Neonatal nasal necrosis: Case series and brief review.

There is limited information available on pressure-related neonatal nasal injuries. We present three neonates born with erythema and purpura of the nasal tip that subsequently ulcerated, then evolved into a thick eschar. Each healed well with conservative management but left behind significant scarring. The sharp demarcation and location of the lesions were suggestive of hypoxic tissue damage akin to halo scalp ring alopecia. Further investigation is necessary to elucidate the etiology and optimal management of this condition.

Low rates of neurological abnormalities in patients with pigmentary mosaicism: A retrospective cohort study from a tertiary dermatology center.

BACKGROUND: Pigmentary mosaicism (PM) is a descriptive term encompassing a range of hyper- and hypo-pigmented phenotypes in various patterns. Information from the neurology literature initially noted neurological abnormalities (NA) in up to 90% of children with PM. The dermatology literature suggests lower associated rates (15%-30%) of NA. Variations in terminology, inclusion criteria, and small population sizes makes interpreting existing PM literature complicated. We aimed to assess rates of NA in children presenting to dermatology with PM. METHODS: We included patients <19 years, diagnosed with PM, nevus depigmentosus and/or segmental café au lait macules (CALM) seen in our dermatology department between 1 January 2006 and 31 December 2020. Patients with neurofibromatosis, McCune-Albright syndrome, and non-segmental CALM were excluded. Data collected included pigmentation, pattern, site(s) affected, presence of seizures, developmental delay, and microcephaly. RESULTS: One hundred fifty patients were included (49.3% female), with a mean age at diagnosis of 4.27 years. Patterns of mosaicism were ascertained for 149 patients and included blaschkolinear (60/149, 40.3%), blocklike (79/149, 53.0%), or a combination of both patterns (10/149, 6.7%). Patients with a combination of patterns were more likely to have NA (p < .01). Overall, 22/149 (14.8%) had NA. Nine out of twenty-two patients with NA had hypopigmented blaschkolinear lesions (40.9%). Patients with ≥4 body sites affected were more likely to have NA (p < .01). DISCUSSION: Overall, our population had low rates of NA in PM patients. A combination of blaschkolinear and blocklike patterns, or ≥4 body sites involved were associated with higher rates of NA.

Central nervous system magnetic resonance imaging abnormalities and neurologic outcomes in pediatric patients with congenital nevi: A 10-year multi-institutional retrospective study.

BACKGROUND: High-risk congenital melanocytic nevi (CMN) are associated with abnormalities of the central nervous system (CNS), prompting magnetic resonance imaging (MRI) screening guidelines. OBJECTIVE: Describe MRI brain and spine abnormalities in children with CMN and report trends between nevus features, MRI findings, and neurologic outcomes. METHODS: Retrospective review of individuals aged ≤18 years with an MRI of the brain and/or spine and at least 1 dermatologist-diagnosed CMN. RESULTS: Three hundred fifty-two patients were identified. Forty-six children had CMN that prompted an MRI of the brain and/or spine (50% male, average age at first image, 354.8 days). In these children, 8 (17%) had melanin detected in the CNS, of whom all had >4 CMN. One developed brain melanoma (fatal). In patients without CNS melanin, 4 had concerning imaging. Concerning MRI patients had more neurodevelopmental problems, seizures, neurosurgery, and death than individuals with unremarkable imaging. Three hundred six patients received MRIs for other reasons; none detected melanin. No children with only multiple small CMN (n = 15) had concerning imaging. LIMITATIONS: Lack of a control group, cohort size, and retrospective methods. CONCLUSION: MRI of the brain and spine is useful for detecting intervenable abnormalities in high-risk children. Healthy infants with few small CMN may not require screening MRI.

Utility of routine patch testing prior to surgical repair of pectus excavatum: A multidisciplinary experience via retrospective review at a single tertiary care center.

BACKGROUND: The Nuss procedure is the only implant procedure in which routine, rather than selective, preoperative patch testing is recommended. This practice has recently been called into question. OBJECTIVE: To evaluate an updated experience of pre-implant patch testing in patients undergoing the Nuss procedure. METHODS: A retrospective chart review of Nuss procedures from 2012 through 2020. RESULTS: Forty-five patients were identified for data collection. From 2012 to 2014, none of the 14 patients were patch tested. From 2015 to 2020, 26 of 31 (83.9%) were patch tested. Of those tested, only 2 had a positive patch test. A hypoallergenic titanium bar was inserted in each case. In total, there were zero bar reactions. CONCLUSION: The risks of patch testing are extremely small and should be weighed against the serious implications of even one bar allergy when deciding on future guidelines.

Dermatologic conditions in Down syndrome.

Down syndrome (DS) is the most common chromosomal condition and affects many organs including the skin. Dermatologists are an integral part of the DS care team. This is a review of both common and rare dermatologic conditions in DS. We provide practical strategies for a successful dermatology interview and examination. We explore the downstream effects of trisomy of chromosome 21, in particular on the immune system, and how these insights may enhance our pathophysiologic understanding of their cutaneous conditions.

Darier sign in a teenager with primary cutaneous marginal zone lymphoma.

Primary cutaneous marginal zone lymphoma (PCMZL) is a low-grade B-cell lymphoma that arises in the skin. An adolescent male presented with dermal nodules on the arms, legs, and back with a positive Darier sign, ultimately diagnosed as PCMZL. The nodules demonstrated a partial response to doxycycline in the setting of prior Lyme disease followed by a complete response to rituximab.

Impact of congenital cutaneous hemangiomas on newborn care in the United States.

Little is known regarding the characteristics of newborns with congenital cutaneous hemangioma (CH) and the burden of CH on newborn care. The objective of this study is to describe the burden of CH on newborn inpatient stays in the United States. Specific aims include characterizing newborns with CH, assessing factors predictive of CH and procedures performed during hospitalization, determining characteristics associated with increased cost of care and length of stay in newborns with CH, and investigating trends in prevalence, length of stay, and cost of care. This is a nationally representative retrospective cohort study (National Inpatient Sample, 2009-2015). Sociodemographic factors associated with CH and risk factors for increased cost of care/length of stay were evaluated using weighted multivariable regression models. Overall prevalence of CH is 17.0 per 10,000 newborns. Cost of care and length of stay for newborns with CH are increasing over time. Controlling for all covariates, white (aOR 1.69), female (aOR 1.52) newborns from higher income families (aOR 1.44) were more likely to be born with CH (p < 0.001). Newborns with CH who were premature (aOR 3.88), underwent more procedures (aOR 8.81), and born in urban teaching hospitals (aOR 2.66) had the greatest cost of care (p < 0.001). Premature (aOR 3.74) newborns with CH in urban teaching hospitals (aOR 1.31) had the longest hospital stays (p < 0.001). The burden of CH in newborns is substantial and increasing over time. Understanding contributors to costly hospital stays is critical in developing evidence-based guidelines to reduce the growing impact of CH on newborn care.

Primary livedoid vasculopathy associated with mononeuritis multiplex.

A 40-year-old woman presented with painful ulcerations on the bilateral lower extremities. A biopsy confirmed the diagnosis of livedoid vasculopathy (LV). She was treated initially with aspirin and pentoxifylline, and with the addition of dipyridamole she has had no recurrence of her ulcerations to date. Despite this positive response to treatment she reported numbness and paresthesias in her legs. Nerve conduction studies confirmed a diagnosis of mononeuritis multiplex. This case highlights mononeuritis multiplex as a rarely described complication of LV, and suggests that early recognition of symptoms and a multidisciplinary approach are necessary for optimal management of this condition.

Dermatologic conditions in Down syndrome: A single-center retrospective chart review.

BACKGROUND: Current literature addressing dermatologic conditions associated with Down syndrome is limited, with emphasis on rare skin conditions and lack of consensus on the incidence of more common disorders. OBJECTIVE: We sought to evaluate dermatologic conditions in patients with Down syndrome diagnosed and managed by dermatologists. METHODS: This was a retrospective analysis of 101 pediatric and adult patients with Down syndrome seen by the University of Massachusetts Dermatology Department between 2008 and 2018. RESULTS: Folliculitis was the most common diagnosis overall (30.7%), followed by seborrheic dermatitis (26.7%) and hidradenitis suppurativa (22.8%). Eczematous dermatitis, alopecia areata, and xerosis were the most common diagnoses observed in children aged 0-12 years; hidradenitis suppurativa, folliculitis, and seborrheic dermatitis in adolescents aged 13-17 years; and folliculitis, seborrheic dermatitis, and xerosis in adults 18 years and older. Other notable diagnoses present overall included onychomycosis (9.9%) and psoriasis (8.9%). Malignant cutaneous tumors were present in two patients, specifically basal cell carcinoma and malignant melanoma in situ. LIMITATIONS: This was a retrospective, single-institution study. CONCLUSION: Dermatologic conditions in patients with Down syndrome vary by age but are most often adnexal and eczematous disorders. Trisomy of chromosome 21 and the resulting downstream effects, specifically on the immune system, may account for these findings.

A Comparison of Psoriasis Severity in Pediatric Patients Treated With Methotrexate vs Biologic Agents.

Importance: Few studies have compared the use of methotrexate and biologics, the most commonly used systemic medications for treatment of moderate to severe psoriasis in children. Objective: To assess the real-world, 6-month reduction in psoriasis severity and long-term drug survival (rate and duration of adherence to a specific drug) of methotrexate vs biologics in plaque psoriasis in children. Design, Setting, and Participants: A retrospective medical records review was conducted at 20 European and North American centers. Treatment response was based on site-reported Psoriasis Area and Severity Index (PASI) and/or Physician Global Assessment (PGA) scores at baseline and within the first 6 months of treatment. Participants included all 234 consecutively seen children with moderate to severe psoriasis who received at least 3 months of methotrexate or biologics from December 1, 1990, to September 16, 2014, with sufficient data for analysis. Data analysis was performed from December 14, 2015, to September 1, 2016. Main Outcomes and Measures: PASI, with a range from 0 to 72 (highest score indicating severe psoriasis), and/or PGA, with a scale of 0 (clear), 1 (minimal), 2 (mild), 3 (moderate), 4 (severe), and 5 (very severe). Results: Of 234 pediatric patients (103 boys [44.0%]; 131 girls [56.0%]) treated with methotrexate and/or biologics, 163 patients (69.7%) exclusively received methotrexate, 47 patients (20.1%) exclusively received biologics, and 24 children (10.2%) received methotrexate and biologics sequentially. Of the latter cohort, 23 children were treated initially with methotrexate. Mean (SD) age at initiation was 11.6 (3.7) years for methotrexate and 13.3 (2.9) years for biologics (73.2% for etanercept) (P = .002). Among patients evaluated by a scoring method at 6-month follow-up, 75% or greater improvement in PASI (PASI75) was achieved in 12 of 30 patients (40.0%) receiving methotrexate and 20 of 28 patients (71.4%) receiving biologics, and PGA was clear/almost clear (PGA 0/1) in 41 of 115 patients (35.6%) receiving methotrexate and 18 of 37 patients (48.6%) receiving biologics. Achieving PASI75 and/or PGA 0/1 between baseline and 6 months was more likely with biologics than methotrexate (PASI75: odds ratio [OR], 4.56; 95% CI, 2.02-10.27; P < .001; and PGA 0/1: OR, 2.00; 95% CI, 0.98-4.00; P = .06). Decreased mean PASI and PGA scores were associated with biologics more than with methotrexate (PASI effect, -3.13; 95% CI, -4.33 to -1.94; P < .001; and PGA effect, -0.31; 95% CI, -0.56 to -0.06; P = .02). After 1, 3, and 5 years of use, overall drug survival rates for methotrexate were 77.5%, 50.3%, and 35.9%, and for biologics, the rates were 83.4%, 64.3%, and 57.1%, respectively. Biologics were associated with a better confounder-corrected drug survival than methotrexate (hazard ratio [HR], 2.23; 95% CI, 1.21-4.10; P = .01). Discontinuation owing to lack of response was comparable (HR, 1.64; 95% CI, 0.80-3.36; P = .18). Conclusions and Relevance: Methotrexate and biologics appear to be associated with improvement in pediatric psoriasis, although biologics seem to be associated with greater reduction in psoriasis severity scores and higher drug survival rates than methotrexate in the real-world setting. Additional studies directly comparing these medications should be performed for confirmation.

Pityrosporum folliculitis: A retrospective review of 110 cases.

BACKGROUND: Pityrosporum folliculitis is an under-recognized eruption of the face and upper portion of the trunk that may be confused with, or occur simultaneously with, acne vulgaris. OBJECTIVE: We sought to characterize risk factors for Pityrosporum folliculitis, its clinical presentation, and its response to treatment. METHODS: A retrospective chart review was performed on all patients age 0 to 21 years seen at our facility from 2010 to 2015 with Pityrosporum folliculitis confirmed by a potassium hydroxide preparation. RESULTS: Of 110 qualifying patients, more than 75% had acne that had recently been treated with antibiotics, and when recorded, 65% reported pruritus. Clinical examination demonstrated numerous 1- to 2-mm monomorphic papules and pustules that were typically on the forehead extending into the hairline and on the upper portion of the back. The most common treatment was ketoconazole shampoo, which led to improvement or resolution in most cases. Some patients required oral azole antifungals. LIMITATIONS: This study was retrospective and relied on providers describing and interpreting the clinical findings and potassium hydroxide preparations. No standard grading system was used. CONCLUSION: Unlike classic acne vulgaris, Pityrosporum folliculitis was more common after antibiotic use. It presented as fine monomorphic, pruritic papules and pustules along the hairline and on the upper portion of the back, and it improved with topical or oral azole antifungal therapy.

Safety of Systemic Agents for the Treatment of Pediatric Psoriasis.

Importance: Use of systemic therapies for moderate to severe psoriasis in children is increasing, but comparative data on their use and toxicities are limited. Objective: To assess patterns of use and relative risks of systemic agents for moderate to severe psoriasis in children. Design, Setting, and Participants: A retrospective review was conducted at 20 centers in North America and Europe, and included all consecutive children with moderate to severe psoriasis who used systemic medications or phototherapy for at least 3 months from December 1, 1990, to September 16, 2014. Main Outcomes and Measures: The minimal core data set included age, sex, severity of psoriasis, systemic interventions, monitoring, adverse events (AEs), and reason for discontinuation. Results: For 390 children (203 girls and 187 boys; mean [SD] age at diagnosis, 8.4 [3.7] years) with psoriasis who used 1 or more systemic medications, the mean interval between diagnosis and starting systemic therapy was 3.0 years. Methotrexate was used by 270 patients (69.2%), biologic agents (primarily etanercept) by 106 (27.2%), acitretin by 57 (14.6%), cyclosporine by 30 (7.7%), fumaric acid esters by 19 (4.9%), and more than 1 medication was used by 73 (18.7%). Of 270 children taking methotrexate, 130 (48.1%) reported 1 or more AEs associated with methotrexate, primarily gastrointestinal (67 [24.8%]). Folic acid 6 days per week (odds ratio, 0.16; 95% CI, 0.06-0.41; P < .001) or 7 days per week (OR, 0.21; 95% CI, 0.08-0.58; P = .003) protected against gastrointestinal AEs more than once-weekly folic acid, regardless of the total weekly dosage. Methotrexate-associated hepatic transaminase elevations were associated with obesity (35 of 270 patients [13.0%]), but a folic acid regimen was not. Injection site reactions occurred in 20 of 106 patients (18.9%) treated with tumor necrosis factor inhibitors, but did not lead to discontinuation of treatment. Having 1 or more AEs related to medication, gastrointestinal AE, laboratory abnormality, or AE leading to discontinuation of the drug was more likely with methotrexate than tumor necrosis factor inhibitors, but having 1 or more infections related to medication (predominantly upper airway) was less likely. Six patients developed a serious treatment-related AE (methotrexate, 3; fumaric acid esters, 2; and adalimumab, 1), but methotrexate and biologic agents were taken for a mean duration that was 2-fold greater than the mean duration for cyclosporine or fumaric acid esters. No patient developed tuberculosis or a malignant neoplasm. Conclusions and Relevance: Medication-related AEs occur less often with tumor necrosis factor inhibitors than with methotrexate. Folic acid administration 6 or 7 times per week protected more against methotrexate-induced gastrointestinal AEs than did weekly administration. A prospective registry is needed to track the long-term risks of systemic agents for pediatric psoriasis.