Background: and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods: We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse. Results: The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates. Conclusions Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.

Background: and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods: We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse. Results: The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates. Conclusions Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.

Background: and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods: We collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children’s Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse. Results: The median age at onset was 7 years (range 2–16 years). The median number of relapses was 2 (range 1–8), and patients were followed up for a median of 65 months (range 5–214 months). The first relapse was experienced before 3 months from onset by 15 patients (34.9%). The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%). The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%). Many of the patients (n=18, 41.9%) were not specifically categorized. A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis. Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted. Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates. Conclusions Our 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes. The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.

Angiofibroma and shagreen patches are common cutaneous manifestations of tuberous sclerosis complex (TSC) and have significant physical and psychological repercussions for patients. Several treatments have been proposed to improve lesions; however, clear treatment guidelines have not yet been presented. Thus, we introduce a case of angiofibroma and shagreen patch improved by application of pulsed dye laser, ablative fractional CO2 laser, and topical rapamycin, and present clinical implications for the treatment of angiofibroma and shagreen patch in TSC.

Paradoxical reactions in patients treated with tumor necrosis factor-alpha inhibitors (TNFis) have an estimated prevalence of 1.5% to 5%. Such reactions usually present as psoriasiform eruptions on the trunk and extremities along with palmar and flexural involvement. When affecting the scalp, new-onset psoriasis induced by TNFi can result in non-scarring or scarring alopecia. Although the paradoxical reaction was first reported in 2003, this TNFi-associated psoriatic alopecia (TiAPA) has been recently reported with increasing frequency. This condition is characteristically reversible and requires clinical and histopathological identification from other diseases for proper treatment. The cessation of TNFi therapy may not be mandatory, and decision to continue TNFi therapy depends on the severity of TiAPA and the riskbenefit ratio of treatment modification on the underlying disease. Herein, we report a case of TiAPA in a patient with inflammatory bowel disease whose alopecia improved following suspension of TNFi. We also describe the clinical and histopathological diagnostic criteria based on review of the literature.

Background: Umbilical skin lesions are rare but diverse and are rarely diagnosed by biopsy. Objective: This study aimed to analyze the clinical and histopathological features of umbilical skin lesions that require histopathological investigation and to aid in the diagnosis of diseases in patients with umbilical skin lesions. Methods: We performed a retrospective analysis of the patients who visited Korea University Medical center for umbilical skin lesions between January 2008 and December 2019. Age, sex, clinical features, etiologies, and histopathological features were obtained from the patients’ medical records. Results: In total, 41 cases of histologically confirmed umbilical lesions were observed. There were 29 female patients (70.73%). The lesions included hypertrophic scars (11/41), epidermal cysts (7/41), verruca (6/41), seborrheic keratosis (4/41), nevus (4/41), steatocystoma (2/41), and one case each of endometriosis, hidrocystoma, neurofibroma, soft fibroma, foreign body granuloma, tick bite, and heterotopic gastric mucosa. Conclusion The most common umbilical skin lesions were hypertrophic scars, probably because of the increasing number of laparoscopic surgeries. Compared to the known trends in other countries, fewer biopsies were performed on the umbilical lesions for the diagnosis of metastatic cancer in this study, which might have been due to the low incidence of advanced gastric cancer and the early detection of cancer through endoscopy and computed tomography scan in Korea. Clinically, changes in treatment modality and diagnostic development can alter the frequency of the previously well-recognized diseases; therefore, medical personnel should be aware of the changing incidence of related diseases.

Background: Umbilical skin lesions are rare but diverse and are rarely diagnosed by biopsy. Objective: This study aimed to analyze the clinical and histopathological features of umbilical skin lesions that require histopathological investigation and to aid in the diagnosis of diseases in patients with umbilical skin lesions. Methods: We performed a retrospective analysis of the patients who visited Korea University Medical center for umbilical skin lesions between January 2008 and December 2019. Age, sex, clinical features, etiologies, and histopathological features were obtained from the patients’ medical records. Results: In total, 41 cases of histologically confirmed umbilical lesions were observed. There were 29 female patients (70.73%). The lesions included hypertrophic scars (11/41), epidermal cysts (7/41), verruca (6/41), seborrheic keratosis (4/41), nevus (4/41), steatocystoma (2/41), and one case each of endometriosis, hidrocystoma, neurofibroma, soft fibroma, foreign body granuloma, tick bite, and heterotopic gastric mucosa. Conclusion The most common umbilical skin lesions were hypertrophic scars, probably because of the increasing number of laparoscopic surgeries. Compared to the known trends in other countries, fewer biopsies were performed on the umbilical lesions for the diagnosis of metastatic cancer in this study, which might have been due to the low incidence of advanced gastric cancer and the early detection of cancer through endoscopy and computed tomography scan in Korea. Clinically, changes in treatment modality and diagnostic development can alter the frequency of the previously well-recognized diseases; therefore, medical personnel should be aware of the changing incidence of related diseases.

Background: Eccrine porocarcinoma (EPC) is a rare malignant cutaneous adnexal tumor. Other than several scattered case reports, no comprehensive review on EPC has been conducted in Korea. Objective: To clinicopathologically review all EPC cases from our institutions as well as those reported in Korea. Methods: Medical records and histopathological slides of EPC cases in the skin biopsy registries of our institutions were retrospectively reviewed. Additionally, EPC cases reported in Korea before June 2019 were retrieved by searching the PubMed, KoMCI, KoreaMed, and KMbase databases. Results: Nine EPC cases from our institutions were included in the study. In addition, 27 reports of 28 patients with EPC were reported in Korea. A total of 37 patients with EPC were identified, consisting of 19 males (male:female ratio, 1.06:1; mean age at diagnosis, 65.6 years). The most common site of primary tumor was the head and neck (29.7%). Wide excision was the most common (78.4%) treatment method. Initial metastasis work-up imaging studies were performed in 18 patients (48.6%), and metastasis was confirmed in eight patients (21.6%). Conclusion EPC is a rare cutaneous carcinoma in Korea. EPC usually affects elderly patients, with no sexual predilection. Due to possible metastasis, careful diagnosis and appropriate metastasis workups are warranted in EPC.