Pachyonychia congenita: A father and son with a novel variant in the KRT16 gene.

This study underscores the significance of identifying the clinical manifestations of pachyonychia congenita (PC) and emphasizes the patterns of genetic inheritance. A 12-month-old boy presented with a "white hairy tongue" and, following a comprehensive evaluation, was diagnosed with PC. His father exhibited similar symptoms. Genetic testing revealed a KRT16 pathogenic variant (c.616 T > G) in both the patient and his father, marking it as a novel variant in the PC literature. This case contributes to a broader understanding of PC's genetic diversity and its clinical presentations.

Simultaneous versus sequential fractional CO2 laser and subcision combination for management of post-acne atrophic scars: A split-face comparative study.

BACKGROUND: A combination of fractional CO2 laser and subcision is usually employed for the treatment of post-acne atrophic scars. However, the efficacy and safety of both simultaneous and sequential combination therapies should be explored. AIMS: To compare the efficacy and safety of simultaneous and sequential fractional CO2 laser and subcision combination therapies for post-acne atrophic scars. PATIENTS AND METHODS: This single-blind, split-face clinical trial included 34 patients with post-acne atrophic scars at our institution. Each patient underwent three sessions of subcision combined with fractional CO2 laser, with a 1-month interval between each session. The left side of the face was treated with simultaneous combination therapy, whereas the right side was treated with sequential combination therapy. Treatment efficacy was assessed at 4, 8, and 12 weeks; and 3 and 6 months after the last session. RESULTS: Simultaneous and sequential treatments demonstrated comparable efficacy. Regarding the adverse events, the side of the face undergoing simultaneous treatment experienced longer swelling duration, higher pain levels during laser treatment, and shorter downtime. CONCLUSIONS: Despite the longer swelling time and higher pain levels during laser treatment in the simultaneous treatment side, the effectiveness and satisfaction level of the CO2 fractional laser and subcision for treatment of the acne scars were comparable between the two combinations, with a shorter downtime for the simultaneous than for the sequential combination therapy.

Comorbidity Burden and Treatment Patterns of Psoriasis in Vietnam: Real-World Data from the EXPAND Study.

INTRODUCTION: Psoriasis is a multi-faceted, immune-mediated inflammatory disease associated with a wide range of comorbidities. Real-world data on treatment patterns, comorbidities, and economic burden in patients with psoriasis are needed for comprehensive patient care in Vietnam. METHODS: A retrospective chart review study was conducted using secondary data extracted from patients' medical records of two hospitals in Vietnam, with the aim of identifying adult patients with a confirmed diagnosis of psoriasis. The index date was defined as the date of first diagnosis between 1 January 2020 and 31 October 2021. Sociodemographic factors, disease characteristics, comorbidities, medication usage, drug survival, and medication costs were analyzed. RESULTS: A total of 661 patients were identified (mean ± standard deviation [SD] age 43.5 ± 14.8 years). The most prevalent comorbidity was dyslipidemia (49.6% of patients), followed by hypertension (23.4%), and psoriatic arthritis (10.4%). In total, 44% of patients received biologic therapies. Overall, 66.7% and 54.3% of patients receiving biologic and non-biologic therapies, respectively, had ≥ 1 comorbidity. Only 23.2% of patients with psoriasis-related comorbidities stopped therapy with biologics. Biologics had a longer retention time (17.0 months) than non-biologics (6.0 months) in patients with comorbidities. Patients with comorbidities had significantly higher total annual healthcare costs than those without comorbidities (in US dollars: USD901 vs. USD304; p < 0.001), mainly due to the relatively higher costs associated with the use of biologics. CONCLUSION: Patients with psoriasis in Vietnam experience a high disease and economic burden due to comorbidities. Evidence from this real-world study supports the need for routine monitoring of and an appropriate treatment course for psoriasis-related comorbidities.

Crouzon syndrome with acanthosis nigricans: a case report and literature review.

Crouzon syndrome with acanthosis nigricans is an autosomal dominant disease, with typical features of classic Crouzon craniosynostosis, verrucous hyperplasia, and hyperpigmentation of the skin. While several mutations in FGFR2 cause classic Crouzon syndrome, Crouzon syndrome with acanthosis nigricans results from a point mutation in the fibroblast growth factor receptor 3 gene (FGFR3). We report the case of an 8-year-old Vietnamese girl diagnosed with Crouzon syndrome with acanthosis nigricans, showing typical clinical features, including a crouzonoid face and dark plaques on the skin. Genetic testing showed a missense variation in FGFR3, associated with Crouzon syndrome with acanthosis nigricans. Following diagnosis, we treated acanthosis nigricans with 10% urea cream. This case study and literature review discuss the cutaneous manifestations and dermatological treatments while demonstrating the importance of clinical examination and evaluation of the patient's medical history during diagnosis. Our findings contribute to the global pool of data, providing practical insights into the manifestations of Crouzon syndrome.

Real-world management of abnormal scarring using topical silicone gel: expert consensus and case series from the Asian SCARS Expert Group.

Preventing abnormal scar formation and correcting non-aesthetic mature scars are important to prevent physical and psychosocial consequences of abnormal scarring. Evidence-based guidelines for scar management in Asian patients recommend first-line silicone-based products. Dermatix®* Ultra and Dermatix Ultra Kids are topical silicone gels containing a vitamin C ester that helps lighten scar tissue. Herein, we report a case series including patients with hypertrophic and keloid scars treated with Dermatix, showing that Dermatix is effective for scar treatment and prevention, as well as expert consensus supporting the safe and effective use of Dermatix.

Sexual Dysfunction and Associated Factors in Women with Psoriasis.

Context: Psoriasis, a chronic inflammatory skin disease, may negatively affect sexual function; however, data on the sexual health of female patients with psoriasis in Vietnam are lacking. Aims: To assess the risk of sexual dysfunction (SD) and its associated factors in female patients with psoriasis who visited the Ho Chi Minh City Hospital of Dermato-Venereology from April 2020 to October 2020. Settings and Design: Cross-sectional study. Methods and Material: A total of 302 female patients with psoriasis aged 18 to 49 years were recruited. The Female Sexual Function Index (FSFI) was used to assess the risk of SD (cut-off value at 26). Results: The risk of SD accounted for 79.1% of the study population and was not associated with age, comorbidities, weight, or trigger factors. Urban dwellers had greater odds of SD risk than non-urban dwellers (OR = 2.63). Similar trends were observed in terms of higher education than grade 12, less than once a week of physical activities, and psychological stress as a trigger factor (OR = 1.89, 2.65, and 3.41, respectively). Female psoriasis patients with SD risk had a lower age of onset and higher weight, BMI, waist circumference, and PASI than patients who did not (P < 0.05). Psychological stress and high PASI were independent risk factors of SD risk in female patients with psoriasis (P < 0.05). Conclusions: Factors with negative impacts on psoriasis may also increase the risk of SD in female patients; among these factors, psychological stress and high PASI were the strongest predictors of SD in female patients with psoriasis.

Long-Term Effectiveness and Drug Survival of Secukinumab in Vietnamese Patients with Psoriasis: Results from a Retrospective ENHANCE Study.

INTRODUCTION: Psoriasis (PsO), an immune-mediated inflammatory skin disorder, has substantial negative impact on patients' quality of life. Secukinumab, an approved treatment for moderate-to-severe plaque PsO, has an established long-term efficacy and safety profile. This study aims to provide real-world evidence of long-term effectiveness and retention rate of secukinumab in Vietnamese patients with PsO. METHODS: This retrospective, observational study collected medical records of adult patients with moderate-to-severe PsO receiving secukinumab treatment from Ho Chi Minh City Hospital of Dermato-Venereology. The primary objective was to evaluate secukinumab effectiveness in PsO as measured by 75% improvement in psoriasis area and severity index (PASI 75) at month 12. Secondary objectives were PASI 90/100, absolute PASI ≤ 3 and ≤ 5, Dermatology Life Quality Index (DLQI), and retention rate over 48 months. RESULTS: In total, 232 patients with moderate-to-severe PsO met inclusion criteria; 68.1% were male, with median age and age of onset of 39 and 27.5 years, respectively. Median time from onset of PsO to secukinumab treatment was 120 months, 95.3% were prior biologics/disease-modifying antirheumatic drugs naive and 41.4% received concomitant therapies for PsO; 82.3% had national insurance coverage. At month 12, 93.9% of patients achieved PASI 75 (primary endpoint); 80.2/56.9% achieved PASI 90/100; 91.4 and 84.8% patients achieved absolute PASI ≤ 5 and ≤ 3, respectively. The response was sustained over 48 months, with 91.9%/78.0%/52.0% of patients achieving PASI 75/90/100, 89.5% and 82.1% patients achieving absolute PASI ≤ 5 and ≤ 3, respectively. At month 12, 61.4% of patients achieved DLQI 0/1 which was sustained up to month 48 (69.2%). Secukinumab adherence rate of 84.9% at month 12 dropped to 34.2% at month 48. Patients receiving concomitant therapy and national insurance showed higher adherence rate. CONCLUSION: Secukinumab demonstrated long-term effectiveness in real-world Vietnamese patients with moderate-to-severe PsO, with treatment adherence being higher in patients having concomitant therapies and national insurance.

New onset of psoriasis following COVID-19 vaccination.

The cutaneous side effects of COVID-19 vaccines are being studied and their immunogenicity is most likely linked to the pathophysiology of psoriasis. Although uncommon, several cases of exacerbation and new onset of psoriasis have been reported globally after vaccination. To contribute to the literature on this intriguing topic, we present three cases of de novo psoriasis in adult patients following COVID-19 vaccination. Our observations and a literature review show that this occurrence is independent of the type and brand of vaccines.

Diverse clinical manifestations of Cantú syndrome: The first case series in Vietnam.

Cantú syndrome (CS) is an extremely rare autosomal dominant hereditary disease characterized by congenital hypertrichosis, distinct coarse facial features, cardiac defects, and other abnormalities in the skeletal and neurological systems. At present, cases with pathognomonic clinical manifestations are increasingly confirmed by genetic analysis. Two causative genes for CS are the well-known ABCC9 and the more rarely reported KCNJ8. Here, we report three Vietnamese children with CS, confirmed through genetic testing, presenting de novo ABCC9 mutations. The patients shared some common clinical manifestations, including congenital hypertrichosis, distinctive facial features, and a history of polyhydramnios during pregnancy. Concerning the various cardiac and neurological problems in the lifetime of patients with CS, an accurate diagnosis and appropriate management, especially genetic counseling, should be clinically applied in CS. Thus, our findings might modestly contribute to the global CS data, providing practical insights into CS manifestations.

The First Vietnamese Patient of LEOPARD Syndrome due to a PTPN11 Mutation: A Case Report and Review of the Literature.

LEOPARD syndrome is a rare congenital anomaly that involves several organs. Patients with this syndrome develop multiple lentigines resembling a leopard's hide. LEOPARD is an acronym of the major features constituting the syndrome including lentigines, electrocardiographic conduction defects, ocular hypertelorism, pulmonary valve stenosis, anomalies of genitalia, retardation of growth, and deafness. The syndrome is rare, and only 200 cases have been reported yet worldwide. We present the case of an 8-year-old female patient who visited the Ho Chi Minh City Hospital of Dermato-Venereology because of multiple brownish-black "dots" on her face and body. On examination, she also showed abnormalities in the maxillofacial bones, vertebrae, shoulders, sternum, and teeth, as well as deaf-mutism and growth retardation, which are typical of LEOPARD syndrome. Genetic analysis revealed a PTPN11 gene mutation in this case. To the best of our knowledge, this is the first case of LEOPARD syndrome reported in Vietnam.