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.

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.

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.

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.

Purpose: This study aimed to describe adult living donor liver transplantation (LDLT) for acute liver failure and evaluate its clinical significance by comparing its surgical and survival outcomes with those of deceased donor liver transplantation (DDLT). Methods: We retrospectively reviewed the medical records of 267 consecutive patients (161 LDLT recipients and 106 DDLT recipients) aged 18 years or older who underwent liver transplantation between January 2006 and December 2020. Results: The mean periods from hepatic encephalopathy to liver transplantation were 5.85 days and 8.35 days for LDLT and DDLT, respectively (P = 0.091). Among these patients, 121 (45.3%) had grade III or IV hepatic encephalopathy (living, 34.8% vs. deceased, 61.3%; P < 0.001), and 38 (14.2%) had brain edema (living, 16.1% vs. deceased, 11.3%; P = 0.269) before liver transplantation. There were no significant differences in in-hospital mortality (living, 11.8% vs. deceased, 15.1%; P = 0.435), 10-year overall survival (living, 90.8% vs. deceased, 84.0%; P = 0.096), and graft survival (living, 83.5% vs. deceased, 71.3%;P = 0.051). However, postoperatively, the mean intensive care unit stay was shorter in the LDLT group (5.0 days vs. 9.5 days, P < 0.001). In-hospital mortality was associated with vasopressor use (odds ratio [OR], 3.40; 95% confidence interval [CI], 1.45–7.96; P = 0.005) and brain edema (OR, 2.75; 95% CI, 1.16–6.52; P = 0.022) of recipient at the time of transplantation. However, LDLT (OR, 1.26; 95% CI, 0.59–2.66; P = 0.553) was not independently associated with in-hospital mortality. Conclusion LDLT is feasible for acute liver failure when organs from deceased donors are not available.

Purpose: We used arterial spin labeling (ASL) perfusion magnetic resonance (MR) imaging to evaluate cerebral perfusion abnormalities in patients with seizures within 24 hours of symptom onset. Materials and Methods: A retrospective search of our institutional database identified 27 patients who had undergone ASL perfusion studies for seizures or seizure-like symptoms.The inclusion criteria were as follows: 1) history of seizure, 2) MR examination performed within 24 hour of seizure onset, and 3) localized perfusion abnormality on ASL. We evaluated the presence, location, and extent of perfusion abnormalities on ASL and signal abnormalities on fluid-attenuated inversion recovery (FLAIR), diffusion-weighted image (DWI), and susceptibility-weighted image (SWI), respectively. All pathological MR findings, accompanying focal neurological symptoms, and electroencephalogram (EEG) findings were compared. Results: The mean time from symptom onset to MR examination was 5 h 54 min. All patients (n = 27) showed localized increased perfusion on ASL perfusion imaging. On FLAIR imaging, 20 patients (74.1%) showed hyperintensity in the area of perfusion abnormality.In 19 patients (70.4%), DWI showed hyperintensity of the lesion with decreased apparent diffusion coefficient value (ADC). Seven patients (25.9%) showed a focal parenchymal area of pseudo-narrowed cortical veins on SWI, associated with focal hyperperfusion. In 20 patients (74.1%), the extent of perfusion abnormalities on ASL was greater than that of signal abnormalities on FLAIR or DWI. In 14/16 patients (87.5%) with abnormal EEG findings, the area with EEG findings and the location of the hyperperfusion abnormality on ASL corresponded. Conclusion In patients with seizures within 24 hours of symptom onset, ASL perfusion imaging revealed localized hyperperfusion, which was more frequent than signal intensity abnormalities on FLAIR or DWI. The locations of hyperperfusion areas correlated with EEG abnormalities. Thus, the ASL sequence may be a useful clinical assessment protocol for evaluating patients with seizures.

Background: In this study, we aimed to compare the long-term therapeutic outcomes of drug-eluting bead transarterial chemoembolization (DEB-TACE) with those of radiofrequency ablation (RFA) for the initial treatment of a single small (≤ 3 cm) hepatocellular carcinoma (HCC). Methods: From January 2010 to December 2021, 259 consecutive patients who underwent DEB-TACE (67 patients) or RFA (192 patients) as a first-line treatment for a single small HCC were enrolled in this retrospective study. The therapeutic outcomes, including cumulative intrahepatic local tumor progression (LTP), progression-free survival (PFS), and longterm overall survival (OS) rates, were compared between the two groups before and after propensity score (PS) matching. Multivariate Cox proportional hazard models were used to evaluate the prognostic factors and differences in OS and PFS between the two groups for all 92 patients after PS matching. Results: After PS matching, the 1-, 2-, 3-, and 5-year LTP rates were lower in the RFA group than those in the DEB-TACE group (P < 0.001), and the 1-, 2-, 3-, and 5-year PFS rates in the RFA group were higher than those in the DEB-TACE group (P = 0.007). However, the 1-, 2-, 3-, and 5-year OS rates were not significantly different between the RFA and DEB-TACE groups (P = 0.584).Moreover, the OS was not significantly different between the RFA and DEB-TACE groups in the univariate and multivariate analyses, with a hazard ratio (HR) of 0.81. The PFS was significantly higher in the RFA group than that in the DEB-TACE group in the univariate analyses, with a HR of 0.44 (P = 0.009). Multivariate Cox regression analysis showed that albumin (P = 0.019) was an independent prognostic factor for OS. Additionally, the major complication rates were not significantly different between the DEB-TACE and RFA groups (P = 1.000). Conclusion The LTP and PFS rates of RFA were superior to those of DEB-TACE in the initial treatment of single small HCC after PS matching. However, the OS rates were not significantly different between RFA and DEB-TACE. Therefore, DEB-TACE may be considered an efficient substitute for RFA in some patients with a single small HCC who are ineligible for RFA.

Purpose: Studies have yielded contradictory results on whether donor sex and donor-recipient sex disparity affect hepatocellular carcinoma (HCC) recurrence after living donor liver transplantation (LDLT). The present study assessed whether donor sex or donor-recipient sex disparity affects HCC recurrence after LDLT at a high-volume center. Methods: This study included 772 HCC patients who underwent LDLT between January 2006 and December 2015 at Asan Medical Center. Patients were divided into 4 groups based on the sex of the donor and recipient: male-to-male (n = 490, 63.5%), male-to-female (n = 75, 9.7%), female-to-male (n = 170, 22.0%), and female-to-female (n = 37, 4.8%). Results: Disease-free survival (DFS; P = 0.372) and overall survival (OS; P = 0.591) did not differ significantly among the 4 groups. DFS also did not differ significantly between LDLT recipients with male and female donors (P = 0.792) or between male and female recipients (P = 0.084). After patient matching with an α-FP/des-γ-carboxy prothrombin/tumor volume score cutoff of 5logs, donor-recipient sex disparity did not significantly affect DFS (P = 0.598) or OS (P = 0.777). There were also no differences in DFS in matched LDLT recipients with male and female donors (P = 0.312) or between male and female recipients (P = 0.374). Conclusion Neither donor sex nor donor-recipient sex disparity significantly affected posttransplant HCC recurrence.