No abstract available.

Incontinentia pigmenti is an uncommon genodermatosis. It affects female infants predominantly, described first by Bardach in l925, with the diagnosis of systematized nevus and Bloch in 1926, and Sulzberger in 1928 rnore compIetely. Skin lesions are characterized by 3 stages such as vesicobullous, verucous and finally pigmentary lesions and leave brownish pigmentation on the extremities and trunk. Hesides skin lesions some ectodermaI and mesodermal organs are affected and show developmental abnormalities. We experienced a case of incontinentia pigmenti in a 45-day-old female infant and present it with the review of literature. She showed extensive vesicobullopustular eruption with linear and reticular pigmentation on the extremities and trunk. Clinical and histopathologic findings of these lesions are compatible with Bloch-Sulzberger type of incontinentia pigmenti.
Diagnosis ; Extremities ; Female ; Humans ; Incontinentia Pigmenti* ; Infant ; Mesoderm ; Nevus ; Pigmentation ; Skin

BACKGROUND: Calcinosis cutis may occur when connective tissue is abnormal (dystrophic), or where calcium or phosphate levels in the blood are high(metastatic); alternatively, there may be no obvious underlying cause(idiopathic). The exact incidence of calcinosis cutis in dermatologic patients is not well-kniown and the pathomechanism of it remains to be elucidated. OBJECTIVE: This study was performed to analyze the incidence, clinical and histopathological features of calcinosis cutis. METHODS: We studied 72 patients with calcinosis cutis who visited our department between January 1985 and December 1996. The patients were classified into 3 types (dystrophic, metastatic, and idiopathic) and were analyzed clinically and histopathologically. Results : The results were summerized as follows; 1. There were 60 cases(83.3%) and 12 cases(16.7%) of the dystrophic and idiopathic types, respectively but the metastatic type was not observed. The frequency of dystrophic calcification in individual disorders was 88.2% in pseudoxanthoma elasticum, 66.7% in trichilemmal cyst and dermatomyositis, and 64.6% in pilomatrichoma. 2. In the idiopathic type, it was common in females aged over 50 years and the mean duration of lesions was 2.8+/-2.0 years. The predilection site was the flank(5 cases) and most of the lesions(7 cases) showed skin-colored nodules 3. Histopathologically, calcified material of the idiopathic type was located predominently in the dermis(10 cases) and was present as large masses surrounded by a foreign body reaction. 4. In the dystrophic type, calcified deposits were noted predominently within the shadow cells of pilomatricoma, in the degenerated elastic fibers of pseudoxanthoma elasticum, and in the keratinized area of basal cell carcinoma, squamous cell carcinoma, trichilemmal cyst, and epidermal cyst. Foreign body reactions with giant cells and mononuclear cell infiltrations were often found around large deposits of calcium. Conclusion : The incidence of calcinosis cutis in dermatologic patients is relatively low, but a variety of disorders can be associated with cutaneous calcification. Therefore, dermatologists should be familiar with the different forms of cutaneous calcification and the dermatoses that manifest them.
Calcinosis* ; Calcium ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Connective Tissue ; Dermatomyositis ; Elastic Tissue ; Epidermal Cyst ; Female ; Foreign Bodies ; Foreign-Body Reaction ; Giant Cells ; Humans ; Incidence ; Pilomatrixoma ; Pseudoxanthoma Elasticum ; Skin Diseases

Multiple endocrine neoplasi~a type 3 is a rare, heritable or sporadic, multiple neoplastic disorder that is charracterized by thyroid medullary careinoma, pheochromocytoma, multiple mucosal neuroma, and marfanoid habitus. The most important disorder of the syndrome is mucosal neuroma that is an early diagnostic sign of multiple endocrine neoplasia type 3. Early diagnosis of MEN type 3 determines prognosis of the disease. We present a case of multiple endocrine neoplasia type 3 of the sporadic pattern in a 27-year-old man who had typical medullary thyroid carcinoma, mucosal neuroma, marfanoid habitus, and megacolon.
Adult ; Early Diagnosis ; Humans ; Male ; Megacolon ; Multiple Endocrine Neoplasia* ; Neuroma* ; Pheochromocytoma ; Prognosis ; Thyroid Gland ; Thyroid Neoplasms

BACKGROUND: Evaluating the segmental wall motion of left ventricle is important in patients with myocardial infarction for choosing therapeutic modality and predicting prognosis. Radionuclide Multigated Angiography(MUGA) is a reliable noninvasive method for the evaluation of left ventricular performance. Methods : MUGA scan(LV ejection fraction, phase image histogram, regional wall motion) was performed and analyzed in 45 patients with myocardial infarction(31 : acute MI, 14: old MI) and 13 normal controls. RESULTS: 1) The LVEF of acute and old MI group was significantly reduced and the SDph of acute and old MI group was significantly increased as compared with that of control group(p<0.05). 2) In acute MI group, the LVEF of group without, IV Urokinase was more reduced than that of group with IV Urokinase and the SDph of group without IV Urokinase was more increased than that of group with IV Urokinase(p<0.05). As a result of wall motion scoring, the linear correlation exists between SDPh and sum of wall motion scoring(r=0.62, p<0.01). 3) In MI group, the LVEF of anterior wall MI was more reduced than that of inferior wall MI and the SDPh of anterior wall MI was more increased than that of inferior wall MI(p<0.05). 4) In acute anterior wall MI, the reverse correlation exists between LVEF and SDPh and the linear correlation exists between sum of wall motion scoring and SDPh(r=-0.73, 0.72, p<0.01). But there are no statistical significances of correlation between them in acute inferior MI(r=-0.44, 0.42, p>0.05), in old anterior MI(r=-0.65, 0.47, p>0.05) and in old inferior MI(r=-0.47, 0.46, P>0.05). CONCLUSION: These results suggest that Phase angle(SDPh) is thought to be valuable index to evaluate left ventricular function with application of other indeces in Myocardial infarction. Left ventricular function measured by SDph in acute or anterior MI is lower than old or inferior MI.
Heart Ventricles ; Humans ; Myocardial Infarction* ; Prognosis ; Urokinase-Type Plasminogen Activator ; Ventricular Function, Left

A 26-year-old Korean man had an erythematous scaly plaque on his right thigh for fifteen years. He was diagnosed with chronic eczema and treated with topical steroid, but no clinical improvement was obtained for 15 years. Laboratory studies including complete blood cell count, urinalysis, liver and renal function test, and peripheral blood smear were normal. A biopsy specimen showed hyperkeratosis, acanthosis, and mononuclear cellular infiltration in the epidermis. These cells had a perinuclear halo and showed a Pautrier's microabscess like configuration. In the dermis, there was a band like infiltration of inflammatory cells. Infiltrates of the epidermis were negative for leukocyte common antigen(CD45) and positive for Pan T cell(CD45RO). He was treated with topical PUVA twice a week for twelve weeks. Clinical and pathological improvements were obtained. We propose PUVA may be a useful therapeutic modality to treat Woringer Kolopp disease.
Adult ; Biopsy ; Blood Cell Count ; Dermis ; Eczema ; Epidermis ; Humans ; Leukocytes ; Liver ; Pagetoid Reticulosis* ; Thigh ; Urinalysis

We report the clinical evolution of two male patients with generalized granulama annulare(GGA) who were controlled with hydroxychloroquine. In the first case, a 3-year-old boy was treated with systemic steroid, and in the second case, a 68-year-old man was treated with prednisolone, niacin, and retinoic acid. But, remission and recurrence of the skin lesions were repeated. A good clinieal response was achieved in both cases after administration of hydroxychloroquine without side effects or any signs of recurrence. Hydroxychloroquine may be helpful to treat the GGA that have side effects to the systemic corticosteroid or resistence to other therapeutic modalities.
Aged ; Child, Preschool ; Granuloma Annulare* ; Granuloma* ; Humans ; Hydroxychloroquine* ; Male ; Niacin ; Prednisolone ; Recurrence ; Skin ; Tretinoin

This study was performed to investigate the effect of octreotide on the contractility of rat vas deferens. The -smooth muscle strips isolated from the prostatic portion were myographied in isolated organ bath. Electric -field stimulation (monophasic square wave, duration : 1. mSec, voltage : 50 V, frequency : 5 Hz or 30 Hz, train : 10 Sec) produced reproducible contraction. The contraction was composed of two component, first phasic component (FPC) and second tonicc component (STC).. These contractions were abolished by -tetrodotoxin (1 microM). Octreotide inhibited the field stimulation induced contractions both FPC and STC concentration- dependently. The FPC was decreased by a desentization of purinergic receptor by pretreatment of mATP, and the STC was decreased by pr,,creatment of reserpine (3 mg/kg, EP) 24 hours before experiments. Octreotide reduced the field stimulation induced contraction in the presence of mATP and of reserpinized muscle strips. The inhibitory effect of octreotide was more potent at 5 Hz than at 30 Hz. Octreotide did not affect basal ton and exogenous norepinephrine- or ATP-induced contraction. These results suggest that octreotide inhibit the contractility of the isolated rat vas deferens by inhibition of the release of neurotransmitters, both ATP and norepinephrine from adrenergic nerve terminal.
Adenosine Triphosphate ; Animals ; Baths ; Neurotransmitter Agents ; Norepinephrine ; Octreotide* ; Rats* ; Reserpine ; Vas Deferens*

No Abstract Available.
Hyperpigmentation*

The prominent mandibular angle is one of the disfiguring features in the Oriental, commonly seen and considered to be unattractive. Because it gives a square and muscular facial appearance, its surgical correction is dine frequently as a modality of facial contouring surgery in the Oriental. The reduction mandibular angleplasty is not a simple surgical technique for the unexperienced surgeon. We adopted C-arm fluoroscopy as a method of identifying the osteotomy line for the beginners. The reduction mandibular angleplasties assisted by C-arm fluoroscopy were performed in 9 patients, providing symmetric and satisfactory results. Now we are sure that the reduction mandibular angleplasty assisted by C-arm fluoroscopy can be an accurate and safe method for the unexperienced surgeon, especially the patient with deep-seated prominent mandibular angle.
Fluoroscopy* ; Humans ; Osteotomy