Parental and Child-Reported Health-Related Quality of Life in Pediatric Patients With Vascular Malformations.

Health-related quality of life (HRQoL) in pediatric patients with vascular malformations (VaM) remains challenging to measure due to an absence of disease-specific tools and limited correlation with clinical measures. In this cross-sectional study of 50 children with VaM we evaluated self and parental-reported physical and psychosocial HRQoL using the PedsQL tool and analyzed scores according to type of VaM and age categories. We found HRQoL is considerably decreased in children with VaM and perceived similarly by patients and their parents, except for those with simple VaM and ages 13-18 years, who scored physical domains better than their parents.

Vascular malformations in pediatric patients: 10-year experience of a vascular anomalies clinic.

Background: Vascular malformations (VaMs) are caused by errors in vascular morphogenesis. Diagnosis and treatment can be complex. Few specialized centers care for these patients, and limited literature exists regarding their characteristics and clinical course.

Livedo-like acute cutaneous graft-versus-host disease in two children.

We present two pediatric patients who exhibited an unusual clinical presentation of cutaneous acute graft-versus-host disease (GVHD), characterized by livedo-like appearance. Such manifestations of cutaneous acute GVHD have not been previously documented.

Responsiveness to Change of the Morphea Activity Measure in Pediatric Patients.

Importance: Detecting activity of morphea can be complex but is crucial for adequate treatment and outcome assessment. The Morphea Activity Measure (MAM) was recently validated, but its responsiveness to change in disease activity has not been studied.

Objective: To evaluate the internal and external responsiveness of MAM to changes in disease activity in pediatric patients.

On-person adaptive evolution of Staphylococcus aureus during treatment for atopic dermatitis.

The opportunistic pathogen Staphylococcus aureus frequently colonizes the inflamed skin of people with atopic dermatitis (AD) and worsens disease severity by promoting skin damage. Here, we show, by longitudinally tracking 23 children treated for AD, that S. aureus adapts via de novo mutations during colonization.

Concordance between referral and final diagnoses of pediatric patients with vascular malformations.

Background: Vascular malformations (VaM) are a heterogeneous group of disorders resulting from the dysmorphogenesis of blood vessels. Although correct classification is relevant to providing adequate treatment according to evidence-based medicine, diagnostic terminology may be misused or need clarification.

Methods: We conducted a retrospective study to measure agreement and concordance between referral and final confirmed diagnoses of 435 pediatric patients with VaM newly referred to the multidisciplinary Vascular Anomalies Clinic (VAC) using Fleiss kappa (κ) concordance analysis.

Development and Validation of the Morphea Activity Measure in Patients With Pediatric Morphea.

Importance: Morphea is an insidious inflammatory disorder of the skin and deeper tissues. Determining disease activity is challenging yet important to medical decision-making and patient outcomes.

Objective: To develop and validate a scoring tool, the Morphea Activity Measure (MAM), to evaluate morphea disease activity of any type or severity that is easy to use in clinical and research settings.

The use of mTOR inhibitors for the treatment of kaposiform hemangioendothelioma. A systematic review.

Background: Kaposiform hemangioendothelioma (KHE) is a locally aggressive and potentially lethal vascular tumor of infancy. Current consensus recommendations include the use of vincristine and/or systemic steroids as first-line treatment. Mammalian target of rapamycin (mTOR) inhibitors represent a promising therapy for patients with KHE.

Assessing pain catastrophizing and functional disability in pediatric epidermolysis bullosa patients.

Background/objectives: The primary objective was to assess pain catastrophizing and functional disability in pediatric patients with epidermolysis bullosa (EB) and their parents/guardians. Secondary objectives included examining relationships between pain catastrophizing, functional disability, and correlations with other factors (e.g.

Epidermolysis Bullosa Acquisita: A Rare Autoimmune Blistering Disease in Children.

A 7-year-old girl presented with a 2-year history of recurrent blisters on the skin and oral mucosa. The patient was otherwise healthy, and her family history was unremarkable for any dermatologic or other medical disease. Examination revealed multiple tense vesicles, milia, and atrophic scars present over the extensor surface of the extremities and erosions on the oral mucosa (Figure 1).

North American Blastic Plasmacytoid Dendritic Cell Neoplasm Consortium: position on standards of care and areas of need.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with historically poor outcomes and no worldwide consensus treatment approach. Unique among most hematologic malignancies for its frequent cutaneous involvement, BPDCN can also invade other extramedullary compartments, including the central nervous system. Generally affecting older adults, many patients are unfit to receive intensive chemotherapy, and although hematopoietic stem cell transplantation is preferred for younger, fit individuals, not all are eligible.

Systemic treatment for severe atopic dermatitis in children: a case series.

Background: Atopic dermatitis (AD) is children's most frequent chronic inflammatory skin disease. In most patients, this condition is controlled with topical treatments; however, some patients with severe AD do not respond to these treatments, requiring systemic therapy. There is insufficient information about the ideal dose, time of use, clinical response, and safety of systemic therapy in children with severe AD.

Covid-19 mortality in children and adolescents in Mexico.

Objective: To estimate Covid-19 and pre-pandemic low respiratory infection (LRI) mortality in children and adolescents in Mexico.

Materials And Methods: We estimated the percentage of total mortality attributable to Covid-19 (95% confidence intervals; 95%CI) and made the corresponding estimates for pre-pandemic LRI mortality.

Results: In 2019, LRIs represented 8.

Bleach baths for atopic dermatitis: A systematic review and meta-analysis including unpublished data, Bayesian interpretation, and GRADE.

Background: Bleach bathing is frequently recommended to treat atopic dermatitis (AD), but its efficacy and safety are uncertain.

Objective: To systematically synthesize randomized controlled trials (RCTs) addressing bleach baths for AD.

Methods: We searched MEDLINE, EMBASE, CENTRAL, and GREAT from inception to December 29, 2021, for RCTs assigning patients with AD to bleach vs no bleach baths.

Infections With Enterohepatic Non- Species in X-Linked Agammaglobulinemia: Clinical Cases and Review of the Literature.

The genus is classified into two main groups according to its habitat: gastric and enterohepatic. Patients with X-linked agammaglobulinemia (XLA) appear to be associated with invasive infection with enterohepatic non-Helicobacter pylori species (NHPH), mainly and . Such infections are difficult to control and have a high potential for recurrence.

Atypical purpura and other features associated with unfavorable outcomes of IgA vasculitis (Henoch-Schönlein purpura) in children: A retrospective study.

The prognosis of IgA vasculitis (also known as Henoch-Schönlein purpura) is determined by renal or other organ involvement. We conducted a retrospective study to identify the initial features of 106 children with IgA vasculitis and their association with unfavorable outcomes. Location of purpura above the waist and an altered urinalysis at diagnosis predicted a more aggressive course of disease.

Early-onset juvenile dermatomyositis: A report of two cases and review of the literature.

Juvenile dermatomyositis (JDM) is an uncommon disease in children younger than 3 years of age. The clinical manifestations may be different than in older children, often delaying the diagnosis. We present two patients with early-onset JDM and review the literature describing the unique clinical characteristics in this age group.