1.A Case of Type 1 Segmental Darier’s Disease with Bilateral Presentation
Youngbeom KIM ; Gi-Wook LEE ; Jun-Oh SHIN ; Dongyoung ROH ; Jungsoo LEE ; Kihyuk SHIN ; Hoon-Soo KIM ; Hyun-Chang KO ; Moon-Bum KIM ; Byungsoo KIM
Korean Journal of Dermatology 2025;63(1):1-4
Darier’s disease is characterized by greasy and scaly papules that primarily affect seborrheic and intertriginous areas which is caused by a mutation in the ATP2A2 gene. Histopathologically, the disease is characterized by acantholysis and dyskeratosis. Among the diverse presentations, the segmental type follows a linear distribution along the lines of Blaschko. Herein, we present a case of a 54-year-old male with generalized erythematous papules that had been linearly distributed across his body for two decades. Lesions on his trunk and extremities were confined to the right side, whereas those on the scalp and face exhibited multiple segmental presentations. Histopathological examination revealed acantholysis and dyskeratosis in the epidermis, confirming the diagnosis of type 1 segmental Darier’s disease. This case underscores the rarity of type 1 segmental Darier’s disease, particularly with multiple segmental involvement and highlights the complexity and variability of this dermatological condition.
2.A Case of Type 1 Segmental Darier’s Disease with Bilateral Presentation
Youngbeom KIM ; Gi-Wook LEE ; Jun-Oh SHIN ; Dongyoung ROH ; Jungsoo LEE ; Kihyuk SHIN ; Hoon-Soo KIM ; Hyun-Chang KO ; Moon-Bum KIM ; Byungsoo KIM
Korean Journal of Dermatology 2025;63(1):1-4
Darier’s disease is characterized by greasy and scaly papules that primarily affect seborrheic and intertriginous areas which is caused by a mutation in the ATP2A2 gene. Histopathologically, the disease is characterized by acantholysis and dyskeratosis. Among the diverse presentations, the segmental type follows a linear distribution along the lines of Blaschko. Herein, we present a case of a 54-year-old male with generalized erythematous papules that had been linearly distributed across his body for two decades. Lesions on his trunk and extremities were confined to the right side, whereas those on the scalp and face exhibited multiple segmental presentations. Histopathological examination revealed acantholysis and dyskeratosis in the epidermis, confirming the diagnosis of type 1 segmental Darier’s disease. This case underscores the rarity of type 1 segmental Darier’s disease, particularly with multiple segmental involvement and highlights the complexity and variability of this dermatological condition.
3.A Case of Type 1 Segmental Darier’s Disease with Bilateral Presentation
Youngbeom KIM ; Gi-Wook LEE ; Jun-Oh SHIN ; Dongyoung ROH ; Jungsoo LEE ; Kihyuk SHIN ; Hoon-Soo KIM ; Hyun-Chang KO ; Moon-Bum KIM ; Byungsoo KIM
Korean Journal of Dermatology 2025;63(1):1-4
Darier’s disease is characterized by greasy and scaly papules that primarily affect seborrheic and intertriginous areas which is caused by a mutation in the ATP2A2 gene. Histopathologically, the disease is characterized by acantholysis and dyskeratosis. Among the diverse presentations, the segmental type follows a linear distribution along the lines of Blaschko. Herein, we present a case of a 54-year-old male with generalized erythematous papules that had been linearly distributed across his body for two decades. Lesions on his trunk and extremities were confined to the right side, whereas those on the scalp and face exhibited multiple segmental presentations. Histopathological examination revealed acantholysis and dyskeratosis in the epidermis, confirming the diagnosis of type 1 segmental Darier’s disease. This case underscores the rarity of type 1 segmental Darier’s disease, particularly with multiple segmental involvement and highlights the complexity and variability of this dermatological condition.
4.A Case of Type 1 Segmental Darier’s Disease with Bilateral Presentation
Youngbeom KIM ; Gi-Wook LEE ; Jun-Oh SHIN ; Dongyoung ROH ; Jungsoo LEE ; Kihyuk SHIN ; Hoon-Soo KIM ; Hyun-Chang KO ; Moon-Bum KIM ; Byungsoo KIM
Korean Journal of Dermatology 2025;63(1):1-4
Darier’s disease is characterized by greasy and scaly papules that primarily affect seborrheic and intertriginous areas which is caused by a mutation in the ATP2A2 gene. Histopathologically, the disease is characterized by acantholysis and dyskeratosis. Among the diverse presentations, the segmental type follows a linear distribution along the lines of Blaschko. Herein, we present a case of a 54-year-old male with generalized erythematous papules that had been linearly distributed across his body for two decades. Lesions on his trunk and extremities were confined to the right side, whereas those on the scalp and face exhibited multiple segmental presentations. Histopathological examination revealed acantholysis and dyskeratosis in the epidermis, confirming the diagnosis of type 1 segmental Darier’s disease. This case underscores the rarity of type 1 segmental Darier’s disease, particularly with multiple segmental involvement and highlights the complexity and variability of this dermatological condition.
7.Effectiveness and Safety of Low-Dose Oral Minoxidil in Female Pattern Hair Loss:A Single-Center Study of 44 Patients
Youngbeom KIM ; Giwook LEE ; Jun-Oh SHIN ; Dong Young ROH ; Yeona KIM ; Sang-Hyeon WON ; Jungsoo LEE ; Kihyuk SHIN ; Hoonsoo KIM ; Hyun-Chang KO ; Byung Soo KIM ; Moon-Bum KIM
Korean Journal of Dermatology 2024;62(3):136-142
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.
8.The Korean Society for Neuro-Oncology (KSNO) Guideline for the Management of Brain Tumor Patients During the Crisis Period: A Consensus Survey About Specific Clinical Scenarios (Version 2023.1)
Min-Sung KIM ; Se-Il GO ; Chan Woo WEE ; Min Ho LEE ; Seok-Gu KANG ; Kyeong-O GO ; Sae Min KWON ; Woohyun KIM ; Yun-Sik DHO ; Sung-Hye PARK ; Youngbeom SEO ; Sang Woo SONG ; Stephen AHN ; Hyuk-Jin OH ; Hong In YOON ; Sea-Won LEE ; Joo Ho LEE ; Kyung Rae CHO ; Jung Won CHOI ; Je Beom HONG ; Kihwan HWANG ; Chul-Kee PARK ; Do Hoon LIM ;
Brain Tumor Research and Treatment 2023;11(2):133-139
Background:
During the coronavirus disease 2019 (COVID-19) pandemic, there was a shortage of medical resources and the need for proper treatment guidelines for brain tumor patients became more pressing. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, has undertaken efforts to develop a guideline that is tailored to the domestic situation and that can be used in similar crisis situations in the future. As part II of the guideline, this consensus survey is to suggest management options in specific clinical scenarios during the crisis period.
Methods:
The KSNO Guideline Working Group consisted of 22 multidisciplinary experts on neuro-oncology in Korea. In order to confirm a consensus reached by the experts, opinions on 5 specific clinical scenarios about the management of brain tumor patients during the crisis period were devised and asked. To build-up the consensus process, Delphi method was employed.
Results:
The summary of the final consensus from each scenario are as follows. For patients with newly diagnosed astrocytoma with isocitrate dehydrogenase (IDH)-mutant and oligodendroglioma with IDH-mutant/1p19q codeleted, observation was preferred for patients with low-risk, World Health Organization (WHO) grade 2, and Karnofsky Performance Scale (KPS) ≥60, while adjuvant radiotherapy alone was preferred for patients with high-risk, WHO grade 2, and KPS ≥60. For newly diagnosed patients with glioblastoma, the most preferred adjuvant treatment strategy after surgery was radiotherapy plus temozolomide except for patients aged ≥70 years with KPS of 60 and unmethylated MGMT promoters. In patients with symptomatic brain metastasis, the preferred treatment differed according to the number of brain metastasis and performance status. For patients with newly diagnosed atypical meningioma, adjuvant radiation was deferred in patients with older age, poor performance status, complete resection, or low mitotic count.
Conclusion
It is imperative that proper medical care for brain tumor patients be sustained and provided, even during the crisis period. The findings of this consensus survey will be a useful reference in determining appropriate treatment options for brain tumor patients in the specific clinical scenarios covered by the survey during the future crisis.
9.The Korean Society for Neuro-Oncology (KSNO) Guideline for the Management of Brain Tumor Patients During the Crisis Period: A Consensus Recommendation Using the Delphi Method (Version 2023.1)
Min-Sung KIM ; Se-Il GO ; Chan Woo WEE ; Min Ho LEE ; Seok-Gu KANG ; Kyeong-O GO ; Sae Min KWON ; Woohyun KIM ; Yun-Sik DHO ; Sung-Hye PARK ; Youngbeom SEO ; Sang Woo SONG ; Stephen AHN ; Hyuk-Jin OH ; Hong In YOON ; Sea-Won LEE ; Joo Ho LEE ; Kyung Rae CHO ; Jung Won CHOI ; Je Beom HONG ; Kihwan HWANG ; Chul-Kee PARK ; Do Hoon LIM ;
Brain Tumor Research and Treatment 2023;11(2):123-132
Background:
During the coronavirus disease 2019 (COVID-19) pandemic, the need for appropriate treatment guidelines for patients with brain tumors was indispensable due to the lack and limitations of medical resources. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, has undertaken efforts to develop a guideline that is tailored to the domestic situation and that can be used in similar crisis situations in the future.
Methods:
The KSNO Guideline Working Group was composed of 22 multidisciplinary experts on neuro-oncology in Korea. In order to reach consensus among the experts, the Delphi method was used to build up the final recommendations.
Results:
All participating experts completed the series of surveys, and the results of final survey were used to draft the current consensus recommendations. Priority levels of surgery and radiotherapy during crises were proposed using appropriate time window-based criteria for management outcome. The highest priority for surgery is assigned to patients who are life-threatening or have a risk of significant impact on a patient’s prognosis unless immediate intervention is given within 24–48 hours. As for the radiotherapy, patients who are at risk of compromising their overall survival or neurological status within 4–6 weeks are assigned to the highest priority. Curative-intent chemotherapy has the highest priority, followed by neoadjuvant/adjuvant and palliative chemotherapy during a crisis period. Telemedicine should be actively considered as a management tool for brain tumor patients during the mass infection crises such as the COVID-19 pandemic.
Conclusion
It is crucial that adequate medical care for patients with brain tumors is maintained and provided, even during times of crisis. This guideline will serve as a valuable resource, assisting in the delivery of treatment to brain tumor patients in the event of any future crisis.
10.A Case of Hystrix-Like Ichthyosis and Deafness Syndrome with a Rare Variant of Gap Junction Protein Beta 2 Gene Mutation
Youngbeom KIM ; Hak Jun KIM ; Yeona KIM ; Sang-Hyeon WON ; Kyungnam BAE ; Jungsoo LEE ; Kihyuk SHIN ; Hoonsoo KIM ; Byung Soo KIM ; Moon-Bum KIM ; Hyun-Chang KO
Korean Journal of Dermatology 2023;61(6):371-373
Hystrix-like ichthyosis and deafness (HID) syndrome is a rare ectodermal dysplasia characterized by erythrokeratoderma and hearing impairments. HID syndrome is a nonocular variant of keratitis ichthyosis deafness (KID) syndrome caused by an autosomal dominant mutation in the gap junction protein β 2 (GJB2) gene. The GJB2 gene encodes connexin 26, a transmembrane protein involved in cell–cell attachment in almost all tissues. We report a case of a 25-year-old man with generalized hyperkeratotic plaques, diffuse palmoplantar keratoderma, and nail deformities since birth. The patient also had a history of recurrent bacterial skin infections in the existing hyperkeratotic lesions. Histopathological examination revealed compact hyperkeratosis and irregular acanthosis in the epidermis, along with upper dermal lymphocytic infiltration. Audiometry revealed high-frequency sensorineural hearing loss. Genetic analysis revealed a missense mutation in the GJB2 gene. Based on clinicopathological findings and genetic testing, HID syndrome was diagnosed.

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