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Background: Dupilumab is approved for moderate-severe atopic dermatitis (AD) in patients aged ≥6 months by the US Food and Drug Administration and Health Canada; however, there are little real-world data because providers have limited practical experience with this recently approved therapy., Objectives: To describe the real-world effectiveness and safety in patients aged <12 years with moderate-severe AD currently receiving or previously having received dupilumab., Methods: A multicenter retrospective study was conducted at six Canadian sites. Cases were divided into Group 1 ≤2 years old, Group 2 >2 to <6 years old, and Group 3 ≥6 to <12 years old. Medical history and details of dupilumab treatment were collected. The primary outcome was to measure the improvement in eczema area and severity index. Secondary outcomes examined included the children's dermatology life quality index/infant's dermatitis quality of life, peak pruritus numerical rating scale, and delay to dupilumab access for patients who were considered off-label for dupilumab due to their age., Results: Sixty three pediatric patients (37 males) with moderate-to-severe AD were included; the mean age was 6.4 years old (range: 2-11) when dupilumab treatment was started. Overall, 75% (36/48) achieved EASI-75% and 71% (34/48) achieved EASI-90. EASI-75 and EASI-90 were achieved in 90% (17/19) and 73% (12/19) in patients <6 years old, and 76% (22/29) and 59% (17/29) in patients >6 years old, respectively. No serious adverse events were reported., Conclusions: Dupilumab is safe and effective for patients under the age of 12. However, even for experienced providers, access to the medication was challenging., (© 2023 The Authors. Pediatric Dermatology published by Wiley Periodicals LLC.)

Background: Staphylococcal scalded skin syndrome (SSSS) is a toxin-mediated, blistering skin disorder that mainly affects infants and children. There is limited literature regarding pediatric SSSS. The purpose of this study was to describe the epidemiology, clinical features, and management of pediatric SSSS., Methods: Retrospective cohort study of pediatric patients with a clinical diagnosis of SSSS seen at the Hospital for Sick Children in Toronto, Ontario, Canada, from January 1994 to March 2016., Results: We included 84 patients with a clinical diagnosis of SSSS; 49/84 (58%) were male. Mean age of diagnosis was 3.1 ± 2.4 years. All patients presented with erythema and exfoliation, while 64/84 (76%) presented with vesicles/ bullae. Skin tenderness was the most common symptom, present in 68/84 (81%) subjects. Staphylococcus aureus was more commonly isolated from periorificial cultures than from bullae. Mean hospitalization was 4.7 ± 2.3 days. No difference was found in admission duration between children receiving clindamycin and those that did not (3.6 ± 2.2 vs 3.9 ± 2.34 days, P = .63). Skin debridement was the only risk factor leading to more complications and prolonged hospitalization (P = .03). Severe complications were seen in 4 (5%) cases, and no fatalities were observed., Conclusions: Healthcare providers should be aware of SSSS and consider it in the differential diagnosis of infants and children with new onset erythema, exfoliation, and/or vesiculation. Suspected culprit pathogens were more often obtained from periorificial swabs; however, these isolates were not tested for exfoliative toxin to confirm causality. Antibiotic treatment should be guided by sensitivity testing. Addition of clindamycin as an anti-toxin agent had no effect on the duration of hospitalization, and this should be further investigated. Surgical debridement of the skin in patients with SSSS should be discouraged., (© 2020 Wiley Periodicals LLC.)

Background: Congenital morphea is a form of localized scleroderma that presents at birth. There is limited information on its presentation and progression., Methods: Patients with congenital morphea were identified from five pediatric dermatology and rheumatology tertiary care centers in Canada, the United States, and Italy from 2001 to 2016. Cases from the literature were identified by searching Ovid (EMBASE and MEDLINE) from inception to June 30, 2017. Disease characteristics and prevalence of extracutaneous involvement were analyzed., Results: Thirteen patients were identified from the five centers, and 13 cases were described in the literature, representing 25 patients, with one duplication. Fourteen patients (56%) were female. Median age at diagnosis was 2.9 years (interquartile range 1.2-5.1 years). Linear morphea, including en coup de sabre and Parry-Romberg syndrome, was the most common subtype observed (n = 19, 76%), followed by circumscribed (n = 5, 20%), generalized (n = 2, 8%), and mixed (n = 2, 8%). The face (n = 14, 56%), scalp (n = 8, 32%), and trunk (n = 6, 24%) were the most common locations affected. Most lesions were active at diagnosis (n = 19, 76%), but all patients with follow-up later became inactive. Extracutaneous involvement was seen in 12 (48%) patients, all of whom had linear morphea. Musculoskeletal sequelae were seen in those with linear morphea of the extremities (4/5, 80%), and neurologic involvement was seen in those with linear morphea of the head (8/13, 62%)., Conclusion: Congenital morphea is associated with extracutaneous manifestations and delayed diagnosis. More research is needed to determine whether early recognition, monitoring, and treatment can alter the disease course., (© 2018 Wiley Periodicals, Inc.)

Acrodermatitis enteropathica (AE) is a rare disease that results from a defective gene, SLC39A4, and is characterized by dermatitis, alopecia, and diarrhea. We report a case of AE presenting with only periorificial and acral dermatitis in which genetic testing revealed two novel compound heterozygous missense mutations for SLC39A4. This case demonstrates that not all AE mutations alter zinc transporters in the same manner and highlights the phenotypic variability of AE., (© 2015 Wiley Periodicals, Inc.)

Chemotherapy used in the treatment of malignancies produces multiple mucocutaneous adverse reactions that may be clinically challenging. These mucocutaneous reactions are common and sometimes not diagnosed. The objective of this study was to determine the clinical patterns of the mucocutaneous manifestations during and after chemotherapy in children with a hematologic malignancy and to determine whether nutritional status influences the clinical presentation. We recruited patients aged 6 months to 16 years diagnosed with leukemia and lymphoma from a pediatric hematology outpatient clinic between November 2008 and May 2010. The patients were divided into two groups: Group 1, recently diagnosed patients, included in the study before receiving chemotherapy, and Group 2, patients in surveillance who had not had chemotherapy for at least 3 months. A dermatologic examination was performed, and biopsy and mycological and bacteriological tests were conducted if necessary, with 6 months of follow-up. We evaluated 89 patients and included 65 in the study: 40 boys and 25 girls with an average age of 8.3 years. All patients had skin lesions at some time during their baseline assessment or follow-up. The manifestations found were anagen effluvium, xerosis, and acral hyperpigmentation. To our knowledge, this is the first comparative study of skin manifestations associated with chemotherapy in a Mexican pediatric population. The mucocutaneous manifestations associated with chemotherapy are important causes of morbidity. All of the children in our study had skin lesions on assessment. We did not find an association between skin manifestations and nutritional status., (© 2011 Wiley Periodicals, Inc.)