Scleromyxedema with multiple systemic involvement: Successful treatment with intravenous immunoglobulin.

Scleromyxedema is a rare connective tissue disorder characterized by a generalized lichenoid eruption and sclerodermoid induration with histologic features of dermal mucin deposition. A 44-year-old man presented with a 3-year history of generalized progressive skin thickening and sclerosis. He had diffuse skin-colored to erythematous firm papules coalescing into indurated plaques over his whole body. He had been diagnosed with scleromyxedema from a skin biopsy with monoclonal gammopathy of undetermined significance (MGUS) at another tertiary hospital 3 years earlier. He had been treated with systemic corticosteroids and methotrexate, but his systemic symptoms (dyspnea, dysphagia, skin swelling, and induration) had worsened over the past year, so he visited our clinic seeking further evaluation and management. The patient received high-dose intravenous immunoglobulin (IVIG) therapy once a month in combination with systemic corticosteroids. After three courses of IVIG, his cutaneous symptoms and dyspnea had improved dramatically. Herein we report a case of scleromyxedema with systemic involvement with significant improvement following IVIG therapy.

Clinicopathologic Features and Prognostic Factors of Primary Cutaneous Melanoma: a Multicenter Study in Korea.

BACKGROUND: Malignant melanoma is a cutaneous malignancy with a high mortality rate and high potential for metastases. Detailed information on the clinicopathologic characteristics and prognostic factors of cutaneous melanoma is currently limited in Korea. This study aimed to identify the epidemiological and clinicopathologic characteristics of primary cutaneous melanoma in Korean patients, and to assess which prognostic variables could influence both the development of metastases in primary cutaneous melanoma and overall survival (OS). METHODS: A total of 261 patients diagnosed with primary cutaneous melanoma in seven medical centers between 1997 and 2017 were retrospectively investigated with regard to clinical presentation, localization of the tumor, histopathologic subtype, and survival time. RESULTS: The nodular histologic subtype, ulceration, and Breslow thickness were significantly associated with the development of metastasis; and overweight and obesity (body mass index > 23) were significantly associated with increased Breslow thickness. The location of the metastases appeared to influence OS: brain metastases were associated with the highest risk of death, followed by gastrointestinal, lung, and extra-regional lymph node metastases. CONCLUSION: In this study, tumor thickness, nodular histologic subtype, and ulceration predicted metastatic spread of primary cutaneous melanoma. In addition, OS was associated with the location of metastases. Obesity was related to the prognosis of primary cutaneous melanoma. Clinicians should bear these findings in mind when forming a diagnosis because of the risk of a poor prognosis.

Association between Homocysteine Levels and Psoriasis: A Meta-Analysis.

BACKGROUND: Psoriasis is a multifactorial disease associated with an increased risk for metabolic syndrome and cardiovascular diseases. Elevated levels of homocysteine (Hcy) are a marker of cardiovascular risk. Several studies have evaluated the associations between psoriasis and Hcy levels; however, the results remain inconclusive. OBJECTIVE: We performed a systematic review of the literature and a meta-analysis to better understand the relationship between psoriasis and Hcy. METHODS: Five scientific databases (MEDLINE, Embase, Cochrane Library, Scopus, and Web of Science) were searched to identify relevant studies. A review of 307 publications identified 16 studies that directly assessed plasma levels of Hcy in psoriasis patients. RESULTS: A total of 16 studies including 2,091 subjects were included in the meta-analysis. Hcy levels were significantly higher in psoriasis patients relative to healthy controls (weighted mean difference [WMD], 3.30; 95% confidence interval [CI], 1.58∼5.02; I 2=82.1%). Subgroup analyses revealed that patients with higher mean psoriasis area severity index (PASI) scores (PASI>10) had significantly higher Hcy levels compared to healthy controls (WMD, 4.17; 95% CI, 1.18∼7.16; I 2=88.3%), whereas patients with lower mean PASI scores (PASI ≤10) had not (WMD, 0.76; 95% CI, -1.84∼3.35; I 2=72.2%). CONCLUSION: This meta-analysis found that psoriasis patients, in particular those with PASI >10, had significantly higher Hcy levels compared to healthy controls. Further research is needed to determine the association between Hcy levels and psoriasis severity.

Comparison of the Efficacy of Azithromycin Versus Doxycycline in Acne Vulgaris: A Meta-Analysis of Randomized Controlled Trials.

BACKGROUND: Acne vulgaris is one of the most common disorders of the pilosebaceous unit. Although doxycycline is considered to be a first-line anti-acne antibiotic, various other antibiotics have been tried due to its adverse effects and contraindications. We performed a meta-analysis of randomized controlled trials (RCTs) that compared the efficacy of oral azithromycin pulse therapy with that of oral daily doxycycline in the management of moderate to severe acne vulgaris. METHODS: Five scientific databases (MEDLINE, EMBASE, Cochrane Library, SCOPUS, and Web of Science) were searched to identify relevant studies. A review of 1,341 publications produced six RCTs that met our predefined inclusion criteria. The clinical outcome measures were remaining acne lesion counts, patients' self-assessment of treatment, and the investigators' assessment of treatment after 12 weeks. RESULTS: We included six studies assessing 906 patients with moderate to severe acne vulgaris. Meta-analyses of clinical outcome measures revealed no significant difference between the two groups regarding remaining acne lesion counts (p=0.27), patients' self-assessment of treatment (p=0.67), and the investigators' assessment of treatment (p=0.32). The incidence of severe adverse events leading to the discontinuation of therapy was higher in the doxycycline daily therapy group when compared with the azithromycin pulse therapy group. CONCLUSION: This study indicates that azithromycin pulse therapy is equivalent to doxycycline at 12 weeks in the efficacy of the treatment for moderate to severe acne vulgaris Therefore, oral azithromycin pulse therapy may be a good alternative to doxycycline in the management of acne for those unable to tolerate doxycycline.

Sox9 is a ß-catenin-regulated transcription factor that enhances the colony-forming activity of squamous cell carcinoma cells.

Squamous cell carcinoma (SCC) is a common skin cancer, of which the incidence is relatively high, ranking second among the non­melanoma skin cancers. It is known that numerous intracellular signal regulators are involved in the pathogenesis of SCC. The Wnt/ß-catenin signaling pathway serves an important role in cancer development. However, the downstream effectors of ß­catenin remain to be clearly elucidated yet. The present study investigated the functional importance of Wnt/ß­catenin signaling in cutaneous SCC. ß­catenin expression was reduced using recombinant adenovirus expressing specific microRNA (miR). Knockdown of ß­catenin resulted in a marked reduction of the colony-forming activity of the SCC cells, SCC12. In an attempt to identify the ß­catenin downstream genes, it was found that Sox9 was regulated by ß­catenin in SCC12 cells. Overexpression of a constitutively active form of ß­catenin led to the induction of Sox9, while knockdown of ß­catenin resulted in downregulation of Sox9. When the expression of Sox9 was reduced using specific miR, colony-forming activity of the SCC12 cells was significantly reduced. When Sox9 was overexpressed in cells where ß­catenin was knocked down, it partially restored the colony­forming potential. Taken together, the present results suggested that Sox9 is a ß-catenin downstream transcription factor and is positively involved in SCC development.

Regulation of lipid production by light-emitting diodes in human sebocytes.

Light-emitting diodes (LED) have been used to treat acne vulgaris. However, the efficacy of LED on sebaceous lipid production in vitro has not been examined. This study investigated the efficacy of 415 nm blue light and 630 nm red light on lipid production in human sebocytes. When applied to human primary sebocytes, 415 nm blue light suppressed cell proliferation. Based on a lipogenesis study using Oil Red O, Nile red staining, and thin-layered chromatography, 630 nm red light strongly downregulated lipid production in sebocytes. These results suggest that 415 nm blue light and 630 nm red light influence lipid production in human sebocytes and have beneficial effects on acne by suppressing sebum production.

Application of the QuantiFERON®-TB Gold test in erythema induratum.

Erythema induratum (EI) was originally described by Ernest Bazin in 1861 as nodular lesions on the legs in association with tuberculosis (TB). A 33-year-old woman was referred to our hospital with tender ulcerative nodules on her legs. Skin biopsies revealed lobular inflammation of the subcutaneous fat with vasculitis. Although conservative treatment was given, the lesions did not improve. Several months later as the lesions persisted, we suspected a TB infection and additional tests were performed. The chest roentgenogram results were normal. The skin biopsies were negative for acid-fast bacilli (AFB). Mycobacterial cultures of the blood and skin were negative. However, the QuantiFERON®-TB Gold test was positive, which led to a diagnosis of latent TB infection being made. The patient was treated with multidrug antituberculosis therapy, which successfully resolved her skin lesions. Therefore, reported herein is the application of the QuantiFERON®-TB Gold test for the confirmation of latent TB infection in EI patients.

Roles of TLR7 in activation of NF-κB signaling of keratinocytes by imiquimod.

Imiquimod is known to exert its effects through Toll-like receptor 7 (TLR7) and/or TLR8, resulting in expression of proinflammatory cytokines and chemokines. Keratinocytes have not been reported to constitutively express TLR7 and TLR8, and the action of imiquimod is thought to be mediated by the adenine receptor, not TLR7 or TLR8. In this study, we revealed the expression of TLR7 in keratinocytes after calcium-induced differentiation. After addition of calcium to cultured keratinocytes, the immunological responses induced by imiquimod, such as activation of NF-κB and induction of TNF-α and IL-8, were more rapid and stronger. In addition, imiquimod induced the expression TLR7, and acted synergistically with calcium to induce proinflammatory cytokines. We confirmed that the responses induced by imiquimod were significantly inhibited by microRNAs suppressing TLR7 expression. These results suggest that TLR7 expressed in keratinocytes play key roles in the activation of NF-κB signaling by imiquimod, and that their modulation in keratinocytes could provide therapeutic potential for many inflammatory skin diseases.

Clinical Features of Systemic Contact Dermatitis Due to the Ingestion of Lacquer in the Province of Chungcheongnam-do.

BACKGROUND: Lacquer contains an allergen, which can cause severe contact dermatitis. Systemic dermatitis resulting from the ingestion of lacquer is quite common in Korea, until now. OBJECTIVE: The purpose of this study is to elucidate the clinical features and laboratory findings of systemic contact dermatitis (SCD), due to the ingestion of lacquer in Chungcheongnam-do. METHODS: We retrospectively reviewed the medical records of 33 patients with SCD, after ingestion of lacquer from Soonchunhyang University Hospital in Cheonan, over a 6-month period. RESULTS: In this study, 33.3% of patients ate lacquer, as a health food, and some (15.2%) by encouragement of friends or spouse. The most common way of ingestion was the lacquer-boiled chicken (48.5%), but many also ate lacquer tree sprouts (42.4%). The skin lesions developed as erythematous maculopapular eruptions, erythema multiforme, erythroderma, purpura, wheals and vesicles. On laboratory findings, 13 patients (52%) exhibited leukocytosis and 11 patients had elevated eosinophil counts. CONCLUSION: The general public is becoming more aware of the toxic effects of lacquer ingestion, but still does not fully understand the dangers of lacquer tree sprouts, and this ignorance is frequently causing SCD in Chungcheongnam-do.

Efficacy and safety of 1,064 nm Q-switched Nd:YAG laser treatment for removing melanocytic nevi.

BACKGROUND: Until recently, the removal of melanocytic nevi has been performed with a CO(2) laser or Er:YAG laser. These lasers have been useful for removing affected spots. However, enlargement of spots or some sequelae, including depressed or hypertrophic scars, could develop as unwanted results. The Q-switched Nd:YAG laser has been used to remove deep-seated melanocytes, such as Ota nevus or tattoos. However, there have been no previous experiments performed to test the efficacy and safety of this laser treatment for melanocytic nevi. OBJECTIVE: The objective of this study was to investigate the efficacy and safety of the 1,064 nm Q-switched Nd:YAG laser for removing melanocytic nevi, including congenital nevomelanocytic and acquired nevomelanocytic nevi. METHODS: Two thousand and sixty four Korean patients with small melanocytic nevi were treated with a Q-switched Nd:YAG laser from 2005 to 2009. High-resolution photographs were taken in identical lighting and positions before and after the six weeks of treatment to observe the procedural efficacy. RESULTS: About 70% of the nevi treated using a 1,064 nm Q-switched Nd:YAG laser were completely removed after one session. The other 30% were completely treated within three sessions. The appearance of sequelae such as hollow scars noticeably decreased compared to the results seen in CO(2) or Er:YAG laser treatments. CONCLUSION: Use of the 1,064 nm Q-switched Nd:YAG laser is a safe and effective treatment modality for melanocytic nevi.

Childhood granulomatous periorificial dermatitis.

Childhood granulomatous periorificial dermatitis (CGPD), also known as facial Afro-Caribbean childhood eruption (FACE), is a distinctive granulomatous form of perioral dermatitis. It is a condition of unknown etiology, characterized by monomorphous, small, papular eruptions around the mouth, nose and eyes that histopathologically show a granulomatous pattern. It affects prepubescent children of both sexes and typically persists for several months but resolved without scarring. We report a 9 year-old girl with multiple, discrete, monomorphic, papular eruptions of 2-months duration on the perioral and periocular areas. Histopathological examination demonstrated upper dermal and perifollicular granulomatous infiltrate.

An unusual case of congenital dermal melanocytosis.

Dermal melanocytosis is characterized by the presence of ectopic melanocytes in the dermis. The most common forms include the Mongolian spot, blue nevus, nevus of Ota, and nevus of Ito. Some types of dermal melanocytosis do not fit into any of these morphologic categories, however. Our case demonstrated an extensive amount of uniform deep blue patches of nevi with unilateral distribution on the left face, neck, chest, shoulder, and back. On histopathologic examination, a number of elongated melanocytes scattered throughout the dermis were found. We herein report a case of congenital unilateral dermal melanocytosis.

A case of eczema herpeticum with hailey-hailey disease.

Eczema herpeticum is the dissemination of herpes simplex virus in the setting of a preexisting skin disease. Hailey-Hailey disease [familial benign chronic pemphigus] is a blistering dermatosis that is inherited as an autosomal dominant trait and it usually presents itself around the third or fourth decades. Coexistence of eczema herpeticum and Hailey-Hailey disease is an infrequent occurrence. Four such cases have been reported in the English and German medical literature. We report here on an unusual case of eczema herpeticum that coexisted with Hailey-Hailey disease in a 47 years old man and we review the relevant literatures.