 50%), and others(n=5, 15.6%) according to the laboratory results. RESULTS: Of the 32 patients, 29 patients were improved. There were no differences in gestational age and birth weight among the three groups. In the vitamin D deficiency group, age of onset was later than those of the transient hypoparathyroidism group and relative hypoparathyroidism with hyperphosphatemia group(51.6+/-2.7 vs 8.3+/-.5, 8.2+/-.6 days). In the age when all laboratory results were normalized, transient hypoparathyroidism group was younger than those of vitamin D deficiency group and relative hypoparathyroidism group(33.2+/-4.6 vs 93.6+/-8.5, 77.1+/-2.4 days). In the total treatment period, relative hypoparathyroidism with hyperphosphatemia group was longer than those of vitamin D deficiency group and transient hypoparathyroidism group(68.9+/-3.5 vs 42.0+/-5.0, 25.0+/-4.3 days). Others included two 22q11.2 deletion syndrome patients, a congenital hypoparathyroidism, a pseudohypoparathyroidism, and an early neonatal hypocalcemia. CONCLUSION: Transient hypoparathyroidism and hyperphosphatemia were major causes of neonatal hypocalcemia. And high calcitonin and peripheral organ resistance to parathyroid hormone act on hypocalcemia. In infants after one month, vitamin D deficiency was also an important cause of hypocalcemia. Most of the patients were improved within 1-2 months after proper management, but relative hypoparathyroidism with hyperphosphatemia group needed longer treatment. So, it is necessary to perform a systematic study for several complex causes that explain above fact.
Birth Weight ; Calcitonin ; Chungcheongnam-do ; DiGeorge Syndrome ; Gestational Age ; Humans ; Hyperphosphatemia ; Hypocalcemia ; Hypoparathyroidism ; Infant* ; Medical Records ; Parathyroid Hormone ; Prognosis ; Pseudohypoparathyroidism ; Retrospective Studies ; Seizures ; Vitamin D Deficiency

BACKGROUND: Recent clinial observations have suggested that colchicine, which is in frequent use in gout can affect the conneciive tissue metabolism in skin and other ti.ues. OBJECTIVE: This study suggest that further development of colchiine might provide a novel means of modulating collagen gen expression in patients with fibrotic disease. METHOD: We examed the effect of colchicine on the expres, on of collagen, fibronectin and collagenase gene by skin filroblast culture hy Nort.hern and dot-blot iybridization. Result : The rate of transcription of genomic DNA corresponding o type I collagen and libvonectin was reduced in colchicine-treated cultures but collagenase was not. reduced. Canclusion : The microti.ikile disruptive agent, colchicine, reduced the expression of type I collagen and fibronectin in a dose-(lependent manner. This st.udy suppor that colchicine is one of the promising antifibrogenic drugs curvently being tested as a treatment, of hun an fibrotic disease.
Colchicine* ; Collagen Type I ; Collagen* ; Collagenases ; DNA ; Fibroblasts ; Fibronectins ; Gene Expression* ; Gout ; Humans ; Metabolism ; Skin