The hyperimmunoglobulinemia E (Jobs) syndrome (HIES) is characterized by marked elevated levels of IgE, recurrent cutaneous and systemic staphylococcal infections, atopic-like dermatitis, and defective neutrophil chemotaxis. Three cases of HIES have been reported in Korea, but not in the dermatology literature. We report a case of HIES with cutaneous infections and MRSA (methicillin-resistant Staphylococcus aureus). A 15-month-old girl presented with intractable pruritic excoriated papular pustular skin lesions and multiple subcutaneous abscesses. Surgical drainage of the abscesses and a course of antibiotic treatment in addition to topical steroids for about 7 weeks resulted in a remarkable improvement.
Abscess ; Chemotaxis ; Dermatitis ; Dermatology ; Drainage ; Female ; Humans ; Hypergammaglobulinemia* ; Immunoglobulin E ; Infant ; Job Syndrome* ; Korea ; Methicillin-Resistant Staphylococcus aureus ; Neutrophils ; Skin ; Staphylococcal Infections ; Staphylococcus ; Steroids

BACKGROUND: Cyclosporin A (CsA), a potent immunosuppressant, has been reported to be effective in the treatment of severe atopic dermatitis (AD). OBJECTIVE: The aim of this study was to evaluate the efficacy and side-effects of CsA in Korean patients with severe AD. MATERIALS AND METHODS: 16 patients with recalcitrant AD took CsA for at least 6 weeks. Among them, 11 patients were followed up for more than 16 weeks. Initial dose was 5mg/kg/day (maximum 300 mg/day) and the dose was reduced according to their therapeutic responses. SCORAD (Scoring AD) was used to evaluate clinical efficacy of CsA. During the 1st month of therapy, the therapeutic efficacy and side-effects were evaluated every 2weeks and after 1 month, every month. We checked blood pressure and laboratory abnormalities including liver function test, blood urea nitrogen (BUN), creatinine (Cr) and urinalysis at each visit in addition to observing clinical adverse effects. RESULTS: Significant reduction of SCORAD was noted in 15 patients after 6 weeks of CsA therapy. Only one patient stopped CsA therapy because of the elevation of blood pressure. Three patients showed albuminuria, which disappeared after CsA dose reduction. CONCLUSION: CsA can be used effectively and safely in severe Korean AD patients. Albuminuria seems to be a peculiar side-effect in Korean patients.
Albuminuria ; Blood Pressure ; Blood Urea Nitrogen ; Creatinine ; Cyclosporine* ; Dermatitis, Atopic* ; Humans ; Liver Function Tests ; Urinalysis

The authors analyzed 30 patients whose celiac artery or its branches were dissected during the procedure of transcatheter arterial embolization (TAE) for hepatocellular carcinoma (HCC) and were followed up angiographically. The incidence of arterial dissection was 1.25%. The dissection occurred most frequently in the celiac artery (40% 12/30) and the proper hepatic artery(28% 7/30). The frequency of arterial dissection was affected by the status of the vessel and the experience of the operator. The follow-up angiography revealed complete recanalization in 40% (12/30), irregularity and narrowing of the lumen in 23% (7/30), pseudoaneurysm formation in 23%(7/30), and complete obstruction in 13% (4/30) of the cases. The rate of recanalization in the celiac artery was lower than that of any other arteries in our series. Recamalization to a certain degree without therapeutic intervention was observed in 72% of the dissected arteries enabling and contributing to subsequent successful TAE within 2 months in 78% of the patients with the dissection.
Aneurysm, False ; Angiography ; Arteries ; Carcinoma, Hepatocellular ; Celiac Artery* ; Follow-Up Studies* ; Humans ; Incidence

Although ultrasound has not been used to clarify bone lesions because of their high acoustic impendence and attenuation coefficient, aneurysmal bone cyst of the pelvis was imaged with ultrasound. Dual fluid levels and multiple loculations were discovered by ultrasound imaging through thin cortical bone. Ultrasonography is a simple, non-invasive method which can be utilized to detect bony lesions of an expansile nature and reveal characteric findings in an aneurysmal bone cyst.
Adolescent ; *Blood ; Bone Cysts/*diagnosis/surgery ; Case Report ; Female ; Human ; *Ilium/pathology/surgery ; Tomography, X-Ray Computed

BACKGROUND: Thymopentin(TP-5) is an immunomodulatory agent which may be a promising new drug in the treatment of patients with severe atopic dermatitis. OBJECTIVE: The aim of this study was to evaluate the efficacy of thymopentin for treatment of severe atopic dermatitis. MATERIAL AND METHOD: Fifteen patients with severe atopic dermatitis received subcutaneous injections of 50mg thymopentin three times per week for 6 weeks. Clinical extent and severity parameters were assessed at baseline, at regular intervals during therapy and 4 weeks posttherapy. Use of antihistamine and topical steroid were permitted. RESULT: Significant reduction in severity scores and body surface area involvement was observed and no significant side effects were noted(p<0.05). CONCLUSION: Thymopentin may be considered to be an effective adjunctive therapeutic agent in the treatment of severe atopic dermatitis. Further studies will be needed to determine the action mechanism of thymopentin.
Body Surface Area ; Dermatitis, Atopic* ; Humans ; Injections, Subcutaneous ; Thymopentin*

The ischemic coitis proximal to obstructive colon cancer was firstly reported by MacCallum and Kaisch in 1947. Nowsdays, its clinical importance and the study of pathogenesis is reported frequently. Because of the rare inciedence of this complicated condition this lesion could be overlooked which then could result anastomotic leakage, fecal fistula and peritonitis. Recently, we have examined a 61 year-old Korean male patient who had the complaints of acute abdominal pain and bloody diarrhea. The diagnosis of gangrenous ischemic colitis associated with obstucting rectal cancer was confirmed on the basis of sigmoidoscopic and operative and associated histopathological findings. After prompt surgical intervention, the patient was discharged with good improvement. The literatures are reviewed here with the case presentation.
Abdominal Pain ; Anastomotic Leak ; Colitis, Ischemic* ; Colonic Neoplasms ; Diagnosis ; Diarrhea ; Fistula ; Humans ; Male ; Middle Aged ; Peritonitis ; Rectal Neoplasms

To evaluate the significance of placental histology, a collaborative histological and cytogenetic study was performed on the products of 88 spontaneous abortions, and subsequently bcl-2 immunostaining was performed on 62 cases. The morphometric parameters included were DCIRCLE, FORMSHAPE, CPRATIO, and the expression of bcl-2 immunostainig was graded in four categories (I to IV). The results were as follows: 1) 40% (n=35) were chromosomally abnormal: trisomies predominated (57%, n=20) and was followed by triploidy (14%, n=5), double trisomy (6%, n=2), monosomy X (6%, n=2), inversion (9) (6%, n=2). 2) mean of DCIRCLE in chromosomally abnormal pregnancy was 40 micrometer larger than that in chromosomally normal pregnancy (p=0.012, one side t-test), while no difference was found in FORMSHAPE and CPRATIO between chromosomally abnormal and normal pregnancy. 3) bcl-2 expression was found in syncytiotrophoblast and cytotrophoblast. bcl-2 expression was weaker in chromosomally abnormal pregnancy with intensity I and II of 59% than chromosomally normal pregnancy with intensity I and II of 24%. 4) In comparison bcl-2 expression with DCIRCLE, in chromosomally normal abortion one (10%) in I & II and one (3%) in III & IV showed large DCIRCLE (above 360 micrometer), while 11 (85%) in I & II and 3 (33%) in III & IV in chromosomally abnormal pregnancy. It would mean that bcl-2 protein is necessary in preservation of pregnancy and placental morphology. Abnormal villous diameter and weak bcl-2 expression may be suggestive of chromosomal anomaly. Besides other histologic parameters, application of bcl-2 immunostaining and morphometric analysis probably give more sensitive and specific results in identifying chromosomally abnormal abortion.
Abortion, Spontaneous ; Cytogenetics ; Female ; Humans ; Placenta* ; Pregnancy* ; Triploidy ; Trisomy ; Trophoblasts ; Turner Syndrome

Inflammatory linear verrucous epidermal nevus(ILVEN) is a relatively rare skin disorder, characterized by chronic inflammatory psoriasiform pattern. This chronic skin disorder is generally believed to be resistant to various treatments, although some authors have reported success with 13-cis-retinoic acid and 585 nm pulsed laser therapy. We experienced a 15-month aged female ILVEN patient who has been suffered from linear erythematous verrucous pathes mainly located on left inguinal area, hand, and foot with persistent itching sense. This skin lesion have been developed at birth, and more spread out toward right extremity after 1 year old age . We initially treated this skin lesion with topical corticosteroids and oral anti-histamines, but this treatment was not successful. So we retreated this patient with etretinate(oral retinoid) and topical 13-cis-retinoic acids, we found that therapeutic responses to these applications were more remarkable. Now, we closely observe this patient's clinical course further more.
Adrenal Cortex Hormones ; Etretinate ; Extremities ; Female ; Foot ; Hand ; Humans ; Isotretinoin ; Laser Therapy ; Nevus, Sebaceous of Jadassohn* ; Parturition ; Pruritus ; Skin

Malposition of catheter in the subdural space is a possible complication during attempted epidural anesthesia. We report here an inadvertent subdural block confirmed by the radiographic contrast materiaL Epidural anesthesia was planned for a full-term gravida and epidural catheter was inserted about 3 cm cranially at the level of L1-2. After injection of 19.5 ml local anesthetic, blood pressure was 70/50 mmHg. Fluid was rapidly infused and intravenous ephedrine 10 mg was given. After then, vital signs were well maintained and twin babies were delivered without events. Twentyfive minutes later, she complained chest tightness and became tachypneic. Ketamine 50 mg was given and endotracheal intubation was performed with ease. She obeyed command, but could not open her eyes for some duration. Self respiration returned after 110 minutes and minute ventilation was 6 liter/minute at that time. 160 minutes later, extubation was done and radiological examination was performed using contrast materiaL The cathter tip was positioned in anterior subdural space at T12 level.
Anesthesia, Epidural* ; Blood Pressure ; Catheters ; Ephedrine ; Humans ; Intubation, Intratracheal ; Ketamine ; Respiration ; Subdural Space ; Thorax ; Twins ; Ventilation ; Vital Signs

BACKGROUND: Psoriatic lesions have reduced water-holding capacity and show increased transepidermal water loss (TEWL). The effect of D-PUVA therapy, which combines topical calcipotriol and PUVA therapy, on epidermal barrier function has not yet been evaluated. OBJECTIVE: The purpose of this study was to verify the change of TEWL in lesional and normal skin according to D-PUVA therapy in psoriasis patients. METHODS: TEWL was measured consecutively by TEWAMETER TM210®, in 13 psoriasis patients who received D-PUVA therapy. Clinical grading was done according to psoriasis severity index (PSI). RESULTS: TEWL of psoriatic lesion decreased as D-PUVA continued. TEWL of normal-looking skin gradually increased, although the increase was trivial. Clinical grading of scale and in-filtration followed the pattern of PSI in the decrease of TEWL, while that of erythema did not. CONCLUSION: In psoriatic plaques, TEWL was decreased according to the improvement. In normal-looking skin, D-PUVA therapy caused only a little effect on TEWL.
Erythema ; Humans ; Psoriasis ; PUVA Therapy ; Skin ; Water*