ABSTRACT
Benign cephalic histiocytosis (BCH) is an uncommon subtype of non-Langerhans cell histiocytosis. A 5-month-old boy presented with multiple yellowish facial papules and plaques, which later spread to his trunk and both extremities. Laboratory tests, including lipid profile, were normal. Histological examination revealed non-foamy histiocytes, lymphocytes, and some eosinophils in the dermis. Immunohistochemical staining was positive for CD68and factor XIIIa, but negative for CD1a and S-100. Additionally, the patient developed multiple café-au-lait spots with axillary and inguinal freckling. Next-generation sequencing identified a pathogenic variant of the neurofibromatosis type 1 (NF1) gene. Herein, we report a rare case of BCH in a patient with NF1. Although many cases of NF1 accompanied by juvenile xanthogranuloma have been reported, the association between BCH and NF1 has not been elucidated. However, considering that BCH may be a clinicopathological variant of juvenile xanthogranuloma, an association between the two diseases can be considered.
ABSTRACT
Multiple miliary osteoma cutis (MMOC) is a rare variant of osteoma, characterized by multiple eruptive hard nodules on the face. A 70-year-old female presented with multiple solid skin-colored papules on both cheeks, unresponsive to conventional medical treatments. She reported receiving an injection of an unknown cosmetic filler substance into her face by an unlicensed medical practitioner 20 years ago. Facial computed tomography showed multiple small calcifications immediately adjacent to foreign material assumed to be the filler substance in the dermis. Histological examination revealed osteoclasts, osteocytes, and eosinophilic bony tissue in the dermis, suggestive of osteoma cutis. Although the pathogenesis remains unclear, inflammation caused by injected foreign material may induce metaplastic transformation of multipotent mesenchymal cells into osteoblasts, resulting in heterotopic ossification. Dermatologists should be aware that MMOC may occur following injection of foreign material by unlicensed practitioners. Performing a detailed history and clinical evaluation may aid in the diagnosis of such recalcitrant skin lesions.
ABSTRACT
Background@#Oxidative stress is generally accepted as one of the principal pathogenesis of vitiligo, and keratinocyte-melanocyte interactions are also thought to play critical roles. It is well-known that antioxidant response and autophagy protect cells against oxidative damage, but the details and the compensatory relationship between the two mechanisms in the keratinocytes of vitiligo lesions remain unclear. @*Objective@#To evaluate the antioxidant response and autophagy status of patients with vitiligo and to explore the interactions between these two mechanisms. @*Methods@#Ten patients with clinicopathologically proven vitiligo and 10 normal controls were enrolled in our Department of Dermatology, Soonchunhyang University Hospital, Bucheon, Korea. Tissue samples of vitiligo lesions in the patient group and normal skin in the control group were immunohistochemically stained for nuclear factor erythroid 2-related factor 2 (Nrf2), Beclin-1, microtubule-associated protein light chain 3 (LC3)-II, and p62. The immunopositivity of epidermal keratinocytes was evaluated. @*Results@#Keratinocytes in vitiligo lesions had a significantly lower expression of Nrf2 (p=0.002) than that in the cells of normal controls. The levels of autophagy markers did not differ significantly between the two groups, but decreases in the Beclin-1 and LC3-II levels, and an increase in the p62 level in the patient group may indicate a small decrease in autophagy of patients with vitiligo. @*Conclusion@#Decreased antioxidant response and reduced autophagy may trigger melanocyte apoptosis in vitiligo lesions.
ABSTRACT
Background@#In 2015, the Korean Atopic Dermatitis Association (KADA) working group published consensus guidelines for treating atopic dermatitis (AD). @*Objective@#We aimed to provide updated consensus recommendations for systemic treatment of AD in South Korea based on recent evidence and experience. @*Methods@#We compiled a database of references from relevant systematic reviews and guidelines on the systemic management of AD. Evidence for each statement was graded and classified based on thestrength of the recommendation. Forty-two council members from the KADA participated in three rounds of voting to establish a consensus on expert recommendations. @*Results@#We do not recommend long-term treatment with systemic steroids forpatients with moderate-to-severe AD due to the risk of adverse effects. We recommend treatment with cyclosporine or dupilumab and selective treatment with methotrexate or azathioprine for patients with moderate-to-severe AD. We suggest treatment with antihistamines as an option for alleviating clinical symptoms of AD. We recommend selective treatment with narrowband ultraviolet B for patients with chronic moderate-to-severe AD. We do not recommend treatment with oral antibiotics for patients with moderate-to-severe AD but who have no signs of infection. We did not reach a consensus on recommendations for treatment with allergen-specific immunotherapy, probiotics, evening primrose oil, orvitamin D for patients with moderate-to-severe AD. We also recommend educational interventions and counselling for patients with AD and caregivers to improve the treatment success rate. @*Conclusion@#We look forward to implementing a new and updated consensus of systemic therapy in controlling patients with moderate-to-severe AD.
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Background@#In pityriasis versicolor, systemic antifungal agents may be indicated for widespread or refractory lesions rather than topical treatment. Oral ketoconazole is an effective treatment for pityriasis versicolor. To our knowledge, this is the first meta-analysis to compare antifungal agents one-to-one. @*Objective@#To compare the effectiveness of oral azole antifungal agents (fluconazole, itraconazole, and ketoconazole) one-to-one in pityriasis versicolor. @*Methods@#A computerized search was performed in different databases, including PubMed, Cochrane, EMBASE, OVID Medline, KoreaMed, KISS, and MedRIC. Seven randomized controlled trials were included. Further, statistical analyses of the extracted outcome data from the studies were performed using Rex Software (ver. 3.0.1). @*Results@#A total of 1,828 records were identified. The results of the meta-analysis including seven studies revealed no significant differences in the mycological cure rates between fluconazole and ketoconazole (risk ratio [RR]: 1.01, 95% confidence interval [CI]: 0.93∼1.09, p=0.8246), fluconazole and itraconazole (RR: 1.14, 95% CI: 0.81∼1.60, p=0.4512), and ketoconazole and itraconazole (RR: 1.07, 95% CI: 0.96∼1.20, p=0.2265). @*Conclusion@#There was no superiority in the therapeutic effect of any drug among the oral azole antifungals used in pityriasis versicolor.
ABSTRACT
Background@#In pityriasis versicolor, systemic antifungal agents may be indicated for widespread or refractory lesions rather than topical treatment. Oral ketoconazole is an effective treatment for pityriasis versicolor. To our knowledge, this is the first meta-analysis to compare antifungal agents one-to-one. @*Objective@#To compare the effectiveness of oral azole antifungal agents (fluconazole, itraconazole, and ketoconazole) one-to-one in pityriasis versicolor. @*Methods@#A computerized search was performed in different databases, including PubMed, Cochrane, EMBASE, OVID Medline, KoreaMed, KISS, and MedRIC. Seven randomized controlled trials were included. Further, statistical analyses of the extracted outcome data from the studies were performed using Rex Software (ver. 3.0.1). @*Results@#A total of 1,828 records were identified. The results of the meta-analysis including seven studies revealed no significant differences in the mycological cure rates between fluconazole and ketoconazole (risk ratio [RR]: 1.01, 95% confidence interval [CI]: 0.93∼1.09, p=0.8246), fluconazole and itraconazole (RR: 1.14, 95% CI: 0.81∼1.60, p=0.4512), and ketoconazole and itraconazole (RR: 1.07, 95% CI: 0.96∼1.20, p=0.2265). @*Conclusion@#There was no superiority in the therapeutic effect of any drug among the oral azole antifungals used in pityriasis versicolor.
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Background@#Social standards for skin health have changed and have led to a rise in the general population’s interest to achieve flawless skin. @*Objective@#To survey the Korean public’s perception of skin health and dermatologists and skinhealth-related behaviors. @*Methods@#A total of 355 participants of the Skin Health Expo 2018, held by the Dermatological Research Foundation of the Korean Dermatological Association from September 14 to 16, 2018, completed a questionnaire that assessed demographics, status, and behaviors regarding skin health. The questionnaire also included lifestyle characteristics, satisfaction, behavioral factors, and perceptions regarding skin-care products. @*Results@#Participants were using various skin-care products including toners, lotions, hydrating or nutrition creams, sunscreens, essences, serums, facial masks and packs, body, hand, and foot moisturizers, eye creams, and hair products. The participants believed that the most reliable source of information on skin-care product recommendations was the dermatologist (41.55%). However, purchases were also being made at skin-care product stores (27.64%) based on the recommendation of a friend or acquaintance (40.11%). The results also revealed that 23.8% of participants experienced side effects from using skin-care and cosmetic products, and about 40% of participants could not yet distinguish hospitals run by dermatologists. @*Conclusion@#Demand for skin-care products has been increasing, and dermatologists should provide proper information about skin health to prevent adverse effects from skin-care products. Educational and informative activities, such as the expo planned by dermatologists, are necessary to improve skin health in the general population.
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Juvenile pemphigus vulgaris (JPV) is a rare variant of pemphigus vulgaris (PV) occurring in childhood and adolescence that has similar symptoms and the same histological and immunopathological features as classic adult PV. Although rare, advanced cases of JPV can be fatal due to secondary sepsis. Many patients with JPV are misdiagnosed and therefore not properly treated in the early stages of the disease. Although systemic corticosteroids are the therapeutic mainstay, long-term corticosteroid use has various adverse effects. Intravenous immunoglobulin (IVIG) was recently reported to reduce the side effects of corticosteroids. Here, we report a case of JPV in a 14-year-old boy treated with IVIG.
Subject(s)
Adolescent , Adult , Humans , Male , Adrenal Cortex Hormones , Immunoglobulins , Immunoglobulins, Intravenous , Pemphigus , SepsisABSTRACT
BACKGROUND@#Atopic dermatitis is a chronic inflammatory disease associated with long-term use of topical and systemic medications. Several articles have described the use of balneotherapy as an adjuvant treatment for atopic dermatitis in many countries, including Korea.@*OBJECTIVE@#The goal of this study was to investigate the therapeutic efficacy and safety of balneotherapy as an adjuvant treatment of atopic dermatitis.@*METHODS@#Ten patients with atopic dermatitis were enrolled in this study and each patient served as their own control. Testing was conducted on the anterior cubital fossa of the arm. A priori binary randomization was completed to determine which arm would receive balneotherapy or tap water. Patients visited clinics 3 times per week for 2 weeks and had a forearm bath for 15 minutes. Assessment was performed at baseline, and after 1 and 2 weeks of treatment.@*RESULTS@#At the last visit, the balneotherapy group showed improvements in their local eczema area, severity index score, and the degree transepidermal water loss. The physician's global assessment, patient's self-global assessment, and skin hydration was increased in both groups.@*CONCLUSION@#Our study suggests that balneotherapy is a safe adjuvant therapy that could be considered for the treatment of atopic dermatitis. However, more studies are required to demonstrate scientific basis for this treatment.
ABSTRACT
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.
Subject(s)
Humans , Brain , Diagnosis , Korea , Lung , Lymph Nodes , Melanoma , Mortality , Neoplasm Metastasis , Obesity , Overweight , Prognosis , Retrospective Studies , Skin Neoplasms , UlcerABSTRACT
BACKGROUND: Systemic immunomodulatory treatment is actively recommended in the treatment for moderate to severe atopic dermatitis (AD) patients. However, consensus criteria for the classification of AD severity or treatment refractoriness have not been established yet. OBJECTIVE: To establish consensus criteria on the definition of severity classification and treatment refractoriness of AD to provide a basis for proper treatment strategy. METHODS: The Korean Atopic Dermatitis Association (KADA) comprised a task force team to establish a definition of moderate to severe AD. A draft of definition of moderate to severe AD was made on the basis of evidence. The recommendation was confirmed by KADA members through a web-based survey. RESULTS: KADA approved that AD with 16≤eczema area and severity index (EASI)<23 should be basically defined as moderate AD whereas AD with EASI score ≥23 should be considered as severe AD. They agreed that it would be reasonable to raise the severity level if patient's daytime or nighttime pruritus numerical rating scale is equal to or higher than 7 (≥7) or dermatology life quality index score exceeds 10. AD patients who do not reach EASI 50 after appropriate treatment for three months should be considered as a non-responder. Patients with recurrence (EASI ≥16) within three months after cessation of treatment should be considered as a recurrent AD. CONCLUSION: KADA built a consensus of definition of moderate and severe AD and treatment-refractoriness. These guidelines are expected to help physicians determine proper treatment options in need.
Subject(s)
Humans , Advisory Committees , Classification , Consensus , Dermatitis, Atopic , Dermatology , Diagnosis , Pruritus , Quality of Life , Recurrence , Treatment Failure , Withholding TreatmentABSTRACT
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; I²=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; I²=88.3%), whereas patients with lower mean PASI scores (PASI ≤10) had not (WMD, 0.76; 95% CI, −1.84∼3.35; I²=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.
Subject(s)
Humans , Cardiovascular Diseases , Homocysteine , Plasma , PsoriasisABSTRACT
Hidradenitis suppurativa (HS) is a chronic, inflammatory and painful skin disease with recurrent nodules and tracts involving the intertriginous regions. It is known that the patient with HS shows an increased risk of metabolic disorders such as diabetes, metabolic syndrome and autoimmune diseases. Klinefelter syndrome (KS) is a sex chromosomal disorder occurring in males due to an abnormality of sexual differentiation, characterized by 47, XXY karyotype. Also, KS is related with somatic comorbidities such as metabolic syndrome, autoimmune and rheumatologic disorders as HS is. We report a HS patient with KS who shows a big improvement while on tumor necrosis factor-alpha inhibitor treatment.
Subject(s)
Humans , Male , Adalimumab , Autoimmune Diseases , Chromosome Disorders , Comorbidity , Hidradenitis Suppurativa , Hidradenitis , Karyotype , Klinefelter Syndrome , Sex Differentiation , Skin Diseases , Tumor Necrosis Factor-alphaABSTRACT
Patient education is important for successful management of atopic dermatitis; however, due to limited time and resources, patient education remains insufficient. This study aimed to investigate the current state of education provided by Korean dermatologists, pediatric allergists, and allergists to patients with atopic dermatitis. A questionnaire survey consisting of items regarding educational programs for patients with atopic dermatitis was conducted via e-mail. In total, 153 participants responded to the questionnaires, and 26.8% indicated that they have had separate educational programs. The workforce involved in the educational program included nurses, residents or fellows, dieticians, pharmacists, and clinical psychologists. Most education protocols addressed the characteristics and natural course of atopic dermatitis and environmental management. Overall, 96.7% of the participants replied that an additional charge is needed for education; moreover, additional assistance from an academic society or association, in the form of medical staff, organized data, and advertisement, is required to develop and provide a well-structured educational program. A standardized education protocol will effectively provide appropriate education for patients with atopic dermatitis. Arrangement of education fees, covered by the National Health Insurance Service, will lead to the establishment of a structured educational program and participation of an additional medical workforce.
Subject(s)
Humans , Dermatitis, Atopic , Education , Electronic Mail , Fees and Charges , Korea , Medical Staff , National Health Programs , Nutritionists , Patient Education as Topic , Pharmacists , PsychologyABSTRACT
BACKGROUND: Understanding the epidemiology of anaphylaxis is imperative for appropriate diagnosis and treatment, but the prevalence reportedly varies and only a few studies have compared the clinical features of anaphylaxis with the underlying causes in Korea. OBJECTIVE: This study aimed to investigate the etiology and clinical features of anaphylaxis. METHODS: We retrospectively reviewed the medical records of 319 anaphylaxis patients who visited our emergency room and extracted information on the causes, clinical characteristics, and subsequent outpatient visits. RESULTS: Food, drugs, and environmental factors were common causes of anaphylaxis. Statistically significant differences (p<0.001) were observed between children (<18 years of age) and adults (≥18 years of age), with food and drugs identified as the most common causes, respectively. Clinical characteristics of the patients were compared according to the common causes of anaphylaxis. Cutaneous symptoms were observed more frequently in food-induced cases (n=137, 95.1%) compared to drug-induced (n=73, 77.7%) and bee sting-induced (n=18, 78.3%) cases (p<0.001), whereas neurological symptoms were observed more frequently in drug-induced (n=37, 39.4%) and bee sting-induced (n=11, 47.8%) cases than in food-induced (n=18, 12.5%) cases (p<0.01). Drug-induced (n=44, 46.8%) and bee sting-induced (n=11, 47.8%) cases were more severe than food-induced cases (n=32, 22.2%). Out of the 319 assessed patients, only 25, 14, and 14 patients were referred to the pediatrics, allergy, and dermatology departments, respectively, after visiting the emergency room. CONCLUSION: Clinical characteristics of anaphylactic patients differed according to the underlying cause, but these findings are presumably influenced by factors determining the severity of anaphylaxis. We found that subsequent follow-up care in other departments to identify the cause of anaphylaxis was inadequate for most patients.
Subject(s)
Adult , Child , Humans , Anaphylaxis , Bees , Dermatology , Diagnosis , Emergencies , Emergency Service, Hospital , Epidemiology , Follow-Up Studies , Hypersensitivity , Korea , Medical Records , Outpatients , Pediatrics , Prevalence , Retrospective Studies , Tertiary Care CentersABSTRACT
Lenalidomide is an immunomodulatory drug used for the treatment of multiple myeloma. Several cases of hematological, gastrointestinal, and cutaneous side effects have been reported for this drug. A 67-year-old patient with multiple myeloma had initially been treated with bortezomib, but the treatment was discontinued due to neurological side effects. The chemotherapeutic regimen of this patient was changed to lenalidomide. Ten days later, erythema and pruritus developed on the entire body. The lenalidomide dose was subsequently reduced and the patient was additionally treated with topical steroids. Because lenalidomide is supplied by the Korean Orphan Drug Center, physicians have limited experience with the drug, and hence, its side effects tend to be underestimated. In addition, the Korean literature lacks reports on such cases. We describe herein a case of lenalidomide-induced drug eruption presenting as a pruritic rash covering the whole body.