A 38-year-old man presented with a 2-year history of recurreit irticaria, abdominal pain and arthralgia of his finger joints. These symptoms were not con rolled in spite of taking medication and had a tendence to be aggravated in the winter. Laboratory findings revealed hvpocomlementemia, hypergammaglobulinemia and a positive rect on with an ice cube test. Skin biopsy specimen taken from the urticarial lesion of the left upper arm showed leukocytoclastic vasculitis with perivascular neutrophilic infiltrition, nuclear dusts, and extravasation of red blood cells. We thus made the diagnosis of hypocomplementemic urtirarial vasculitis syndrome with cold urticaria. The patient was treatcd ith systemic corticosteroids and antihistamines.
Abdominal Pain ; Adrenal Cortex Hormones ; Adult ; Arm ; Arthralgia ; Biopsy ; Diagnosis ; Dust ; Erythrocytes ; Finger Joint ; Histamine Antagonists ; Humans ; Hypergammaglobulinemia ; Ice ; Neutrophils ; Skin ; Urticaria* ; Vasculitis*

No abstract available.
Porokeratosis*

The epidermal cell-derived thymocyte activating factor (ETAF) produced by keratinocytes and endowed with IL-1 like activities, can potentiate or stimulate a variety of immune reactionsl. In addition to stimulating thymocyte proliferation, it enhanced IL-2 production by mitogen-stimulated lymphocytes. In this study, IL-2 activity stimulated by ETAF was investigated by using peripheral lymphocytes (PBL)and LBRM33 1A5 cell lines. The expression of HLA-DR antigen and the effect of r-IFN-gamma on IL-1 production by keratinocytes were also evaluated : 1. The HLA-DR positive cultured keratinocytes were obser ved in interferon (IFN), interferon-phorbol myristic acid (IFN-PMA) and interferon-lipopolysaccharide (IFN-LPS) groups. There was no HLA-DR expression in the control. PMA. and LPS group. 2. Il-1alpha from supernatants of cultured keratinocytes were produced by all groups including control group. The amounts of IL-1alpha produced by IFN, IFN-PMA and IFN-LPS groups were higher than the other groups. 3. There were no statistical differences between total IL-1alpha from cultured keratinocytes and control or other experimental groups. 4. In experiment for positivie control, the anrounts of IL-2 production by PBL and LBRM33 1A5 cell line were increased proportional to the concentration of added rIL-1. But there was no difference between groups above 1.0 U/ml and 0.5 fmol in LBRM33 1A5 cell line. 5. the production of IL-2 by PBL were increased by phytohemagglutinin (PHA), PHAPMA and PHA-LPS groups. There were no production of IL-2 in control, IFN, LPS, only LPS added group, and IL-1alpha. The IL-2 induced by PHA-PMA group was much higher than the other groups. The production of IL-2 was stimulated by IL-1 of supernatarnts and cell lysates from the cultured keratinocytes, but here was no correlation between IL-2 production and added IL-1. 6. The production of IL-2 by LBRM33 IA5 were increased by PHA, PHA-PMA and PHA-LPS groups. There were no production of IL-2 in the other groups. In summary, the results suggest that supernatants and cell lysates from cultured keratinocytes can stimulate the IL-2 production by human PBL and LBRM33 1A5 cell line. Therefore the ETAF can potentiate or stimulate the immune reactions and enhance IL-2 production by mitogen-stimulated lymphocytes.
Cell Line ; HLA-DR Antigens ; Humans ; Interferons ; Interleukin-1* ; Interleukin-2* ; Keratinocytes* ; Lymphocytes* ; Myristic Acid ; Thymocytes

Crohns disease is a granulomatous isease of the gastrointetin 1 tract, especially terminal ileum, but may involve the lip, mouth, anus, skin, liver and bone. The skin is a common site of extraintestinal involvement in Crohns disease, with the perianil, perineal, and peristomal areas most frequently affected. Vulvar involvement, however, is an uncommon extraintestinal manifestantion of Crohns disease. We report a case of a 16-year-old girl with Crohns disease of the vulva and perianal region. A histopathologic study of the descending colon and vulvar skin showed noncaseating granulomatous reactions composed of lymphocytes, epithelioidell and giant cells. We were able to make the diagnosis of Crohns disease of the vulvar and the patient was treated with prednisolone and metronidazole.
Adolescent ; Anal Canal ; Colon, Descending ; Crohn Disease* ; Diagnosis ; Female ; Giant Cells ; Humans ; Ileum ; Lip ; Liver ; Lymphocytes ; Metronidazole ; Mouth ; Prednisolone ; Skin ; Vulva*

Candida granuloma is a variant of chronic mucocutaneous candidasis associated with chronic infection mainly by Candida albicans in which the skin lesions are rather granulomatous and hyperkeratotic. To date a variety of defects of cell mediated immunity have been shown to be important. We describe a 2-year-old boy with cutaneous candida granuloma and immunologic dysfunction.
Candida albicans ; Candida* ; Child, Preschool ; Granuloma* ; Humans ; Immunity, Cellular ; Male ; Skin

Osteoma of the temporal bone most commonly occurs in the external ear. Osteomas in the middle ear are not common, and only 25 relevant cases (18 papers) have been reported in the English literature. With only 5 cases reported to date, osteoma of the promontory in the middle ear is rare. This study reports a case of asymptomatic osteoma of the promontory in the middle ear mimicking a congenital cholesteatoma in a 4-year-old girl.
Child, Preschool ; Cholesteatoma* ; Ear, External ; Ear, Middle ; Female ; Humans ; Osteoma* ; Temporal Bone

We observed that treatment with dimethyl α-ketoglutarate (DMK) increased the amount of intracellular α-ketoglutarate significantly more than that of α-ketoglutarate in HaCaT cells. DMK also increased the level of intracellular 4-hydroxyproline and promoted the production of collagen in HaCaT cells. In addition, DMK decreased the production of collagenase and elastase and downregulated the expression of selected matrix metalloproteinases (MMPs), such as MMP-1, MMP-9, MMP-10, and MMP-12, via transcriptional inhibition. The inhibition of MMPs by DMK was mediated by the suppression of the IL-1 signaling cascade, leading to the attenuation of ERK1/2 phosphorylation and AP-1 transactivation. Our study results illustrate that DMK, an alkylated derivative of α-ketoglutarate, increased the level of 4-hydroxyproline, promoted the production of collagen, and inhibited the expression of selected MMPs by affecting the IL-1 cascade and AP-1 transactivation in HaCaT cells. The results suggest that DMK might be useful as an anti-wrinkle ingredient.

PURPOSE: The early surgical outcomes of endoscopic thyroidectomy in papillary thyroid cancer (PTC) are comparable to those of conventional open thyroidectomy; however, there is little evidence about long-term outcomes. The aim of this study was to compare the 5-year surgical outcomes of endoscopic versus open thyroidectomy. METHODS: We reviewed 804 patients with PTC who underwent thyroidectomy between October 2008 and October 2010. Of these, 703 patients received conventional open thyroidectomy (OT group) and 101patients underwent endoscopic thyroidectomy (ET group). The clinicopathologic characteristics and surgical outcomes were compared between those treatments. RESULTS: ET was applied significantly more often in young patients and females. The lobectomy and unilateral CCND were performed more frequently in ET, and the mean tumor size was smaller. The prevalence of extrathyroidal extension, multiplicity, and lymphatic invasion was more frequent in OT. The T and TNM stage were more advanced in OT, whereas the N status was similar between treatments. The mean surgical time was significantly longer for ET, while the number of retrieved lymph nodes was greater in OT. However, the stimulated thyroglobulin levels at first RAI ablation, total amount of RAI administration and 5-year recurrence rate did not significantly differ between groups. The incidence of transient hypocalcemia was significantly higher in OT, but the incidence of permanent hypocalcemia and transient/permanent recurrent laryngeal nerve injury were similar in both groups. CONCLUSION: Endoscopic thyroidectomy might be a safe and effective procedure in well-selected PTC patients
Female ; Humans ; Hypocalcemia ; Incidence ; Lymph Nodes ; Operative Time ; Prevalence ; Recurrence ; Recurrent Laryngeal Nerve Injuries ; Thyroglobulin ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy*

PURPOSE: Pathological complete response (pCR) of axillary lymph node (LN) is frequently achieved in patients with clinically node-positive breast cancer after neoadjuvant chemotherapy (NAC). Treatment of the axilla after NAC is not well established and the value of sentinel LN biopsy following NAC remains unclear. This study investigated the predictive value of axillary response following NAC and evaluated the predictive value of a model based on axillary response. METHODS: Data prospectively collected on 201 patients with clinically node-positive breast cancer who were treated with NAC and underwent axillary LN dissection (ALND) were retrieved. A model predictive of axillary pCR was developed based on clinicopathologic variables. The overall predictive ability between models was compared by receiver operating characteristic (ROC) curve analysis. RESULTS: Of 201 patients who underwent ALND after NAC, 68 (33.8%) achieved axillary pCR. Multivariate analysis using axillary LN pCR after NAC as the dependent variable showed that higher histologic grade (p=0.031; odds ratio [OR], 2.537; 95% confidence interval [CI], 1.087–5.925) and tumor response rate ≥47.1% (p=0.001; OR, 3.212; 95% CI, 1.584–6.515) were significantly associated with an increased probability of achieving axillary pCR. The area under the ROC curve for estimating axillary pCR was significantly higher in the model that included tumor response rate than in the model that excluded this rate (0.732 vs. 0.649, p=0.022). CONCLUSION: Tumor response rate was the most significant independent predictor of axillary pCR in response to NAC. The model that included tumor response rate was a significantly better predictor of axillary pCR than the model that excluded tumor response rate.
Axilla ; Biopsy ; Breast Neoplasms* ; Breast* ; Drug Therapy* ; Humans ; Lymph Nodes ; Multivariate Analysis ; Neoadjuvant Therapy ; Odds Ratio ; Polymerase Chain Reaction ; Prospective Studies ; ROC Curve

PURPOSE: B-cell lymphoma 2 (BCL2) is an antiapoptosis protein and an important clinical breast cancer prognostic marker. As the role of BCL2 is dependent on the estrogen receptor (ER) status, this effect might differ according to molecular subtypes. The aim of this study was to evaluate the relationship between the prognostic outcomes and BCL2 expression among the molecular subtypes. METHODS: We retrieved the data of 1,356 patients who were newly diagnosed with malignant breast cancer between November 2006 and November 2011. Immunohistochemistry was used to measure ER, progesterone receptor, human epidermal growth factor receptor 2 (HER2), Ki-67, and BCL2 expression. We classified breast cancer into five molecular subtypes based on the 13th St. Gallen International Expert Consensus, including luminal A, luminal B (HER2-negative), luminal B (HER2-positive), HER2-overexpression, and triple-negative subtypes. We analyzed the clinicopathological features and assessed the correlation between BCL2 expression and clinical outcomes, such as relapse-free survival (RFS) and disease-specific survival (DSS) according to the five molecular subtypes. RESULTS: A total of 605 cases of breast cancer (53.8%) showed BCL2 expression. BCL2-positive expression was associated with young age (<50 years, p=0.036), lower histological grade (p<0.001), low Ki-67 level (<14%, p<0.001), hormone receptor positivity (p<0.001), HER2 negativity (p<0.001), luminal breast cancer (p<0.001), and low recurrence rate (p=0.016). BCL2-positive expression was also associated with favorable 5-year RFS (p=0.008, 91.4%) and DSS (p=0.036, 95.6%) in all the patients. BCL2-positive expression in luminal A breast cancer resulted in significantly favorable 5-year RFS and DSS (p=0.023 and p=0.041, respectively). However, BCL2 expression was not associated with the prognosis in the other subtypes. CONCLUSION: The prognostic role of BCL2 expression in breast cancer is subtype-specific. BCL2 expression differs according to the molecular subtype and is a good prognostic marker for only luminal A breast cancer.
Biomarkers, Tumor ; Breast Neoplasms* ; Breast* ; Consensus ; Estrogens ; Humans ; Immunohistochemistry ; Lymphoma, B-Cell ; Phenobarbital ; Prognosis ; Receptor, Epidermal Growth Factor ; Receptors, Progesterone ; Recurrence ; Weights and Measures