Severe congenital ichthyosiform dermatosis in CHIME syndrome successfully treated with ixekizumab.

Coloboma, congenital heart disease, ichthyosiform dermatosis, intellectual disability, conductive hearing loss, and epilepsy (CHIME) syndrome is a rare autosomal recessive neuroectodermal disorder caused by PIGL gene mutations. There is emerging literature to support the use of interleukin-17 (IL-17) antagonists in the treatment of certain ichthyosiform dermatoses. Here, we report a case of severe ichthyosiform dermatosis in a child with CHIME syndrome who was recalcitrant to multiple topical medications and dupilumab. This is the first reported case of successful treatment of congenital ichthyosiform dermatosis in a CHIME syndrome patient with ixekizumab, an IL-17A antagonist.

Mydriasis and anisocoria in a pediatric hyperhidrosis patient with interesting findings in the family cat.

Here, we report a case of unilateral ocular mydriasis in a pediatric patient with longstanding hyperhidrosis, as well as similar findings in her cat. The patient had been undergoing treatment of her hyperhidrosis with topical glycopyrrolate. This case highlights the potential side effect profile of topical antimuscarinics and the importance of counseling patients on proper precautions.

Infantile Hemangiomas: A Review and Future Opportunities.

Infantile hemangiomas (IHs) are identified in about 5-12% of infants, making them the most common benign tumor of childhood (Figure 1). IHs are vascular growths characterized by an abnormal proliferation of endothelial cells and aberrant blood vessel architecture.1 IHs commonly involute after proliferation, traditionally leading to a non-interventional or "wait and see" management approach. However, a large subset of these growths can become problematic causing morbidities such as ulceration and scarring, disfigurement, or functional impairment. Another subset of these cutaneous hemangiomas may also be markers for visceral involvement or other underlying abnormalities. Historically, treatment options were often rife with unwanted side effects and modest results. However, with newer established treatments which are both safe and effective, there is a time-sensitive need for early identification of high-risk hemangiomas to ensure prompt delivery of treatment for best outcomes. Despite a more recent dissemination of awareness of IHs and these newer treatments, there remains a large subset of infants who still experience a delay in care and poor outcomes which are likely avoidable. There may be some avenues in Missouri to help mitigate these delays.

Assessing and optimizing readability of dermatology patient education materials (PEMs).

Patient education materials (PEMs) are a powerful tool to improve patient understanding; however, inadequate health literacy is a well-established barrier for PEMs to serve their purpose. The average American reads at an 8th grade level and the National Institute of Health (NIH) recommendation for PEMs is at the 6th grade level. The purpose of this study was to assess and optimize PEMs to identify changes that are most effective at lowering the reading level without diluting its educational content. Edits that decrease the number of syllables per word were most effective at improving readability without diminishing educational content when compared to edits involving the total number of words.

Metastatic Choriocarcinoma Masquerading as a Congenital Glabellar Hemangioma.

Infantile choriocarcinoma (ICC) is a rare, highly malignant form of gestational trophoblastic neoplasia. Rapid diagnosis and initiation of treatment are paramount in reaching a successful outcome. Patients with these tumors typically present with a triad of anemia, hepatomegaly, and precocious puberty. Cutaneous manifestations of ICC are extraordinarily rare with few documented cases. Here, we describe a male neonate who presented to our Dermatology clinic with a rapidly growing, markedly vascular glabellar mass associated with abnormal laboratory values suggestive of Kasabach-Merritt phenomenon. The initial clinical impression of infantile hemangioma led to an initial treatment with propranolol. However, the mass continued to enlarge and a biopsy was obtained. Histology revealed a high-grade, poorly differentiated carcinoma. A robust immunohistochemical battery demonstrated tumor reactivity with Glut-1, GATA3, Glypican-3, CAM5.2, and ß-hCG establishing the diagnosis of metastatic choriocarcinoma. The diagnosis was further supported by the elevated serum ß-hCG. In addition to the glabellar mass, imaging demonstrated tumor foci in the liver and lung. Clinical investigation of the mother revealed no evidence of disease.

Household versus individual approaches to eradication of community-associated Staphylococcus aureus in children: a randomized trial.

BACKGROUND: Community-associated Staphylococcus aureus infections often affect multiple members of a household. We compared 2 approaches to S. aureus eradication: decolonizing the entire household versus decolonizing the index case alone. METHODS: An open-label, randomized trial enrolled 183 pediatric patients (cases) with community-onset S. aureus skin abscesses and colonization of anterior nares, axillae, or inguinal folds from 2008 to 2009 at primary and tertiary centers. Participants were randomized to decolonization of the case alone (index group) or of all household members (household group). The 5-day regimen included hygiene education, twice-daily intranasal mupirocin, and daily chlorhexidine body washes. Colonization of cases and subsequent skin and soft tissue infection (SSTI) in cases and household contacts were ascertained at 1, 3, 6, and 12 months. RESULTS: Among 147 cases with 1-month colonization data, modified intention-to-treat analysis revealed S. aureus eradication in 50% of cases in the index group and 51% in the household group (P = 1.00). Among 126 cases completing 12-month follow-up, S. aureus was eradicated from 54% of the index group versus 66% of the household group (P = .28). Over 12 months, recurrent SSTI was reported in 72% of cases in the index group and 52% in the household group (P = .02). SSTI incidence in household contacts was significantly lower in the household versus index group during the first 6 months; this trend continued at 12 months. CONCLUSIONS: Household decolonization was not more effective than individual decolonization in eradicating community-associated S. aureus carriage from cases. However, household decolonization reduced the incidence of subsequent SSTI in cases and their household contacts. CLINICAL TRIALS REGISTRATION: NCT00731783.

Diversity of Staphylococcus aureus strains colonizing various niches of the human body.

OBJECTIVES: As individuals may be colonized with multiple strains of Staphylococcus aureus at different body sites, the objectives of this study were to determine whether S. aureus polyclonal colonization exists within one body niche and the optimal sampling sites and culture methodology to capture the diversity of S. aureus strains in community-dwelling individuals. METHODS: Swabs were collected from the nares, axillae, and inguinal folds of 3 children with community-associated S. aureus infections and 11 household contacts, all with known S. aureus colonization. S. aureus isolates were recovered from each body niche using 4 culture methods and evaluated for polyclonality using phenotypic and genotypic strain characterization methodologies. RESULTS: Within individuals, the mean (range) number of phenotypes and genotypes was 2.4 (1-4) and 3.1 (1-6), respectively. Six (43%) and 10 (71%) participants exhibited phenotypic and genotypic polyclonality within one body niche, respectively. Broth enrichment yielded the highest analytical sensitivity for S. aureus recovery, while direct plating to blood agar yielded the highest genotypic strain diversity. CONCLUSIONS: This study revealed S. aureus polyclonality within a single body niche. Culture methodology and sampling sites influenced the analytical sensitivity of S. aureus colonization detection and the robustness of phenotypic and genotypic strain recovery.

Staphylococcus aureus colonization in children with community-associated Staphylococcus aureus skin infections and their household contacts.

OBJECTIVES: To measure prevalence of Staphylococcus aureus colonization in household contacts of children with acute S aureus skin and soft tissue infections (SSTI), determine risk factors for S aureus colonization in household contacts, and assess anatomic sites of S aureus colonization in patients and household contacts. DESIGN: Cross-sectional study. SETTING: St Louis Children's Hospital Emergency Department and ambulatory wound center and 9 community pediatric practices affiliated with a practice-based research network. PARTICIPANTS: Patients with community-associated S aureus SSTI and S aureus colonization (in the nose, axilla, and/or inguinal folds) and their household contacts. OUTCOME MEASURES: Colonization of household contacts of pediatric patients with S aureus colonization and SSTI. RESULTS: Of 183 index patients, 112 (61%) were colonized with methicillin-resistant S aureus (MRSA); 54 (30%), with methicillin-sensitive S aureus (MSSA); and 17 (9%), with both MRSA and MSSA. Of 609 household contacts, 323 (53%) were colonized with S aureus: 115 (19%) with MRSA, 195 (32%) with MSSA, and 13 (2%) with both. Parents were more likely than other household contacts to be colonized with MRSA (odds ratio, 1.72; 95% CI, 1.12 to 2.63). Methicillin-resistant S aureus colonized the inguinal folds more frequently than MSSA (odds ratio, 1.67; 95% CI, 1.16 to 2.41), and MSSA colonized the nose more frequently than MRSA (odds ratio, 1.75; 95% CI, 1.19 to 2.56). CONCLUSIONS: Household contacts of children with S aureus SSTI had a high rate of MRSA colonization compared with the general population. The inguinal fold is a prominent site of MRSA colonization, which may be an important consideration for active surveillance programs in hospitals.

Effectiveness of measures to eradicate Staphylococcus aureus carriage in patients with community-associated skin and soft-tissue infections: a randomized trial.

BACKGROUND: Despite a paucity of evidence, decolonization measures are prescribed for outpatients with recurrent Staphylococcus aureus skin and soft-tissue infection (SSTI). OBJECTIVE: Compare the effectiveness of 4 regimens for eradicating S. aureus carriage. DESIGN: Open-label, randomized controlled trial. Colonization status and recurrent SSTI were ascertained at 1 and 4 months. SETTING: Barnes-Jewish and St. Louis Children's Hospitals, St. Louis, Missouri, 2007-2009. PARTICIPANTS: Three hundred patients with community-onset SSTI and S. aureus colonization in the nares, axilla, or inguinal folds. INTERVENTIONS: Participants were randomized to receive no therapeutic intervention (control subjects) or one of three 5-day regimens: 2% mupirocin ointment applied to the nares twice daily, intranasal mupirocin plus daily 4% chlorhexidine body washes, or intranasal mupirocin plus daily dilute bleach water baths. RESULTS: Among 244 participants with 1-month colonization data, modified intention-to-treat analysis revealed S. aureus eradication in 38% of participants in the education only (control) group, 56% of those in the mupirocin group (P = .03 vs controls), 55% of those in the mupirocin and chlorhexidine group (P = .05), and 63% off those in the mupirocin and bleach group (P = .006). Of 229 participants with 4-month colonization data, eradication rates were 48% in the control group, 56% in the mupirocin only group (P = .40 vs controls), 54% in the mupirocin and chlorhexidine group (P = .51), and 71% in the mupirocin and bleach group (P = .02). At 1 and 4 months, recurrent SSTIs were reported by 20% and 36% of participants, respectively. CONCLUSIONS: An inexpensive regimen of dilute bleach baths, intranasal mupirocin, and hygiene education effectively eradicated S. aureus over a 4-month period. High rates of recurrent SSTI suggest that factors other than endogenous colonization are important determinants of infection. Trial registration. ClinicalTrials.gov identifier: NCT00513799.