Severe Hyperhidrosis in Apert Syndrome: A Case Report / 대한피부과학회지

Apert syndrome is a rare genetic disorder characterized by malformations of the skull, face, hands, and feet. We report a case of severe hyperhidrosis in a 13-month-old female infant with Apert syndrome who was born with craniosynostosis, midface hypoplasia, and syndactyly of both hands. She had a history of excessive sweating since birth and this was confirmed using the iodine-starch test. Hyperhidrosis was first reported as a key cutaneous manifestation of Apert syndrome in 1993. However, the main focus in the field of dermatology is on antibiotic-refractory acne, which serves as another cutaneous hallmark of the disease. This is the first report in the Korean literature that describes hyperhidrosis in Apert syndrome. We highlight the presentation of hyperhidrosis as a key cutaneous manifestation in Apert syndrome.

Hair Follicle Nevus Located on the Neck: Comparison with Accessory Tragus, Cervical Chondrocutaneous Branchial Remnants and Trichofolliculoma

Hair follicle nevus (HFN) is a rare, benign, follicular hamartoma that most frequently presents as a congenital nodule on the face. We experienced a rare case of HFN on the neck of a 14-year-old boy and performed a pilot immunohistochemical study with cytokeratin 19 (CK19) to compare the staining pattern of hair follicles in HFN and its differential diagnoses, accessory tragus, cervical chondrocutaneous branchial remnants (CCBR) and trichofolliculoma. With hematoxylin and eosin stain, HFN showed numerous tiny hair follicles in the dermis with several sebaceous and eccrine glands, and perifollicular fibrous thickening. With CK19 stain, some hair follicles in HFN and CCBR showed positive expression, a few hair follicles in accessory tragus showed weak expression, and no hair follicles in trichofolliculoma showed expression. The present report supports the view that HFN, accessory tragus and CCBR are within the same spectrum of hamartomas.

Desmoplastic Fibroblastoma of the Scalp Accompanied by Severe Pain; Unusual Location and Symptom

Desmoplastic fibroblastoma is a rare fibrous tumor that usually presents as a painless, slow-growing mass in the subcutaneous tissues and skeletal muscles. It has a wide anatomic distribution, with the most common involvement being the arm and shoulder. Here, we report a case of a tiny painful desmoplastic fibroblastoma arising on the scalp. According to a microscopic examination, this tumor was composed of spindle-shaped fibroblasts in the dense collagenous stroma. On immunohistochemical staining, tumor cells were positive for vimentin and negative for smooth muscle actin, CD34, and S100. Our case is unique in that desmoplastic fibroblastoma developed on the scalp and there was presence of pain despite its small size.

A Case of Indeterminate Dendritic Cell Tumor: A Rare Neoplasm with Langerhans Cell Lineage

No abstract available.

Platycodin D May Improve Acne and Prevent Scarring by Downregulating SREBP-1 Expression Via Inhibition of IGF-1R/PI3K/Akt Pathway and Modulating Inflammation with an Increase in Collagen

BACKGROUND: Although many therapeutic agents have been developed, only a few drugs are known to target multiple pathogenic factors in the treatment of acne. OBJECTIVE: The purpose of this study was to identify a new drug candidate, platycodin D, which is a substance extracted from the root of Platycodon grandiflorum. METHODS: Using western blotting and Cell Counting Kit-8 assay, we studied the effects of platycodin D on SEB-1 sebocytes, fibroblasts, and keratinocytes. We investigated its effects in view of lipogenesis, collagen production, anti-inflammatory activity, and dyskeratinization. RESULTS: In SEB-1 sebocytes, platycodin D showed a sebosuppressive effect by downregulating ERK and insulin- like growth factor-1R/PI3K/Akt/sterol-regulatory element binding protein-1 signaling pathways. In addition, adiponectin, one of the adipokines responsible for sebum production, was decreased in platycodin D-treated SEB-1 sebocytes. In fibroblasts, platycodin D increased collagen production and reduced inflammation by inhibiting nuclear factor kappa B and matrix metalloproteinases. Platycodin D also showed anti-inflammatory effects on keratinocytes. It also suppressed keratin 16 expression induced by lipopolysaccharide. Furthermore, platycodin D showed no cytotoxicity on both SEB-1 sebocytes and fibroblasts. CONCLUSION: Our data demonstrate the clinical feasibility of platycodin D for acne treatment and the prevention of acne scarring by sebosuppressive and anti-inflammatory effects, as well as through an increase in collagen levels.

Hydroxyurea-induced Longitudinal Melanonychia: A Case Report and Dermoscopic Findings / 대한피부과학회지

No abstract available.

Korean Version of Inventory of Complicated Grief Scale: Psychometric Properties in Korean Adolescents

We aimed to validate the Inventory of Complicated Grief (ICG)-Korean version among 1,138 Korean adolescents, representing a response rate of 57% of 1,997 students. Participants completed a set of questionnaires including demographic variables (age, sex, years of education, experience of grief), the ICG, the Children's Depression Inventory (CDI) and the Lifetime Incidence of Traumatic Events-Child (LITE-C). Exploratory factor analysis was performed to determine whether the ICG items indicated complicated grief in Korean adolescents. The internal consistency of the ICG-Korean version was Cronbach's alpha=0.87. The test-retest reliability for a randomly selected sample of 314 participants in 2 weeks was r=0.75 (P<0.001). Concurrent validity was assessed using a correlation between the ICG total scores and the CDI total scores (r=0.75, P<0.001). The criterion-related validity based on the comparison of ICG total scores between adolescents without complicated grief (1.2±3.7) and adolescent with complicated grief (3.2±6.6) groups was relatively high (t=5.71, P<0.001). The data acquired from the 1,138 students was acceptable for a factor analysis (Kaiser-Meyer-Olkin Measure of Sampling Adequacy=0.911; Bartlett's Test of Sphericity, chi2=13,144.7, P<0.001). After omission of 3 items, the value of Cronbach's alpha increased from 0.87 for the 19-item ICG-Korean version to 0.93 for the 16-item ICG-Korean version. These results suggest that the ICG is a useful tool in assessing for complicated grief in Korean adolescents. However, the 16-item version of the ICG appeared to be more valid compared to the 19-item version of the ICG. We suggest that the 16-item version of the ICG be used to screen for complicated grief in Korean adolescents.

Microcystic Adnexal Carcinoma Misdiagnosed as Desmoplastic Trichoepithelioma on Preoperative Biopsy

Microcystic adnexal carcinoma is a rare type of tumor, with about 300 cases reported globally. Due to its similar histology with other tumors, it is occasionally misdiagnosed as desmoplastic trichoepithelioma, basal cell carcinoma, syringoma, and so on. We present a patient with a mass on the perioral area who was preoperatively diagnosed with trichoepithelioma. Microcystic adnexal carcinoma was diagnosed after excisional biopsy and a wide excision. Defects were reconstructed with a mucosal advancement flap. There was no recurrence and there were no significant complications during the 18-month follow-up period. Because superficial punch biopsy has limitations in width and depth, surgeons should always consider the possibility of malignancy of a mass even if a biopsy shows a benign result.

Corrigendum: Expression of the spinal 5-HT7 receptor and p-ERK pathway in the carrageenan inflammatory pain of rats (Korean J Anesthesiol 2015 April 68(2): 170-174) / 대한마취과학회지

This article was inadvertently omitted Acknowledgments section for grant support.

Comparison of FcRgamma-Deficient and CD57+ Natural Killer Cells Between Cord Blood and Adult Blood in the Cytomegalovirus-Endemic Korean Population

BACKGROUND: FcRgamma-deficient natural killer (NK) cells (g-NK cells) have been associated with cytomegalovirus (CMV) infection. However, the frequency of g-NK cells in a CMV-endemic area (i.e., Korea) has not yet been studied. We examined the frequency of g-NK cells and expression of CD57 on NK cells in cord blood (CB) and adult blood (AB). METHODS: Of the 24 AB samples collected, 95.8% (23/24) were CMV IgG+/IgM-, while 100% of the 13 healthy CB samples were CMV IgG+/IgM-. We performed whole-blood flow cytometry assays to analyze intracellular FcRgamma and CD3zeta expression of CD3-/CD56dim NK cells from 13 CB and 24 AB samples, and surface CD57 expression on CD3-/CD56dim/CD16+ NK cells from 13 CB and 19 AB samples. RESULTS: All CMV seropositive AB samples contained g-NK cells (23/23), and the median proportion of g-NK cells in the CD3-/CD56dim NK cell pool was 35.0% (range: 11-77%). CD57+ NK cells in the CD3-/CD56dim/CD16+ NK cell population were detected in all 19 AB samples tested, but not in any CB samples. CONCLUSIONS: Our data suggest that g-NK cells and CD57+ NK cells are present at a very high frequency in CMV-seropositive AB, but rare in CMV-naive CB.

Expression of the spinal 5-HT7 receptor and p-ERK pathway in the carrageenan inflammatory pain of rats / 대한마취과학회지

BACKGROUND: Although the inhibitory role of the 5-hydroxytrypatmine receptor 7(5-HT7R) on nociceptive processing is generally recognized, an excitatory effect associated with a reduced 5-HT7R expression has also been observed in the nerve injury model. In the carrageenan model, no significant effect is produced by the 5-HT7R activation, but the change in 5-HT7R expression has not been examined. Lesioning of the spinal serotonergic pathway enhances allodynia in the carrageenan model, but it also relieves several other pain states, including in the formalin model. While lesioning suppresses the activation of the extracellular signal-regulated kinase (ERK) of the spinal cord in the formalin model, its role in the carrageenan model has not been reported. METHODS: Following intraplantar injections of carrageenan, the spinal 5-HT7R expression was examined using Western blotting in male Sprague-Dawley rats. The effect of serotonergic pathway lesioning with intrathecal 5,7-dihydroxytryptamine (5,7-DHT) on the expression of the phospho-ERK was measured. RESULTS: The expression of the 5-HT7R in the carrageenan model was not significantly different from that of naive animals. The expression of the spinal p-ERK in the carrageenan model was significantly increased, but returned to the level of a naive rat 1 hour after the carrageenan injection. However, it remained significantly higher 1 hour after the injection in the animals treated with 5,7-DHT than in the naive and control rats. CONCLUSIONS: The expression of the spinal 5-HT7R is not altered by peripheral inflammation with carrageenan, suggesting that the lack of antinociceptive effect of the 5-HT7R activation is partly attributable to the absence of changes in the expression of the 5-HT7R in the spinal cord. The extended increase of the spinal p-ERK might be related to the enhanced pain behavior in the animals with lesions of the spinal serotonergic pathway in the carrageenan model.

Thyroid Dysfunction Associated with Administration of the Long-Acting Gonadotropin-Releasing Hormone Agonist

Gonadotropin-releasing hormone (GnRH) agonist has been used in the treatment of a wide variety of sex-hormone-related diseases, as the administration of GnRH agonist can alter the secretion of gonadotropin and sex hormones. Recently, we found that the long-acting GnRH agonist aggravated hyperthyroidism and induced painless thyroiditis. This is the first report to demonstrate the association of thyroid dysfunction with GnRH agonist injection in Korea. Here, we report three cases and emphasize the clinical importance of this aggravating factor in autoimmune thyroid disease.

A Successful Delivery of a Woman with Pulmonary Arterial Hypertension: Under Close Observation and without Medications

Pregnant women with severe pulmonary arterial hypertension (PAH) have a high maternal mortality risk. Regardless of the PAH severity, the possibility of maternal death increases before and after childbirth. In general principle, if pregnancy happens, termination is the recommendation. Several case reports noted successful deliveries of pregnant women with PAH while using the drugs of anticoagulants, calcium channel blockers, prostaglandins, and phosphodiesterase V inhibitors. However, choices of treatment are generally achieved by clinical experiences because of no established guidelines of treatments for PAH in pregnancy. We describe a case of a 31-year-old pregnant woman with mild PAH who successfully delivered a viable newborn under close observation and without any medications. In the state of no consensus of the management for PAH in pregnancy, prior to termination or empirical drug treatments, close observation without medications could be considered in mild PAH.

Two Cases of Postpartum Thyroiditis Followed by Graves' Disease / 대한갑상선학회지

The most common thyroid dysfunctions that occur after delivery are postpartum thyroiditis (PPT) and Graves' disease (GD). PPT is more likely to occur among patients who had a history of PPT or GD. For that reason, it is possible to assume that both PPT and GD occur concomitantly after delivery. Here we report two cases of atypical postpartum thyroid dysfunctions presenting the simultaneous occurrence of PPT and GD. A 31-year-old woman with history of PPT had thyrotoxicosis and hypothyroidism of PPT followed by GD with mild symptoms. The patient recovered quickly afterwards. In the second case, a 28-year-old woman with a history of GD presented with thyrotoxicosis of PPT followed by severe GD. The patient required long-term antithyroid treatment.

A Case of Hypothyroidism in Remission during Pregnancy

Hypothyroidism should be treated in pregnancy, because it has been associated with an increased risk of adverse pregnancy complications, as well as detrimental effects upon fetal neurocognitive development. The goal of L-thyroxine (LT4) treatment is to normalize maternal serum TSH values within the trimester-specific pregnancy reference range. 50% to 85% of hypothyroid women being treated with exogenous LT4 need to increase the dose during pregnancy. In this study, we report a case of a 29-year-old woman with hypothyroidism who had been in remission and discontinued LT4 treatment during her pregnancy. Three months after delivery she had a relapse of hypothyroidism and was retreated with LT4. Many factors can influence the gestational requirement for LT4, therefore maternal serum TSH should be monitored and the LT4 dose should be adjusted in pregnant patients with treated hypothyroidism.

Determinants of Follow-up Changes of Brachial Ankle Pulse Wave Velocity in Korean Women

BACKGROUND: Pulse Wave Velocity (PWV) correlates well with arterial distensibility and stiffness and is a useful approach for evaluating the severity of systemic arteriosclerosis in adults. In addition, measurement of brachial-ankle PWV (baPWV) has been commonly reported as a simple, noninvasive, and practicable method. Arterial stiffness assessed by PWV could predict cardiovascular morbidity and mortality. In this study, we investigated the association between the changes of baPWV and cardiovascular risk factors in Korean women using data from follow-up evaluations. METHODS: The subjects were 626 women (age, 47.2 +/- 8.2) in whom we measured baPWV and cardiovascular risk factors at baseline and about one year later. Arterial stiffness was evaluated by baPWV and biological parameters were evaluated on the same day. We retrospectively analyzed the relationships between changes of baPWV and those other factors. All analyses were performed with SPSS ver. 20.0 and p-values < 0.05 were considered significant. RESULTS: In correlation analysis, changes of baPWV were affected by changes in systolic blood pressure (SBP), diastolic blood pressure (DBP), pulse pressure, total cholesterol, high density lipoprotein-cholesterol, and low density lipoprotein-cholesterol. Multiple regression analysis of relationship between changes of baPWV and other associated variables shows that improvement of baPWV was significantly positively associated with changes of SBP and hemoglobin A1c (HbA1c), and worsening of baPWV was significantly negatively associated with changes of DBP, age, and SBP in sequence. CONCLUSIONS: In improvement of baPWV, decreases of SBP and HbA1c and in worsening of baPWV, increases of DBP, age, and SBP were significant factors in Korean women.