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대한피부과학회> 대한피부과학회지> 증례 : 선상의 피부 위축으로 진단한 MIDAS 증후군 1예

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증례 : 선상의 피부 위축으로 진단한 MIDAS 증후군 1예

Case Report : MIDAS Syndrome Presenting with Linear Skin Atrophy on the Face

임슬기 ( Seul Ki Lim ) , 이영 ( Young Lee ) , 서영준 ( Young Joon Seo ) , 이증훈 ( Jeung Hoon Lee ) , 임명 ( Myung Im )
  • : 대한피부과학회
  • : 대한피부과학회지 53권5호
  • : 연속간행물
  • : 2015년 06월
  • : 381-383(3pages)

DOI


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MIDAS syndrome (microphthalmia-dermal aplasia-sclerocornea) is an X-linked dominant genetic disease. In most patients, the unbalanced translocation or deletion of the X chromosome short-arm 22.3 band is observed. This disease characteristically presents as linear atrophy of the skin limited to the face and neck, accompanied by congenital eye disease. A 9-month-old female who had linear skin atrophy on the right side of her chin visited our clinic. She also presented with microphthalmia and sclerocornea on her right eye. Results of a chromosomal study revealed a deletion of the X-chromosome short-arm 22.31 band. Here, we report on this MIDAS syndrome patient with linear skin atrophy on the face. (Korean J Dermatol 2015;53(5):381∼383)

UCI(KEPA)

I410-ECN-0102-2015-500-001916385

간행물정보

  • : 의약학분야  > 피부과학
  • : KCI등재
  • : SCOPUS
  • : 월간
  • : 0494-4739
  • : 2713-7627
  • : 학술지
  • : 연속간행물
  • : 1960-2020
  • : 9462


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1원저 : 접촉 피부염 환자에서 모메타손 크림의 피부 장벽 기능에 미치는 영향에 대한 평가

저자 : 소병준 ( Byoung Joon So ) , 박재범 ( Jae Beom Park ) , 천승현 ( Seung Hyun Chun ) , 손상욱 ( Sang Wook Son )

발행기관 : 대한피부과학회 간행물 : 대한피부과학회지 53권 5호 발행 연도 : 2015 페이지 : pp. 347-350 (4 pages)

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Background: It is unclear how the usage of topical steroid agents affects skin barrier function. Objective: In order to follow up on previous research into this topic, we sought to investigate the effects of a 3-week application of topical mometasone cream on the alteration of skin barrier function. Methods: Twenty-six patients who had been clinically diagnosed with allergic contact dermatitis were enrolled. Topical mometasone cream was applied to the skin lesions. Clinical symptoms, transepidermal water loss (TEWL), corneometer unit, and pH value were measured on the initial visit, 1 week after treatment, and 3 weeks after treatment, and their values were compared. Results: Clinical symptoms showed improvement after topical mometasone cream was applied (p<0.05), but changes in TEWL, corneometer units, and pH values failed to show statistical significance (p>0.05). Conclusion: This study found that treatment with topical mometasone cream for 3 weeks has no effect on skin barrier function. We believe that this research will help determine the optimal duration and dosage of topical steroid agents used for treating allergic contact dermatitis. (Korean J Dermatol 2015;53(5):347∼350)

2원저 : 급성기 대상포진에 시행한 중재적 통증 치료술의 효과

저자 : 장용현 ( Yong Hyun Jang ) , 문선영 ( Sun Young Moon ) , 채수열 ( Soo Yuhl Chae ) , 이원주 ( Weon Ju Lee ) , 이석종 ( Seok Jong Lee ) , 김신우 ( Shin Woo Kim ) , 박기범 ( Ki Bum Park ) , 전영훈 ( Young Hoon Jeon ) , 김도원 ( Do Won Kim )

발행기관 : 대한피부과학회 간행물 : 대한피부과학회지 53권 5호 발행 연도 : 2015 페이지 : pp. 351-358 (8 pages)

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Background: Although several interventional pain management procedures (IPs) for reducing the acute herpes zoster (HZ)-related pain have shown some level of effectiveness on early pain relief and the prevention of postherpetic neuralgia (PHN), no conclusive evidence has been presented to support their effectiveness. Objective: We evaluated the effectiveness of IPs during the acute phase of HZ for reducing HZ-related pain. Methods: Sixty-one patients with acute HZ were assessed using the pain visual analogue scale (VAS) that ranges from 0 to 10 at the initial visit and after 1, 3 and 6 months. Changes in pain VAS and the incidence of PHN (pain after 1 month) were compared between 2 patient groups: those who received standard therapy with oral antivirals and analgesics (ST, n=38) and those who received standard therapy with IPs (STIPs, n=23). PHN was defined as either “pain of 1 or higher in pain VAS” or “clinically meaningful PHN (pain of 3 or higher in pain VAS).” Results: Although the initial pain VAS level of patients treated with STIPs (5.74) was higher than that of patients receiving ST (4.09), no significant difference in pain VAS number was seen between the 2 groups at 3 months (0.13 vs. 0.17) and 6 months (0.09 vs. 0.03) of follow-up. The incidence of PHN also was not statistically significant different between the 2 groups at 3 (9.5% vs. 8.3%) and 6 months (9.5% vs. 4.2%). A similar trend was observed in the analysis of HZ patients whose pain VAS level was 3 or higher at the initial assessment. Conclusion: Standard therapy with early IPs is effective for rapidly reducing HZ-related pain. (Korean J Dermatol 2015;53(5):351∼358)

3원저 : 피부과로 의뢰된 정신건강의학과 입원환자에 대한 임상양상 분석

저자 : 황상현 ( Sang Hyeon Hwang ) , 남재희 ( Jae Hui Nam ) , 박지혜 ( Ji Hye Park ) , 이가영 ( Ga Young Lee ) , 김원석 ( Won Serk Kim )

발행기관 : 대한피부과학회 간행물 : 대한피부과학회지 53권 5호 발행 연도 : 2015 페이지 : pp. 359-365 (7 pages)

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Background: Many patients seeking treatment for skin disorders have an underlying psychiatric problem that either causes or exacerbates a skin condition; thus, the demand for dermatologists` expert opinions on such cases is increasing. Objective: The purpose of this study was to analyze and quantify the pattern of dermatologic consultations referred by the department of neuropsychiatry in a tertiary hospital. Methods: We retrospectively studied data from 337 inpatients referred by the department of neuropsychiatry for dermatologic consultation during an 8.5-year period in a tertiary hospital. Results: The percentage of department of neuropsychiatry patients who were referred to the department of dermatology for consultation was 15.3%. The most prevalent age group for referrals was 30∼39 years (26.4%), and the male-to-female ratio was 1:1.6. Bipolar disorder was the most common underlying neuropsychiatric problem (31.1%), and eczema (36.5%) was the most common dermatoses, followed by infectious disease (27.6%). The main reasons for dermatologic consultations were dermatologic disease (71.5%) and treatment-related dermatoses (10.1%). Thirty-five cases were discordant as to the reason for consultation between psychiatrists and dermatologists. Neuropsychiatric medication-related adverse reactions were reported in 9.8% of referred patients. Conclusion: This study revealed the distribution of skin disorders in patients referred by department of neuropsychiatry for dermatologic consultation. We believe its findings could be helpful as educational material to encourage cooperation between the specialties of dermatology and neuropsychiatry. (Korean J Dermatol 2015;53(5): 359∼365)

4원저 : 한국인 중등증 및 중증 건선 환자에서 유스테키누맵 치료의 효능 및 안전성에 대한 연구

저자 : 김경오 ( Kyung O Kim ) , 이상훈 ( Sang Hoon Lee ) , 이성열 ( Sung Yul Lee ) , 황규왕 ( Kyu Uang Whang ) , 박영립 ( Young Lip Park )

발행기관 : 대한피부과학회 간행물 : 대한피부과학회지 53권 5호 발행 연도 : 2015 페이지 : pp. 366-373 (8 pages)

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Background: Psoriasis is a chronic, relapsing inflammatory disease that affects approximately 2∼3% of the population worldwide and often requires lifelong care. Recent advances in understanding the immunogenesis of psoriasis has led to the development of biological agents that target specific immunological pathways. Ustekinumab is a human monoclonal antibody that binds to the p40 subunit common to interleukin-12 and 23, key cytokines in the pathogenesis of psoriasis. Objective: This study aims to address the efficacy and safety of ustekinumab in Korean patients with moderateto- severe psoriasis. Methods: The clinical records of 32 consecutive patients treated with ustekinumab were reviewed retrospectively. Treatment effectiveness was estimated based on reported Psoriasis Area and Severity Index (PASI) 50, 75, and 90 response rates, defined as a ≥50%, ≥75%, or ≥90% reduction from baseline PASI scores, respectively. A stereotyped questionnaire was completed by the physician, and information about adverse events and quality of life was collected. Results: The average baseline PASI score was 25.7. Overall 38%, 56%, and 80% patients achieved PASI 75 response rates at weeks 4, 16, and 52 respectively. Thirteen patients (41%) experienced a mild adverse event such as upper respiratory infection, pruritus, urticaria, nasopharyngitis, headache, hyperglycemia, abnormal hepatic function, or arthralgia. Conclusion: Ustekinumab provides an effective, safe, and well-tolerated alternative for the symptomatic treatment of Korean patients with moderate-to-severe psoriasis. (Korean J Dermatol 2015;53(5):366∼373)

5원저 : 급성 및 만성 두드러기 환자에서 MAST 화학발광법 양성율과 알레르기 항원 검출율에 대한 비교 연구

저자 : 정희철 ( Hee Chul Chung ) , 안성구 ( Sung Ku Ahn )

발행기관 : 대한피부과학회 간행물 : 대한피부과학회지 53권 5호 발행 연도 : 2015 페이지 : pp. 374-380 (7 pages)

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Background: The multiple allergosorbent test chemiluminescent assay (MAST-CLA) is a simple system with high sensitivity and specificity for measuring total and allergen-specific IgE antibodies in patients with allergic skin diseases such as urticaria. Objective: The purpose of this study was to evaluate total IgE class, allergen frequencies, and their correlations in MAST-CLA in acute and chronic urticaria. Methods: We performed a retrospective study using medical documents of 2,028 urticaria patients (average age= 34.85 years) who visited Yonsei Wonju Severance Christian Hospital, Yeongseo Province, Korea, between 2003 and 2012. Positive rates of specific IgE and total serum IgE levels in acute and chronic urticaria patients were analyzed. Results: Among the total 2,028 patients, 1,263 (62.3%) had acute urticaria, and 765 (37.7%) had chronic urticaria. The number of patients with higher than class 2 level of serum total IgE was 1,496 (73.8%): 964 (76.3%) of the acute urticaria group and 532 (69.5%) of the chronic urticaria group. More than half of the patients (1,048; 51.7%) showed a positive reaction to at least one allergen-specific IgE. The positive rate of acute urticaria was 53.0% and chronic urticaria 49.5%. In both acute and chronic urticaria, the most commonly detected allergen was Dermatophagoides farinae, followed by D. pterony and house dust. Most patients showed lower positive reactions to food allergens. Peach was the most common food allergen in both groups. Of the patients who showed positive total IgE, 40.4% did not react to any allergen-specific IgE. Also, 26.3% of the patients who were negative for total IgE showed a positive reaction to more than one allergen-specific IgE. The level of total IgE and the number of positive allergen-specific IgE responses were positively correlated in both acute and chronic urticaria patients. Conclusion: Our results showed that the positive rate of total IgE was higher in acute urticaria patients than in chronic urticaria patients. Both acute and chronic urticaria patient groups showed the highest positive rates for D. farinae. Positive rates for response to food allergens, such as soybean, chicken, yeast, tuna, and salmon, were lower in the chronic urticaria group. For inhalant allergens, the positive rates of response to white oak and Aspergillus were higher in the acute urticaria group. The level of total IgE and the number of positive allergen-specific IgE responses showed a positive correlation in both groups. Thus, MAST-CLA appears to be a simple and convenient method for determining causative allergens in the occurrence and exacerbation of urticaria, but other studies are necessary to confirm these preliminary findings. (Korean J Dermatol 2015;53(5):374∼380)

6증례 : 선상의 피부 위축으로 진단한 MIDAS 증후군 1예

저자 : 임슬기 ( Seul Ki Lim ) , 이영 ( Young Lee ) , 서영준 ( Young Joon Seo ) , 이증훈 ( Jeung Hoon Lee ) , 임명 ( Myung Im )

발행기관 : 대한피부과학회 간행물 : 대한피부과학회지 53권 5호 발행 연도 : 2015 페이지 : pp. 381-383 (3 pages)

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MIDAS syndrome (microphthalmia-dermal aplasia-sclerocornea) is an X-linked dominant genetic disease. In most patients, the unbalanced translocation or deletion of the X chromosome short-arm 22.3 band is observed. This disease characteristically presents as linear atrophy of the skin limited to the face and neck, accompanied by congenital eye disease. A 9-month-old female who had linear skin atrophy on the right side of her chin visited our clinic. She also presented with microphthalmia and sclerocornea on her right eye. Results of a chromosomal study revealed a deletion of the X-chromosome short-arm 22.31 band. Here, we report on this MIDAS syndrome patient with linear skin atrophy on the face. (Korean J Dermatol 2015;53(5):381∼383)

7증례 : 손발의 변연부에 발생한 국소말단과다각화증

저자 : 김예슬 ( Ye Seul Kim ) , 정가희 ( Ga Hee Jung ) , 조문균 ( Moon Kyun Cho ) , 이상훈 ( Sang Hoon Lee ) , 이현주 ( Hyun Ju Lee ) , 이종석 ( Jong Suk Lee ) , 이성열 ( Sung Yul Lee )

발행기관 : 대한피부과학회 간행물 : 대한피부과학회지 53권 5호 발행 연도 : 2015 페이지 : pp. 384-387 (4 pages)

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Focal acral hyperkeratosis is a rare skin disorder, initially described by Dowd et al., which is sporadic or inherited in an autosomal dominant way. Clinically, the condition presents with small yellowish to white papules located on lateral aspects of the hands and feet. Histopathological alterations are limited to the epidermis and there are no changes in the elastic fibers of the dermis. In this case report, a 14-year-old girl had a two-year history of persistent, asymptomatic, multiple papules along the border of the hands and feet. A histologic section of papules from the left hand and foot showed marked hyperkeratosis and hypergranulosis of epidermis. (Korean J Dermatol 2015;53(5):384∼387)

8증례 : 위장관 기질종양 환자에서 Imatinib Mesylate 투여 후 발생한 건선양 약진 1예

저자 : 이예진 ( Ye Jin Lee ) , 이우진 ( Woo Jin Lee ) , 장성은 ( Sung Eun Chang ) , 이미우 ( Mi Woo Lee ) , 최지호 ( Jee Ho Choi ) , 문기찬 ( Kee Chan Moon )

발행기관 : 대한피부과학회 간행물 : 대한피부과학회지 53권 5호 발행 연도 : 2015 페이지 : pp. 388-391 (4 pages)

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Imatinib mesylate (Gleevecⓡ) is a small-molecule inhibitor that selectively inhibits the tyrosine kinase family, including mutated KIT oncoproteins in gastrointestinal stromal tumors (GIST). However, cutaneous reactions to imatinib are common and occur in 7.0% to 88.9% of patients. Nonspecific skin rashes, facial edema, and pruritus are the most common adverse reactions. However, development of psoriasiform drug eruption owing to the drug has rarely been reported. Herein we report on a 66-year-old male patient with GIST who had taken imatinib (400 mg/day) for 2 months. He developed erythematous scaly macules and papules on the trunk and extremities. Histopathological findings were compatible with a psoriasiform drug eruption. (Korean J Dermatol 2015;53(5):388∼391)

9증례 : 요추천자 부위에 발생한 단발성 수포성 비만세포종 1예

저자 : 최승환 ( Seung Hwan Choi ) , 김태훈 ( Tae Hoon Kim ) , 하승민 ( Seung Min Ha ) , 고동엽 ( Dong Yeob Ko ) , 송기훈 ( Ki Hoon Song ) , 김기호 ( Ki Ho Kim )

발행기관 : 대한피부과학회 간행물 : 대한피부과학회지 53권 5호 발행 연도 : 2015 페이지 : pp. 392-395 (4 pages)

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Mastocytosis is characterized by an accumulation of mast cells in various organs, most frequently in the skin. A solitary mastocytoma is a clinical variant of cutaneous mastocytosis. It is defined as a localized collection of mast cells in the skin without evidence of extracutaneous organ involvement. Here we report on a 2-year-old female patient presenting with Solitary erythematous bulla on her lower back. The patient had a history of spinal tap on the lower back for evaluation of meningitis at 5 months of age, which resulted in trauma at the site. Histopathology showed mast cells infiltrating the papillary and reticular dermis and metachromatic purple cytoplasmic granules seen with Giemsa staining. As a result, the patient was diagnosed with a solitary bullous mastocytoma and administered antihistamine. The patient showed complete remission at 3 months. Herein, we report a rare case of solitary bullous mastocytoma occurring at a trauma site. (Korean J Dermatol 2015;53(5):392∼395)

10증례 : 소아에서 발병한 세포형 얼기모양 섬유조직구성 종양

저자 : 김지영 ( Jee Young Kim ) , 최미수 ( Mi Soo Choi ) , 남찬희 ( Chan Hee Nam ) , 김지석 ( Ji Seok Kim ) , 김명화 ( Myung Hwa Kim ) , 박병철 ( Byung Cheol Park ) , 홍승필 ( Seung Phil Hong )

발행기관 : 대한피부과학회 간행물 : 대한피부과학회지 53권 5호 발행 연도 : 2015 페이지 : pp. 396-399 (4 pages)

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Plexiform fibrohistiocytic tumor (PFHT) is a rare mesenchymal neoplasm of intermediate malignancy and possibly of myofibroblast origin. It is morphologically divided into 3 groups: cellular, fibrous, and mixed. A 4-year-old girl presented with an irregular shaped subcutaneous mass on her left popliteal fossa for 6 months. The biopsy specimen showed multinodular tumor islands extending from dermis to subcutaneous layer, composed of histiocytes and osteoclast-like multinucleated giant cells, and circumscribed by fibrous tissue. Immunohistochemical staining was positive for CD68 in giant cells and histiocyte-like cells within tumor islands and faintly positive for smooth muscle actin around nodules. A cellular variant of PFHT was eventually diagnosed. Although PFHT comprises morphologically normal cells, it has the biological potential for malignant change and distal metastasis. Therefore, PFHT is categorized as a neoplasm of intermediate malignancy, and wide total excision with close follow-up is crucial. (Korean J Dermatol 2015;53(5):396∼399)

12
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간행물명 수록권호

KCI등재 SCI SCOUPUS

Annals of Dermatology
33권 1호 ~ 33권 1호

프로그램북(구 초록집)
72권 2호 ~ 72권 2호

KCI등재 SCOUPUS

대한피부과학회지
58권 10호 ~ 58권 10호

KCI등재 SCOUPUS

대한피부과학회지
58권 9호 ~ 58권 9호

KCI등재 SCI SCOUPUS

Annals of Dermatology
32권 6호 ~ 32권 6호

KCI등재 SCI SCOUPUS

Annals of Dermatology
32권 5호 ~ 32권 5호

KCI등재 SCOUPUS

대한피부과학회지
58권 8호 ~ 58권 8호

KCI등재

대한화장품학회지(J. Soc. Cosmet. Scientists Korea)
46권 3호 ~ 46권 3호

KCI등재 SCOUPUS

대한피부과학회지
58권 7호 ~ 58권 7호

KCI등재 SCI SCOUPUS

Annals of Dermatology
32권 4호 ~ 32권 4호

KCI등재 SCOUPUS

대한피부과학회지
58권 6호 ~ 58권 6호

KCI등재

대한화장품학회지(J. Soc. Cosmet. Scientists Korea)
46권 2호 ~ 46권 2호

KCI등재 SCOUPUS

대한피부과학회지
58권 5호 ~ 58권 5호

KCI등재 SCI SCOUPUS

Annals of Dermatology
32권 3호 ~ 32권 3호

KCI등재 SCOUPUS

대한피부과학회지
58권 4호 ~ 58권 4호

KCI등재

대한화장품학회지(J. Soc. Cosmet. Scientists Korea)
46권 1호 ~ 46권 1호

KCI등재 SCOUPUS

대한피부과학회지
58권 3호 ~ 58권 3호

KCI등재 SCI SCOUPUS

Annals of Dermatology
32권 2호 ~ 32권 2호

KCI등재 SCOUPUS

대한피부과학회지
58권 2호 ~ 58권 2호

KCI등재

대한화장품학회지(J. Soc. Cosmet. Scientists Korea)
45권 4호 ~ 45권 4호
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