Cost and effectiveness comparison of sirolimus versus standard treatment in Kasabach-Merritt phenomenon: a real-world evidence study in Thailand.

The conventional treatment of Kasabach-Merritt Phenomenon (KMP) consists of corticosteroids with vincristine/vinblastine or others. The aim of the study is to compare the first-year direct costs and effectiveness between sirolimus and conventional treatment. A retrospective case-control study of KMP patients was conducted at a mean age of 9 months (1 day to 12 years) between 2000 and 2022 from four tertiary centers in Thailand. The direct costs, hematologic and clinical complete response (HCR, CCR), hospitalization, length of stay, and complications were compared. Of 29 patients, 13 underwent sirolimus (four upfront and nine were refractory to the conventional). The first-year total cost had no statistically significant difference between sirolimus VS conventional treatment (8,852.63 VS 9,083.56 USD: p value: 0.94). The therapeutics achievement was the same in both HCR (244.75 VS 168.94 days; p value: 0.60) and CCR (419.77 VS 399.87 days; p value: 0.90). The subgroup analysis of the first-line sirolimus (n = 4) compared with the conventional (n = 25) showed a more reduced total cost (4,907.84 VS 9,664.05 USD; p value: 0.26) rendered net total cost of -4,756.21 USD per patient (cost saving). A more significant contrast of therapeutic achievement by reduction of both HCR (11.67 VS 224.20 days; p value: 0.36) and CCR (38.50 VS 470.88 days; p value: 0.04) was shown. The sirolimus had no difference in hospitalization, length of stay, and complications. Even though, it was unable to identify significant differences in cost-effectiveness. Sirolimus is suitable for all patients who have diagnosis of KMP either for rescue therapy or first-line treatment.

Severe cutaneous adverse drug reactions: incidence, clinical characteristics, treatment, and outcome in pediatric patients.

BACKGROUND: Severe cutaneous adverse drug reactions (SCARs) can cause significant morbidity and mortality. Clinical data regarding such conditions is still limited in the pediatric population. OBJECTIVE: To investigate the incidence, clinical characteristics, treatment, and outcome of SCARs in Thai pediatric patients. METHODS: This retrospective study enrolled 52 patients aged less than 18 years who were diagnosed with acute generalized exanthematous pustulosis (AGEP), drug reaction with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), or SJS/TEN overlap during January 2005 to August 2021 at Siriraj Hospital. RESULTS: SCARs were slightly more prevalent in females than in males (51.9% vs. 48.1%). Median age at diagnosis was 97 months, and median length of hospital stay was 11 days. DRESS, SJS, TEN, AGEP, and SJS/TEN overlap was found in 44.2%, 36.5%, 9.6%, 5.8%, and 3.8%, respectively. The most common etiologies were antimicrobial agents (40.3%) and anticonvulsants (35.5%). Target lesions, vesicobullous lesions, purpura, positive Nikolsky's sign, and skin tenderness were significant in blistering SCARs. Hematologic (84.6%) and hepatic (65.5%) manifestations were common. Treatment varied according to the clinical features of each condition. Systemic corticosteroids showed some benefit in SJS/TEN. One patient diagnosed with TEN died for an overall SCARs mortality rate of 1.9%. CONCLUSION: The unique characteristics of SCARs described herein can lead to timely and accurate diagnosis and proper management.

Factor associated with food allergy among preschool children with atopic dermatitis, and resolution of atopic dermatitis.

BACKGROUND: Food allergy (FA) has been reported in one-third of children with moderate-to-severe atopic dermatitis (AD). OBJECTIVE: To identify factor associated with food allergy among preschool children with AD, and to compare AD resolution between preschool children with and without FA. METHODS: A cross-sectional study using database registry and questionnaire interview was conducted at Siriraj Hospital(Bangkok, Thailand) during 2022, and physician-diagnosed AD children aged ≤ 6 years were enrolled. RESULTS: A total of 110 children (60.9% male, median age: 2.3 years) were included. Of those, 53 and 57 children had AD with and without FA, respectively. Very early-onset AD (≤ 3 months) and moderate-to-severe AD at onset were reported in 43.9% and 26.3% of AD without FA, and in 35.8% and 45.3% of AD with FA, respectively. The most commonly reported FAs were hen's egg, cow's milk, and wheat. Moderate-to-severe AD at onset was found significant associated with FA (aOR: 2.50; p = 0.037). Thirty-one (28.2%) patients experienced completed resolution of AD by 5 years of age. Of those, 19 had AD without FA, and 12 had AD with FA (p = 0.213). The median age at AD resolution was 18 months and 22.5 months in the without and with FA groups, respectively. AD with FA showed a strong trend toward a significantly longer duration to achieving AD resolution after adjusting for onset and severity of AD (aHR: 0.46, p = 0.050). CONCLUSION: Preschool AD children with FA were found to have significantly greater AD severity at AD onset and a longer duration to AD resolution compared to AD children without FA.

Prenatal predisposing factors associated with neonatal lupus erythematosus.

OBJECTIVES: To identify the prenatal predisposing factors related to neonatal lupus erythematosus (NLE). MATERIALS AND METHODS: A retrospective case-control study was made of 131 pregnant women with positive anti-Ro or anti-La autoantibodies and known neonatal outcomes between January 2002 and December 2019 at Siriraj Hospital, Bangkok, Thailand. There were 101 unaffected neonates and 30 NLE cases confirmed postnatally. Demographic and clinical data of the mothers and neonates with and without NLE were statistically compared. RESULTS: NLE was diagnosed in 30 out of 131 cases. A multivariate analysis identified the following significant factors for NLE: maternal anti-La antibodies (odds ratio (OR), 3.591; p = 0.030); and maternal treatment with either hydroxychloroquine (OR, 0.082; p = 0.001) or prednisolone (OR, 0.136; p = 0.017). Of the significant variables examined in the multivariate analysis models, high levels of maternal anti-La antibodies were found to be the strongest predictor of noncardiac NLE (OR, 4.558; p = 0.032), while a female baby was significantly higher in pregnancies complicated by cardiac NLE (OR, 5.374; p = 0.046). Hydroxychloroquine still provided a protective effect for both cardiac and noncardiac NLE (p = 0.039 and 0.032, respectively). CONCLUSIONS: The maternal anti-La antibodies were a beneficial predictor for NLE, especially as their high titers were influentially associated with noncardiac features. A female fetus seemed to present an increased risk for developing a congenital heart block. Nevertheless, the treatment with hydroxychloroquine during the pregnancies demonstrated a potentially protective factor against both cardiac and noncardiac manifestations.

Knowledge about, attitude toward, and practices in skin care among Thai adolescents.

BACKGROUND: Skin care for maintaining skin integrity includes cleansing, skin product use, and photoprotection. Inappropriate skin care can lead to skin problems. AIMS: To evaluate the knowledge, attitude, and practices in skin care among Thai adolescents. PATIENTS/METHODS: Questionnaire-based, descriptive, cross-sectional study. RESULTS: A total of 588 Thai adolescent students (mean age: 15.6 ± 1.8 years, 50.5% female) were included. Of those who responded, 99.5% knew the benefits of cleansing, and 95.9% knew the benefits of skin care products. Skin products, moisturizer, and sunscreen were used by 87.8%, 80.8%, and 71.5% of students, respectively. Female teenagers used moisturizers, cosmetics, and sunscreen significantly more than males (p = 0.001, p = 0.001, and p < 0.001, respectively). High school teenagers applied cosmetics more than junior high school teenagers (p = 0.004). Ninety-three percent of adolescents knew the effects of sunlight, but only 27.4% regularly applied sunscreen. The sources of knowledge were from person, online social media, print media, and television/radio in 88.5%, 77.5%, 30.7%, and 26.1%, respectively. Data from physicians and parents were trusted by 65.3% and 64.2%, respectively. Most (74.1%) adolescents searched for data from more than 1 source. Adolescent females and high school adolescents demonstrated significantly more accurate knowledge and practice in cleansing and photoprotection (p < 0.001) compared with adolescent males and junior high school adolescents. Knowledge and practices did not significantly correlate with underlying skin diseases or monthly allowance. CONCLUSION: Gender and education level were found to significantly influence knowledge and practice in skin care among adolescents in Thailand.

Juvenile dermatomyositis in Thai children: Retrospective review of 30 cases from a tertiary care center.

BACKGROUND: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. AIM: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. METHOD: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. RESULTS: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron's rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. LIMITATIONS: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. CONCLUSION: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur.

Capacitance and transepidermal water loss after soaking in water for different durations: A pilot study.

BACKGROUND: There is a scarcity of data on the effects of duration of bathing and cutaneous properties. AIMS: This study aimed to investigate the changes of capacitance and transepidermal water loss (TEWL) after soaking in water for the different durations. METHOD: This experimental biophysical study included healthy volunteers whose forearms were randomized to receive 3, 5, 10, 15, or 20 min of soaking of the volar aspect of the forearm. Skin hydration and integrity were assessed capacitance and TEWL measurement before and after soaking. RESULTS: Sixty-five subjects (130 forearms) were enrolled with an average age of 33 ± 10.8 years. The change in capacitance after soaking for durations of 3, 5, 10, 15, and 20 min was 41.54 ± 14.57, 47.13 ± 11.80, 40.25 ± 14.95, 40.48 ± 14.19, and 39.97 ± 9.47 AU, respectively. The highest capacitance was observed after soaking for 5 min; however, there was no significant correlation between bathing duration and capacitance (p = 0.256). The capacitance measured immediately after soaking was at the uppermost level, but it rapidly decreased within 5 min. The change in TEWL after soaking for durations of 3, 5, 10, 15, and 20 min was 30.27 ± 9.74, 30.57 ± 7.45, 33.78 ± 9.25, 33.44 ± 7.24, and 35.13 ± 9.37 g/m2 /h, respectively. There was also no significant correlation between duration of soaking and TEWL (p = 0.191); however, TEWL tended to increase with longer soaking duration. LIMITATIONS: This study had a small sample size and measured only capacitance and TEWL. Future studies with more subjects, and that measure other physiologic parameters may further improve our understanding of the effect of bathing on skin. CONCLUSIONS: There was no significant correlation between bathing duration and cutaneous properties including capacitance and TEWL. However, a 5-min soaking provided the highest skin hydration for healthy skin.

Truncus arteriosus as presentation of PHACE syndrome.

PHACE syndrome is a rare neurocutaneous syndrome that describes the association of large segmental infantile haemangioma involving the head and neck, along with other systemic anomalies. Complex congenital heart disease has rarely been reported in this syndrome. We present a report of a patient with PHACE syndrome and truncus arteriosus.

Clinical practice guidelines for the diagnosis and management of atopic dermatitis.

Atopic dermatitis (AD), a chronic, relapsing dermatitis, is characterized by dry and pruritus skin in patients with a personal or family history of atopy. It affects up to 20% of children and 1-3% of adults in most countries worldwide, and leads to significant treatment costs and morbidity. These guidelines are developed in accordance with evidence-based publications and expert opinions. Following simple algorithms, the guidelines aim to assist adult and pediatric physicians in the better care of patients with AD. As with other diseases, there have been several diagnosis criteria proposed over time. Nonetheless, the classical Hanifin and Rajka criterion with no pathognomonic laboratory biomarkers is still the most widely used worldwide for the diagnosis of AD. The management of AD must be considered case by case to provide suitable care for each patient. Basic therapy is focused on avoiding specific/unspecific provoking factors and hydrating skin. Topical anti-inflammatory treatments such as glucocorticoids and calcineurin inhibitors are suggested for disease flare, and proactive therapy is best for long-term control. Other therapies, including antimicrobial agents, systemic antihistamines, systemic anti-inflammatory agents, immunotherapy, phototherapy, and psychotherapy, are reviewed in these guidelines. Crisaborole, a new topical phosphodiesterase 4 inhibitor, can be used twice daily in AD patients over three months old. Dupilumab, a biological drug for patients with moderate-to-severe AD, may be considered in patients with no improvement from other systemic treatments.

Skin hydration and transepidermal water loss after bathing compared between immersion and showering.

BACKGROUND: Various methods of bathing may affect skin properties differently. AIMS: To compare the effects of immersion and showering on skin hydration and transepidermal water loss (TEWL). METHOD: This experimental study included healthy volunteers whose forearms were immersed and showered for 3 minutes. Skin hydration and TEWL were assessed serially before and after immersion and showering of volunteer forearms. RESULTS: Seventy-eight healthy volunteers (49 females, 29 males) were enrolled with an age range of 12-55 years (mean 31.41 ± 10.33). Both methods significantly increased skin hydration and TEWL (P < .001). The capacitance value significantly increased immediately after bathing, and then rapidly decreased within 3 minutes. It returned to baseline by 10 minutes after bathing. There was no statistically significant difference of capacitance between the two methods at any measurement (P > .05). TEWL at every measurement after bathing was significantly increased compared to baseline for both bathing methods (P < .001). The highest TEWL was observed immediately after bathing, but then significantly decreased compared to the previous measurement (P < .001). CONCLUSION: Immersion and showering similarly demonstrated significant increase in skin hydration and TEWL. The increment of capacitance after bathing returned to baseline level within 10 minutes.

Photoprotective habits in children with systemic lupus erythematosus.

BACKGROUND: Systemic lupus erythematosus (SLE) is a complex autoimmune disease that can involve multiple organ systems. Exposure to ultraviolet radiation (UVR) can exacerbate pre-existing SLE, and can even induce systemic manifestations. This study aimed to investigate the photoprotective habits of children with SLE and the factors that significantly influence those photoprotective habits. METHODS: This questionnaire-based cross-sectional study included paediatric SLE patients being treated at the Department of Paediatrics at Siriraj Hospital, Mahidol University, between September 2018 and September 2019. Data were obtained from medical records and a face-to-face interview. RESULTS: Ninety-six patients were enrolled, with a female-to-male ratio of 8:1. The mean age of patients at enrollment was 13.7 ± 2.4 years. Of the 96 patients, 70 (72.9%) reported being directly exposed to sunlight for less than two hours per day, but 39% of patients spent time in the sun during the peak hours of UVR. Up to 95% of patients used sunscreen. However, only 64% of patients applied it every day, and only 35% of patients used an adequate amount of sunscreen. Girls were significantly more likely to apply sunscreen every day than boys were (p = 0.041). SLE patients with a shorter disease duration had significantly greater exposure to sunlight than patients with a disease duration of more than four years (p = 0.040). CONCLUSION: Sunscreen was the most common photoprotective method. However, most patients used sunscreen inappropriately. A shorter disease duration was significantly associated with more sunlight exposure. Regular evaluation and emphasis of the importance of photoprotection should be encouraged among paediatric SLE.

Acne beliefs, treatment-seeking behaviors, information media usage, and impact on daily living activities of Thai acne patients.

BACKGROUND: Acne is a very common skin disease. Information on it is readily available and accessed by most patients. Nevertheless, they tend to have misunderstandings about the disease. AIMS: This study investigated Thai acne patients' perceptions of, and beliefs, about acne; their treatment-seeking behaviors; and the data sources available to them. PATIENTS/METHODS: A cross-sectional, questionnaire-based study was conducted among teenage and adult acne patients at the skin clinics of the Department of Dermatology and the Department of Pediatrics, Siriraj Hospital, January-December 2017. RESULTS: A total of 330 patients with a mean age of 23.89 ± 7.19 years (range: 9-51 years) were enrolled. Hormonal factors were the most common determinant thought to worsen acne (80.6%), followed by dirt (72.4%), inadequate sleep (65.5%), cosmetics (58.2%), and stress (55.8%), whereas frequent facial washing and exercise were the least common (4.8% each). The most common information source utilized by patients was friends (40.9%), followed by digital media (36.8%). Both males and females felt their acne greatly affected their quality of life. Before visiting the hospital, most patients used vitamin supplements and over-the-counter drugs as treatment. CONCLUSIONS: Some patients had good conceptions of certain aspects of acne, such as the influence of hormones or food, whereas others had misunderstandings about the effects of poor hygiene on acne. Friends and websites were the most common information resources exploited by patients. Acne substantially impaired the quality of patients' lives.

The Validity and Reliability of the Thai Version of Children's Dermatology Life Quality Index (CDLQI).

OBJECTIVE: To evaluate the validity and reliability of the Thai version of Children Dermatology Life Quality Index (CDLQI). MATERIAL AND METHOD: CDLQI was translated to Thai and approved by Lewis-Jones MS and Finlay AY The patients, 4- to 15-year-old, with skin diseases and with problems unrelated to the skin were included to complete this questionnaire. Some patients with skin diseases were randomly selected to complete the CDLQI again seven days later to test the reliability. The time to complete the questionnaire was recorded. RESULTS: Two hundred six children, which consisted of 113 patients with skin diseases (26 of this group answered the questionnaire twice) and 93 patients without skin diseases, were enrolled in the study. The mean age and sex distribution of the two groups were not statistically different (p 0.84, p 0.60, respectively). The mean CDLQI score of the patients with skin diseases was 7.5 ± 6.1. The validity of the CDLQI Thai version was p < 0.001 by comparing the scores from a variety of skin diseases with controls. Good reliability was demonstrated by assessing repeatability, which showed strong correlation coefficient of test-retest data with Spearman rank correlation coefficient r(s) 0.94 (p < 0.001). The Cronbach's coefficient alphas showed high internal consistency of the individual item (0.87). The average time to complete all questions was 4.5 ± 2.5 minutes. The younger age group spent longer time than the older age group (p < 0.001). CONCLUSION: The Thai version of CDLQI has good validity and reliability. It should be used to measure quality of life in the management of skin diseases in Thai pediatric patients.

Histiocytoid neutrophilic dermatitis, an unusual histopathology in neonatal lupus erythematosus.

The authors reported histiocytoid neutrophilic dermatitis in neonatal lupus erythematosus (NLE). One-month-old male infant presented with annular erythematous plaques at the face and trunk. Serologic studies revealed positive anti-ribonuclear protein antibodies (RNP) and antinuclear antibodies (ANA). Histopathology showed predominant myeloid lineage mononuclear cells admixed with segmented neutrophils. This finding is uncommon in cutaneous NLE. Cutaneous NLE and LE should be included in the differential diagnosis of histiocytoid neutrophilic dermatitis. Additional immunohistochemistry studies with clinical and serologic correlations are important to differentiate histiocytoid neutrophilic dermatitis from the other diagnoses, especially leukemic cutis in young patients.

Hydroa vacciniforme and solar urticaria.

Hydroa vacciniforme (HV) and solar urticaria (SU) are uncommon immunologically mediated photodermatoses. HV occurs almost exclusively in children, usually beginning in childhood and remitting spontaneously by adolescence. Association with chronic Epstein-Barr virus infection has been reported in HV, which raises the possibility of lymphoproliferative disorders in these patients. SU is characterized by skin erythema, swelling, and whealing immediately after sun exposure. Although several treatment options are available, the management of both conditions remains a challenge.