IMPORTANCE Hidradenitis suppurativa (HS) in pediatric patients has been understudied. Increased awareness and recognition of HS prevalence in children demand efforts to better understand this condition.OBJECTIVE To describe the demographics, clinical features, treatment, associated comorbidities, and outcomes in a large cohort of pediatric patients with HS.DESIGN, SETTING, AND PARTICIPANTS International, multicenter, retrospective medical record review of pediatric patients (aged 1-18 years) with a clinical diagnosis of HS carried out in 10 dermatology clinics across the US,
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.
IMPORTANCE Propranolol for infantile hemangiomas (IH) has been shown to be effective and relatively safe. However, other less lipophilic β-blockers, such as nadolol, may be preferable in individuals who experience propranolol unresponsiveness or adverse events. OBJECTIVE To document the noninferiority and safety of oral nadolol compared with oral propranolol in infants with IH.DESIGN, SETTING, AND PARTICIPANTS This double-blind noninferiority prospective study with a noninferiority margin of 10% compared propranolol with nadolol in infants aged 1 to 6 months with problematic IH. The study was conducted in 2 academic pediatric dermatology centers in Canada between 2016 and 2020. Infants aged 1 to 6 months with a hemangioma greater than 1.5 cm on the face or 3 cm or greater on another body part causing or with potential to cause functional impairment or cosmetic disfigurement.INTERVENTIONS Oral propranolol and nadolol in escalating doses up to 2 mg/kg/d. MAIN OUTCOMES AND MEASUREBetween-group differences comparing changes in the bulk (size and extent) and color of the IH at week 24 with baseline using a 100-mm visual analog scale. RESULTSThe study included 71 patients. Of these, 36 were treated with propranolol. The mean (SD) age in this group was 3.1 (1.4) months, and 31 individuals (86%) were female. Thirty-five infants were treated with nadolol. The mean (SD) age in this group was 3.2 (1.6) months, and 26 individuals (74%) were female. The difference in IH between groups by t test was 8.8 (95% CI, 2.7-14.9) for size and 17.1 (95% CI, 7.2-30.0) for color in favor of the nadolol group, demonstrating that nadolol was noninferior to propranolol. Similar differences were noted at 52 weeks: 6.0 (95% CI, 1.9-10.1) and 10.1 (95% CI, 2.9-17.4) for size and color improvement, respectively. For each doubling of time unit (week), the coefficient of involution was 2.4 (95% CI, 0.5-4.4) higher with nadolol compared with propranolol. Safety data were similar between the 2 interventions.CONCLUSIONS AND RELEVANCE Oral nadolol was noninferior to oral propranolol, indicating it may be an efficacious and safe alternative in cases of propranolol unresponsiveness or adverse events, or when faster involution is required.
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.
Drug‐induced skin disease or cutaneous adverse drug reactions (CADRs) are terms that encompass the clinical manifestations of the skin, mucosae and adnexa induced by a drug or its metabolites. The skin is the organ most frequently affected by drug reactions, which may affect up to 10% of hospitalized patients and occur in 1–3% of multimedicated patients. Most CADRs are mild or self‐resolving conditions; however, 2–6.7% of could develop into potentially life‐threatening conditions. CADRs represent a heterogeneous field and can be diagnostically challenging as they may potentially mimic any dermatosis. Currently, there are between 29–35 different cutaneous drug‐reaction patterns reported ranging from mild dermatitis to an extensively burnt patient. The most frequently reported are maculopapular rash, urticaria/angioedema, fixed drug eruption and erythema multiforme. Less common but more severe patterns include erythroderma, drug reaction with eosinophilia and systemic symptoms, and Stevens–Johnson syndrome/toxic epidermal necrolysis spectrum. Almost any drug can induce a CADR, but antibiotics, nonsteroidal anti‐inflammatory drugs and antiepileptics are the most frequently involved. Different mechanisms are involved in the pathogenesis of CADRs, although in some cases, these remain still unknown. CADRs could be classified in different ways: (i) type A (augmented) or type B (bizarre); (ii) immediate or delayed; (iii) immune‐mediated or nonimmune‐mediated; (iv) nonsevere or life‐threatening; and (v) by their phenotype, including exanthematous, urticarial, pustular and blistering morphology. Recognizing a specific CADR will mostly depend on the ability of the physician to perform a detailed clinical examination, the proper description of the morphology of the skin lesions and supporting laboratory and/or skin biopsy findings.
Background: Esophageal strictures are the common gastrointestinal complications in patients with epidermolysis bullosa (EB) requiring dilation. There is limited information on the best type of intervention, outcomes, and predictors for re-stenosis. Objectives: We aimed to investigate the frequency, clinical presentation of esophageal strictures in EB patients, and to ascertain the predictors of re-stenosis. Methods: We conducted a retrospective, multicenter cohort study involving 7 specialized, international EB centers on patients who were 0 to 50 years of age. Descriptive statistics and hazard risks for re-stenosis were calculated. Results: We identified 125 patients with 497 esophageal stricture episodes over a mean period of observation of 17 (standard deviation [SD] = 11.91) years. Dilations were attempted in 90.74% of episodes, using guided fluoroscopy 45.23%, retrograde endoscopy 33.04%, and antegrade endoscopy 19.07%. Successful dilation was accomplished in 99.33% of attempts. Patients experienced a median of 2 (interquartile range [IQR]: 1–7) stricture episodes with a median interval between dilations of 7 (IQR: 4–12) months. Predictors for re-stenosis included: number of strictures (2 vs 1 stricture: χ2 = 4.293, P = 0.038, hazard ratio [HR] = 1.294 (95% confidence interval [CI]: 1.014--1.652 and 3 vs 1 stricture:χ2 = 7.986, P = 0.005, HR = 1.785 [95% CI: 1.194, 2.667]) and a long (≥1 cm) segment stricture (χ2 = 4.599, P = 0.032, HR = 1.347 (95% CI: 1.026--1.769). Complications were more common with the endoscopic approach (8/86, antegrade endoscopy; 2 /149, retrograde endoscopy vs 2/204, fluoroscopy; χ 2 = 17.39, P-value <0.000). Conclusions: We found excellent dilation outcomes irrespective of the dilation procedure; however, with higher complications in the endoscopic approach. Long (>1 cm) segment involvement and multiple locations were predictive of stricture reoccurrence.
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