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혼합결합조직질환에서 발생한 ‘소금후추가루양상 (Salt and Pepper Appearance)’의 피부색소변화 1예
원상현 ( Sang-hyeon Won ),신기혁 ( Kihyuk Shin ),김우일 ( Woo-il Kim ),양민영 ( Min-young Yang ),이원구 ( Won-ku Lee ),김훈수 ( Hoon-soo Kim ),고현창 ( Hyun-chang Ko ),김병수 ( Byung-soo Kim ),김문범 ( Moon-bum Kim ) 대한피부과학회 2020 大韓皮膚科學會誌 Vol.58 No.6
The salt and pepper appearance of the skin is composed of salt (vitiligo-like depigmentation) and pepper (perifollicular pigmentation). Mixed connective tissue disease (MCTD) is a distinct disease entity with mixed features of systemic lupus erythematosus, systemic sclerosis, myositis, and rheumatoid arthritis with high titers of antibodies to U1 ribonucleoprotein (U1RNP). There are a few reports of pigmentary changes in MCTD. A 51-year-old woman presented with variably sized hypopigmented macules along with relative hyperpigmentation of the perifollicular areas on the face and neck; she was diagnosed with MCTD in a rheumatology clinic. Histopathology revealed dermal sclerosis in both hypo- and hyperpigmented areas, loss of melanocyte and pigment only in the hypopigmented area, and normal number of melanocyte and pigment in the hyperpigmented area. To the best of our knowledge, ‘salt and pepper appearance of the skin’ has rarely been reported in MCTD. (Korean J Dermatol 2020;58(6):423∼426)
BCG 예방 접종 후 발생한 특이 피부 이상반응에 대한 임상 연구
원상현 ( Sang-hyeon Won ),신기혁 ( Kihyuk Shin ),김우일 ( Woo-il Kim ),양민영 ( Min-young Yang ),이원구 ( Won-ku Lee ),김훈수 ( Hoon-soo Kim ),김병수 ( Byung-soo Kim ),김문범 ( Moon-bum Kim ),고현창 ( Hyun-chang Ko ) 대한피부과학회 2020 대한피부과학회지 Vol.58 No.7
Background: Bacille Calmette-Guérin (BCG) vaccination has been widely used to prevent severe infections caused by Mycobacterium tuberculosis. Although BCG vaccination is generally safe, some cutaneous complications may occur. However, cutaneous complications related to BCG infection have been rarely investigated in Korea. Objective: This study aimed to investigate the clinical characteristics of cutaneous complications following BCG vaccination in Korean patients. Methods: We identified 18 patients with cutaneous complications following BCG vaccination in the Pusan National University Hospitals (Busan and Yangsan) from 2002 to 2019. Clinical manifestations and course were examined by the review of medical records and photographs. Results: Among BCG-vaccinated persons, 18 patients manifested with BCG-specific cutaneous complications. The mean age of the patients was 10.6±10.1 months, and 10 (55.6%) were female. The specific cutaneous complications following BCG vaccination were local abscess (7, 38.9%), lymphadenitis (4, 22.2%), BCG reactivation in incomplete Kawasaki disease (4, 22.2%), tuberculid (3, 16.7%), and scrofuloderma (1, 5.6%). Disseminated BCG infection was detected in 2 brothers (11.1%) who had an immunocompromised state and a family history of BCG complication. Conclusion: Specific cutaneous complications after BCG vaccination are rare, but may occur in various manifestations. (Korean J Dermatol 2020;58(7):453∼458)
중등증 및 중증의 한국인 건선 환자에서 Ixekizumab의 효과와 안전성에 관한 연구
원상현 ( Sang-hyeon Won ),신기혁 ( Kihyuk Shin ),김훈수 ( Hoon-soo Kim ),고현창 ( Hyun-chang Ko ),김문범 ( Moon-bum Kim ),김병수 ( Byung-soo Kim ) 대한피부과학회 2020 대한피부과학회지 Vol.58 No.6
Background: Ixekizumab is a humanized IgG4 monoclonal antibody that selectively binds to the IL-17A cytokine and inhibits its interaction with the IL-17 receptor. It has emerged as an effective treatment for moderate to severe psoriasis. However, to date, there are no real-world data for the use of ixekizumab in the treatment of psoriasis in Korean patients. Objective: To evaluate the effectiveness and safety of ixekizumab in the treatment of moderate to severe psoriasis in Korean patients. Methods: This was a retrospective single-center study. Thirty psoriasis patients who were treated with ixekizumab were analyzed. All patients’ medical records, Psoriasis Area and Severity Index (PASI) score, body surface area (BSA), Physicians’ Global Assessment (PGA), and adverse events were investigated. Results: A significant reduction in mean (±standard deviation) baseline PASI score (14.1±2.6) was detected at 4 weeks of ixekizumab therapy (3.8±2.7, p<0.001), with a further improvement at weeks 12 and 24 (0.9±0.7 and 0.5±0.5, respectively) (p<0.001). Our analysis showed that 100%, 87.5%, and 50% of patients achieved PASI 75, 90, and 100 responses, respectively, after 48 weeks of therapy. However, nine patients (30%) experienced a mild adverse event such as injection site reaction, urticaria, upper respiratory tract infection, and stomatitis. No serious adverse events were observed. Conclusion: This study provides evidence for the use of ixekizumab in real-world clinical practice and confirm that it is effective and safe in treating Korean patients with moderate to severe psoriasis. (Korean J Dermatol 2020;58(6):389∼396)
이기욱 ( Gi-wook Lee ),김연아 ( Yeona Kim ),원상현 ( Sang-hyeon Won ),배경남 ( Kyung-nam Bae ),이정수 ( Jungsoo Lee ),신기혁 ( Kihyuk Shin ),김훈수 ( Hoon-soo Kim ),김병수 ( Byung-soo Kim ),김문범 ( Moon-bum Kim ),고현창 ( Hyun-ch 대한피부과학회 2023 대한피부과학회지 Vol.61 No.4
The phenotypic variability of variant Turner syndrome is wide, ranging from characteristic clinical features to those that are hardly distinguishable from the general population. A 4-year-old girl presented with multiple brownish macules and patches on the trunk and upper extremities as well as axillary freckles. Exome sequencing and chromosomal microarray testing revealed a microdeletion at Xp22.33p22.11 leading to a diagnosis of Turner syndrome. Here we describe an unusual case of variant Turner syndrome with multiple café-au-lait spots. (Korean J Dermatol 2023;61(4):244∼247)
김종욱 ( Jongwook Kim ),이기욱 ( Giwook Lee ),신준오 ( Jun-oh Shin ),노동영 ( Dong Young Roh ),김연아 ( Yeona Kim ),원상현 ( Sang-hyeon Won ),이정수 ( Jungsoo Lee ),신기혁 ( Kihyuk Shin ),김훈수 ( Hoonsoo Kim ),고현창 ( Hyun-chang 대한피부과학회 2024 대한피부과학회지 Vol.62 No.3
Congenital smooth muscle hamartoma is a benign proliferation of smooth muscles within the dermis. The classic form presents as well-defined, skin-colored, or hyperpigmented plaques associated with hypertrichosis. However, there have been reports of atypical forms, including a follicular spotted appearance, linear atrophic plaques, and morphea-like forms. In such cases, distinguishing congenital smooth muscle hamartomas from other cutaneous diseases can be challenging. Herein, we report on a 16-month-old boy who presented with a hypopigmented patch and hypertrichosis on his back since birth. Histopathological examination revealed mild acanthosis and well-defined smooth muscle bundles haphazardly oriented in the dermis. These bundles stained positively with Masson’s trichrome stain. Based on these findings, a definitive diagnosis of congenital smooth muscle hamartoma was established. In conclusion, an exceptionally rare case of congenital smooth muscle hamartoma with a hypopigmented appearance is reported. (Korean J Dermatol 2024;62(3):165∼168)
전기외과술로 치료한 안면의 다발성 피부 Rosai-Dorfman Disease 1예
이진수 ( Jinsu Lee ),이기욱 ( Gi-wook Lee ),신준오 ( Jun-oh Shin ),노동영 ( Dongyoung Roh ),김연아 ( Yeona Kim ),원상현 ( Sang-hyeon Won ),이정수 ( Jungsoo Lee ),신기혁 ( Kihyuk Shin ),김훈수 ( Hoonsoo Kim ),고현창 ( Hyunchang Ko 대한피부과학회 2023 대한피부과학회지 Vol.61 No.10
Cutaneous Rosai-Dorfman disease (RDD) is a rare, benign condition mainly affecting the lymph nodes and sometimes involving the skin. RDD is notorious for its poor response to medication, and surgery is often the preferred treatment option for limited lesions. Herein, we present a case of cutaneous RDD in a 49-year-old woman with multiple facial nodules that showed minimal response to treatment with steroids, isotretinoin, and methotrexate. The diagnosis of cutaneous RDD was confirmed based on histopathological examination. The patient showed minimal response to medication; however, electrosurgery resulted in a relatively good cosmetic response. The present case demonstrates that electrosurgery can be an effective treatment option for cutaneous RDD, especially in case of patients with multiple lesions. The rarity of this disease and the limited reports on its management highlights the need for further research on the efficacy of different surgical modalities for cutaneous RDD. (Korean J Dermatol 2023;61(10):639∼642)
여성형탈모에서 저용량 경구 미녹시딜의 효과 및 안정성: 44명의 환자를 대상으로 한 단일기관 연구
김영범 ( Youngbeom Kim ),이기욱 ( Giwook Lee ),신준오 ( Jun-oh Shin ),노동영 ( Dong Young Roh ),김연아 ( Yeona Kim ),원상현 ( Sang-hyeon Won ),이정수 ( Jungsoo Lee ),신기혁 ( Kihyuk Shin ),김훈수 ( Hoonsoo Kim ),고현창 ( Hyun-chan 대한피부과학회 2024 대한피부과학회지 Vol.62 No.3
Background: Female-pattern hair loss (FPHL) is characterized by diffuse hair thinning in the mid-frontal scalp and increased hair shedding. Although the use of low-dose oral minoxidil (LDOM) is increasing significantly in Korea, data on its use are limited. Objective: To determine the efficacy and safety of LDOM in Korean patients with FPHL. Methods: This retrospective, single-center study was conducted at Pusan National University Hospital. The study included female patients with pattern hair loss who received treatment with LDOM at 1.25 mg/d for a minimum of 6 months in combination with other treatments. Patients were eligible for LDOM addition if their previous treatments showed a limited response and the previous treatment regimen remained unchanged throughout the evaluation period. Clinical response to treatment was evaluated using the Sinclair hair loss severity scale. Results: The study included 44 females with FPHL. Before treatment, the mean Sinclair scale score was 2.77, which decreased to 2.27 after treatment. By the 3rd month, one patient (2.3%) experienced worsening, while seven patients (15.9%) showed slight improvement. By the 6th month, the treatment response was as follows: stabilization in 30 patients (68.2%), slight improvement in 13 patients (29.5%), and substantial improvement in one patient (2.3%). Adverse effects, predominantly hypertrichosis, were observed in nine patients. All adverse effects improved upon discontinuation of LDOM and no life-threatening adverse effects were observed during the study. Conclusion: This study provides evidence that LDOM can be an effective therapeutic option with a good safety profile for FPHL. (Korean J Dermatol 2024;62(3):136∼142)