Bullous Aplasia Cutis as a Presenting Sign of Encephalocele.

This case report describes a congenital cystic scalp nodule on a 2-week-old infant.

Assessment of the American Academy of Dermatology diagnostic criteria for pediatric atopic dermatitis and modification into a checkbox form: A cross-sectional study.

BACKGROUND/OBJECTIVES: Diagnostic criteria for atopic dermatitis (AD) are limited in their performance and/or usability. The American Academy of Dermatology (AAD) consensus criteria include hierarchical categories of disease features to improve these metrics but have not been validated. Our objective was to create and validate a checkbox form of the AAD consensus criteria in the pediatric population. METHODS: We performed a cross-sectional study of 100 pediatric patients with AD (n = 58) and diseases in the differential diagnosis of AD (n = 42). RESULTS: Having three or more "Essential," ≥2 "Important," ≥1 "Associated" features of the AAD criteria was optimal for the diagnosis of AD in children. This combination was 91.4% (95% CI, 84.2%-98.6%) sensitive and 95.2% (88.8%-100%) specific. The UK working party criteria and the Hanifin-Rajka criteria had sensitivities of 96.6% (95% CI 91.9%-100%) and 98.3% (95% CI 94.9%-100%) and specificities of 83.3% (95% CI 72.1%-94.6%) and 71.4% (95% CI 57.8%-85.1%), respectively. The AAD criteria had significantly greater specificity than the Hanifin-Rajka criteria (p = .002). CONCLUSIONS: This study represents an important step in validating the AAD consensus criteria and formulating a useable checkbox form for diagnosing AD in the pediatric population.

Severe acne and its variants: Exploring its natural history and heritability.

BACKGROUND: Acne vulgaris varies in clinical severity, from minimal comedonal disease to severe hemorrhagic and ulcerative lesions with scarring. While a family history confers a higher risk for developing acne, the correlation between heritability and clinical severity remains unclear. OBJECTIVE: To examine the natural history and heritability of severe acne with scarring in patients undergoing isotretinoin therapy. METHODS: A total of 101 subjects with severe acne with scarring and its variants, including acne conglobata and acne fulminans, were enrolled. All subjects and adult family members underwent an interview regarding their acne, and a corresponding "historical" Investigator's Global Assessment (hIGA) score (0 = clear, 1 = almost clear, 2 = mild, 3 = moderate, 4 = severe, 5 = very severe) was assigned. Study assessors performed an "examination" Investigator's Global Assessment (eIGA) based on the clinical examination of each subject (0 = clear, 1 = almost clear, 2 = mild, 3 = moderate, 4 = severe, 5 = very severe). A detailed family history and pedigree were documented. RESULTS: Most subjects were Caucasian (44.5%) and male (79.2%) who had previously used doxycycline and/or minocycline (86.1%). The mean eIGA and hIGA scores were 2.7 and 4.4, respectively. 37.2% of subjects had one first-degree relative with a history of moderate or severe acne with scarring; of note, of the patients with hemorrhagic disease, 30% had at least one parent with moderate or severe acne. CONCLUSIONS: Severe forms of acne often "cluster" in families, underscoring the heritable nature of acne and the prognostic value of a family history of moderate or severe disease.

Writing in the Margins of Sexual Function Questionnaires: A Qualitative Analysis of Data From Women With Pelvic Floor Disorders.

BACKGROUND: The impact of pelvic floor disorders (PFDs) on female sexual function is not well understood, partly due to difficulties in measurement and evaluation. AIM: We sought to assess how women with PFDs respond to sexual function questionnaires through an analysis of survey marginalia, or the comments written in the margins of fixed-choice surveys. METHODS: 94 women with PFDs completed validated written sexual function questionnaires (Global Study of Sexual Attitudes and Behaviors survey, Female Sexual Function Index, and the Pelvic Organ Prolapse/Urinary Incontinence Sexual Questionnaire, International Urogynecological Association-Revised). Marginalia, or the additions, eliminations, and changes subjects made (by hand) to survey items, were collected. Data were coded and analyzed qualitatively using grounded theory methodology. OUTCOMES: Themes and emergent concepts related to the content of survey marginalia were the primary outcomes of this study. RESULTS: We observed 177 instances of marginalia across all questionnaires. Qualitative analysis revealed 7 preliminary themes and 2 emergent concepts. Preliminary themes included partner-related topics, loss, problems during intercourse, emotional problems, other medical problems, and survey answer choices failing to capture the spectrum of patient experiences. Emergent concepts revealed highly diverse sexual function in this population and a wide range of factors that influence sexual function. CLINICAL IMPLICATIONS: Conducting qualitative studies alongside sexual function questionnaires can allow for a more meaningful assessment of the sexual function of women with various underlying conditions, such as PFDs. STRENGTHS & LIMITATIONS: This is the first study of its kind to analyze survey marginalia from sexual function questionnaires among women with PFDs. The limitations of this study include the inherently spontaneous nature of marginalia data. In addition, the ways in which study participants responded to sexual function questionnaires in our study may not be reflective of all potential subjects. CONCLUSION: Analysis of survey marginalia from sexual function questionnaires amongst women with PFDs revealed new information regarding patients' histories, concerns, and thoughts. Over half of the women in this study felt the need to expand, explain, or eliminate responses from the questionnaires. Many subjects were no longer sexually active, which accounted for a large majority of participants leaving questions blank or responding with "N/A." Standard sexual evaluation tools may fail to capture the complexity, spectrum, and depth and breadth of patient experiences. Parameshwar PS, Borok J, Jung E, et al. Writing in the Margins of Sexual Function Questionnaires: A Qualitative Analysis of Data From Women With Pelvic Floor Disorders. J Sex Med 2020;17:1705-1714.

The microbiome in preadolescent acne: Assessment and prospective analysis of the influence of benzoyl peroxide.

BACKGROUND/OBJECTIVES: The pathogenesis of preadolescent acne has not been well studied, and it is uncertain if Cutibacterium acnes is a predominant organism in the microbiome in this age group. The aim of this study was to analyze the microbiome of preadolescent females and to assess whether benzoyl peroxide impacts the microbiome. METHODS: The study enrolled girls, aged 7-12 years, with evidence of at least six acne lesions who had not been previously treated. Participants' skin surface of forehead, cheeks, nose, chin, left retroauricular crease, and extruded contents of a comedonal lesion were sampled at baseline. Participants used benzoyl peroxide 4% wash for 6-8 weeks and returned for skin surface sampling and extraction collection. Microbiome analysis was performed using 16S ribosomal RNA gene amplicon sequencing on all swab and lesional extraction samples. RESULTS: Fifty-one participants were enrolled with a median IGA score of 2 (mild). Changes in microbiome diversity were associated with increasing age and number of acne lesions (P = 0.001). C. acnes had higher abundances on forehead and nose, as opposed to cheeks and chin (P = 0.009). Bacterial diversity (alpha diversity) of the skin microbiome was comparable between preadolescent at baseline and after treatment with benzoyl peroxide. CONCLUSION: This is the first large assessment characterizing female acne microbiome in early and late preadolescence. Results show that preadolescent acne can vary in its microbial profile, reflecting surrounding changes associated with the onset of puberty. Although benzoyl peroxide use was associated with decreased acne counts, its effect on microbial diversity was not demonstrated in our study.

Contact Dermatitis in Atopic Dermatitis Children-Past, Present, and Future.

Allergic contact dermatitis (ACD) used to be considered a rarity in children, but recently has been estimated to effect 4.4 million children in the USA alone, with a notable rise in investigative research in the field of pediatric ACD. Researchers have shown that patch testing is safe and effective in afflicted children and that those with atopic dermatitis (AD) have similar sensitization rates, although they have a higher sensitization to certain allergens, thought to be related to the inflammatory (IL-4) milieu. Patch testing assessment guidelines in children include five key considerations: if a patient's dermatitis worsens, changes distribution, fails to improve with topical therapy, or immediately rebounds after removal of topical treatments; if a patient has a particular distribution of dermatitis; if a working patient has hand eczema that fails to improve with therapy; if the patient has AD that started in adolescence or adulthood with definitely no history of childhood eczema; and importantly, if a patient has severe or widespread atopic dermatitis that will require immunosuppressive systemic medication.

Hand-foot-skin reaction related to use of the multikinase inhibitor sorafenib and hard orthotics.

Hand-foot-skin reaction is a distinct clinical condition arising in association with the use of multikinase inhibitors, including sorafenib. Because multikinase inhibitors are increasingly being used in children with cancer, recognition of this previously unfamiliar condition is of importance to pediatric dermatologists. We describe the diagnosis and successful treatment of a case of hand-foot-skin reaction in a child taking sorafenib for an unresectable desmoid tumor.

Idiopathic facial aseptic granuloma-A diagnostic challenge in pediatric dermatology.

Idiopathic facial aseptic granuloma is a distinct, benign lesion that presents in very young children and is characterized by a painless facial nodule that usually appears on the cheek. It is typically characterized by a prolonged course but heals spontaneously or in response to antibiotic treatment. The challenge is to diagnose this entity correctly, ideally based on clinical acumen, to avoid surgical intervention with facial sutures and the resultant scarring and unnecessary treatment interventions. In this article, we discuss three cases of idiopathic facial aseptic granuloma to raise awareness and highlight the diagnostic challenges and possible link to childhood rosacea.

Atopic dermatitis: emerging therapies.

Crisaborole and dupilumab represent the first 2 Food and Drug Administration (FDA)-approved therapies for atopic dermatitis (AD) in more than 15 years, and there are many promising drugs currently in development. This new wave of therapeutics capitalizes on the large body of work clarifying the pathogenesis of AD over the last several decades. In particular, type 2 cytokine-driven inflammation and skin barrier dysfunction are key processes underlying AD pathogenesis.

Atopic dermatitis: therapeutic care delivery: therapeutic education, shared decision-making, and access to care.

Atopic dermatitis (AD) is a chronic skin condition affecting children and adults, with a significant negative impact on patient and caregiver quality of life (QOL). Although effective treatments for AD are available, outcomes are often limited by poor adherence to treatment plans. Effective patient and caregiver education about the disease and its management is a necessary and important component of AD care. Therapeutic patient education (TPE) is a patient-centered process that aims to transfer information and skills necessary to manage and cope with a disease from health care professionals to patients and caregivers. Shared decision making between the health care provider and the patient/caregiver is an integral component of the TPE process and recognizes the importance of both the medical provider's clinical expertise, as well as the patient/caregiver's preferences and experiences regarding their own medical condition and its treatment. TPE programs for patients with AD and their caregivers are typically provided by multidisciplinary teams and utilize a number of different methods and tools to facilitate the transfer of knowledge and skills through both individual care and group-based educational sessions. TPE has been demonstrated to improve outcomes such as AD disease severity, treatment adherence, QOL, and coping with itch. It is important to consider strategies to reduce barriers to cost-effective accessible AD education and treatment.

Atopic dermatitis: skin care and topical therapies.

Atopic dermatitis (AD) pathogenesis is strongly influenced by Type 2 innate lymphoid cell and T-helper cell type 2 lymphocyte-driven inflammation and skin barrier dysfunction. AD therapies attempt to correct this pathology, and guidelines suggest suggest basics of AD therapy, which include repair of the skin barrier through bathing practices and moisturizers, infection control, and further lifestyle modifications to avoid and reduce AD triggers.While some patients' AD may be controlled using these measures, inflammatory eczema including acute flares and maintenance therapy in more severe patients are treated with topical pharmacologic agents such as topical corticosteroids, topical calcineurin inhibitors, and, more recently, topical PDE-4 inhibitors. This model of basic skin therapy and, as needed, topical pharmacologic agents may be used to treat the vast majority of patients with AD and remains the staple of AD therapy.

Atopic dermatitis: phototherapy and systemic therapy.

The majority of atopic dermatitis (AD) patients respond satisfactorily to gentle bathing, frequent moisturizing, and topical medications. Second-line therapies for AD should be used in recalcitrant cases or in patients with uncontrolled disease despite compliance with first-line measures and avoidance of allergens. Recommended advanced therapies include phototherapy, especially narrowband ultraviolet B, systemic immunosuppressants, and a new biologic agent. Few studies have compared head-to-head efficacy of the different immunosuppressant therapies such as cyclosporine, methotrexate, azathioprine and mycophenolate mofetil. Therefore, the agent used is based on provider and patient preferences and can be decided on a case-by-case basis.

Current and emerging topical therapies for atopic dermatitis.

The pathogenesis of atopic dermatitis (AD) involves epidermal barrier dysfunction and T helper cell type 2 (Th2) lymphocyte-driven inflammation. Cytokines, such as interleukin 4 (IL-4) and IL-13, are important in this reaction. They stimulate B cells to produce immunoglobulin E, causing atopic disease. This process has been well characterized, and new therapies for AD, such as phosphodiesterase 4 (PDE-4) inhibitors, Th2-expressed chemoattractant receptor-homologous molecule antagonists, and Janus kinase inhibitors, work by antagonizing this cellular pathway. Recently, there have been many advances in treatment strategies and novel therapies for AD. This review summarizes the clinical evidence supporting the use of current and emerging topical treatments for AD, as well as their safety and efficacy profiles. Crisaborole, a novel PDE-4 inhibitor, is of particular note because phase III clinical trials were recently completed, as summarized here. It is prudent for dermatologists to be current with updates in the field because therapies are constantly changing. In addition to the academic interest, this results in improvement of patient care and advancement of the field.

Phosphodiesterase 4 Inhibitor Therapies for Atopic Dermatitis: Progress and Outlook.

Phosphodiesterase 4 (PDE4) is a cyclic AMP degrading enzyme in leukocytes. Several decades ago, increased PDE activity was demonstrated in patients with atopic dermatitis (AD). Currently, several PDE4 inhibitors in both topical and oral formulation have been developed to target the inflammatory cascade of AD. This review shows the pathogenic rationale behind these inhibitors, and discusses multiple PDE4 inhibitors that are under evaluation or in the market. PDE4 inhibitors may be considered as favorable agents in the repertoire of current interventions for AD.

Current guidelines for the evaluation and management of atopic dermatitis: A comparison of the Joint Task Force Practice Parameter and American Academy of Dermatology guidelines.

Atopic dermatitis (AD) is a chronic pruritic inflammatory disease that commonly presents in the pediatric population. Although definitions and diagnosis of AD have largely been agreed upon, allergists and dermatologists have similar and divergent approaches to the management of AD. This review facilitated integration of the American Academy of Allergy, Asthma & Immunology/American College of Allergy, Asthma & Immunology Joint Task Force 2012 AD Practice Parameter and the 2014 American Academy of Dermatology guidelines to highlight the basic principles of AD management and discuss therapies and management of AD from the distinct perspectives of the allergist and dermatologist.