Eosinophilic pustule smear in the diagnosis of pediatric post-transplant eosinophilic folliculitis.

Hematologic-associated eosinophilic pustular folliculitis is a subtype of eosinophilic pustular folliculitis (EPF) which develops in patients with underlying hematological malignancies after treatment with chemotherapy, bone marrow transplant (BMT), or stem cell transplant (SCT). Few cases of hematological-associated EPF have been reported in pediatric patients. Skin biopsy is considered the gold standard for diagnosis. We describe a case in which Wright staining of a pustule smear for eosinophils provided data to rapidly support a clinical diagnosis of hematologic-associated EPF.

An infant with LUMBAR syndrome complicated by severe perineal ulceration managed with diverting colostomy.

Infantile hemangiomas are the most common childhood vascular lesions. LUMBAR syndrome (lower body hemangioma, urogenital abnormalities/ulceration, myelopathy, bony deformities, anorectal malformations/arterial anomalies, and rectal anomalies) warrants special treatment considerations. Here we describe a case of an infant with LUMBAR syndrome who presented with severe perineal ulceration refractory to standard medical therapy and was managed with a temporary diverting sigmoid colostomy. This case demonstrates that adjunctive surgical management can be considered in infants with aggressive perineal wounds refractory to standard medical therapy.

Green nail syndrome complicated by pseudomonal keratitis in a teenager with artificial nails.

Green nail syndrome (GNS) is a pseudomonal nail infection that presents with characteristic green nail discoloration. It typically affects patients with preexisting nail conditions or chronic exposure to wet environments but can also be seen with local trauma. Our patient presented with a pseudomonal corneal ulcer of the left eye and was incidentally found to have GNS, which developed after home artificial nail application. This unusual case of extensive pediatric GNS illustrates a rare and serious infectious complication of prolonged artificial nails.

Clinical characteristics of infantile hemangiomas with aggressive, persistent, and destructive ulceration.

BACKGROUND/OBJECTIVES: Ulceration is a common complication of infantile hemangioma (IH). Severe, persistent ulceration occurs in a minority of patients. This study aims to characterize the clinical features of IH with aggressive ulceration (AU). METHODS: Multicenter retrospective study of clinical features of IH with AU. RESULTS: Thirty-five patients with AU were identified and included in the study. The majority of AU occurred in segmental IH (23/35, 65%). Segmental IH with AU were large (≥10 cm2 ; 16/23, 69%, p < .001) with a thin (<3 mm) superficial component (16/23, 69%, p < .001). Localized IH with AU had a thick (>3 mm) superficial component (11/12, 92%, p < .001). All diaper area IH with AU (9/35) were segmental with thin superficial component (100%, p = .02). IH with AU in the head/neck (10/35) were more commonly localized (67%) and mixed (62.5%), while segmental, thick superficial morphology was more common on trunk (9/35) and upper extremities (7/35). CONCLUSIONS: IH resulting in AU differ in clinical features by anatomic site. Those in the diaper area are nearly always segmental with thin superficial component, whereas other sites tend to be localized, mixed, with thick superficial component. These distinct phenotypes may prove useful in the clinical setting for physicians to identify patterns of IH ulceration with increased risk of aggressive, persistent ulceration.

Cutaneous reactions in children treated with MEK inhibitors, BRAF inhibitors, or combination therapy: A multicenter study.

BACKGROUND: Treatment with BRAF inhibitors (BRAFI) and MEK inhibitors (MEKI) causes cutaneous reactions in children, limiting dosing or resulting in treatment cessation. The spectrum and severity of these reactions is not defined. OBJECTIVE: To determine the frequency and spectrum of cutaneous reactions in children receiving BRAFI and MEKI and their effects on continued therapy. METHODS: A multicenter, retrospective study was conducted at 11 clinical sites in the United States and Canada enrolling 99 children treated with BRAFI and/or MEKI for any indication from January 1, 2012, to January 1, 2018. RESULTS: All children in this study had a cutaneous reaction; most had multiple, with a mean per patient of 3.5 reactions on BRAFI, 3.7 on MEKI, and 3.4 on combination BRAFI/MEKI. Three patients discontinued treatment because of a cutaneous reaction. Treatment was altered in 27% of patients on BRAFI, 39.5% on MEKI, and 33% on combination therapy. The cutaneous reactions most likely to alter treatment were dermatitis, panniculitis, and keratosis pilaris-like reactions for BRAFI and dermatitis, acneiform eruptions, and paronychia for MEKI. CONCLUSIONS: Cutaneous reactions are common in children receiving BRAFI and MEKI, and many result in alterations or interruptions in oncologic therapy. Implementing preventative strategies at the start of therapy may minimize cutaneous reactions.

Body site distribution of pediatric-onset morphea and association with extracutaneous manifestations.

BACKGROUND: The distribution of pediatric-onset morphea and site-based likelihood for extracutaneous complications has not been well characterized. OBJECTIVE: To characterize the lesional distribution of pediatric-onset morphea and to determine the sites with the highest association of extracutaneous manifestations. METHODS: A retrospective cross-sectional study was performed. Using clinical photographs, morphea lesions were mapped onto body diagrams using customized software. RESULTS: A total of 823 patients with 2522 lesions were included. Lesions were more frequent on the superior (vs inferior) anterior aspect of the head and extensor (vs flexor) extremities. Linear morphea lesions were more likely on the head and neck, whereas plaque and generalized morphea lesions were more likely on the trunk. Musculoskeletal complications were more likely with lesions on the extensor (vs flexor) extremity (odds ratio [OR], 2.0; 95% confidence interval [CI], 1.2-3.4), whereas neurologic manifestations were more likely with lesions on the anterior (vs posterior) (OR, 2.8; 95% CI, 1.7-4.6) and superior (vs inferior) aspect of the head (OR, 2.3; 95% CI, 1.6-3.4). LIMITATIONS: Retrospective nature and the inclusion of only patients with clinical photographs. CONCLUSION: The distribution of pediatric-onset morphea is not random and varies with body site and within individual body sites. The risk stratification of extracutaneous manifestations by body site may inform decisions about screening for extracutaneous manifestations, although prospective studies are needed.

Successful treatment of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome with tofacitinib.

Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome is a rare autoinflammatory disorder. Cutaneous manifestations of CANDLE syndrome include characteristic recurring violaceous annular plaques comprised of an immature dermal mononuclear cell infiltrate. In CANDLE syndrome, deleterious genetic mutations inhibit proteasome-immunoproteasome function, resulting in cellular accumulation of ubiquitinated waste proteins that activate type I interferon signaling to drive inflammation. We describe a report of successful treatment of a 12-year-old girl with CANDLE syndrome with tofacitinib.

Multidisciplinary management of a previously unreported presentation of severe aplasia cutis congenita.

Aplasia cutis congenita (ACC) is characterized by the complete or partial absence of skin at birth, with 85% of cases of ACC involving the scalp vertex. The etiology of ACC is unclear and appears to be multifactorial. We present the case of a 3-month-old boy who presented with a diagnosis of non-scalp ACC affecting approximately 80% of his total body surface area at birth. This case adds to the literature due to the patient's survival beyond the first day of life and his unique and severe distribution of defects, which led to respiratory compromise and required multidisciplinary management.

A retrospective multicenter study of fatal pediatric melanoma.

BACKGROUND: Pediatric melanoma is rare and diagnostically challenging. OBJECTIVE: To characterize clinical and histopathologic features of fatal pediatric melanomas. METHODS: Multicenter retrospective study of fatal melanoma cases in patients younger than 20 years diagnosed between 1994 and 2017. RESULTS: Of 38 cases of fatal pediatric melanoma identified, 57% presented in white patients and 19% in Hispanic patients. The average age at diagnosis was 12.7 years (range, 0.0-19.9 y), and the average age at death was 15.6 years (range, 1.2-26.2 y). Among cases with known identifiable subtypes, 50% were nodular (8/16), 31% were superficial spreading (5/16), and 19% were spitzoid melanoma (3/16). One fourth (10/38) of melanomas arose in association with congenital melanocytic nevi. LIMITATIONS: Retrospective nature, cohort size, and potential referral bias. CONCLUSIONS: Pediatric melanoma can be fatal in diverse clinical presentations, including a striking prevalence of Hispanic patients compared to adult disease, and with a range of clinical subtypes, although no fatal cases of spitzoid melanoma were diagnosed during childhood.

Clinical and histologic presentation of pediatric reactive granulomatous dermatitis.

OBJECTIVE: To characterize the clinical and histologic presentation of reactive granulomatous dermatitis (RGD) in the pediatric population. METHODS: In this multicenter retrospective chart review, 7 pediatric patients with biopsy-proven RGD were identified. Photographs, histology reports, and clinical course were reviewed to discover patterns in demographics, comorbid conditions, autoimmune sequelae, drug exposures, infections, morphology, and histologic features. RESULTS: Overall, 7 patients were included and analyzed. Most were female and Hispanic. All presented with a similar dermatologic phenotype previously described in the adult literature including macular erythema and annular, pink to violaceous, edematous papules and plaques, often involving proximal extremities and extensor joints. All biopsies demonstrated variable collagen alteration and a perivascular interstitial infiltrate of histiocytes with or without mucin. Neutrophils or karyorrhexic debris were present in 4/7 of the biopsies, and eosinophils were occasionally seen (2/7 cases). In all cases, RGD was associated with active SLE or led to a new diagnosis, and initiation of systemic treatment improved cutaneous disease. CONCLUSIONS: Pediatric RGD was more common in female patients and ethnic minorities, and strongly associated with SLE. Clinical and histologic presentations were consistent across all cases with only minor variations, suggesting that recognition and confirmation might be expedited by familiarity with these dominant patterns. Diagnosis of RGD in pediatric patients should prompt screening for SLE.

Infant skin care: updates and recommendations.

PURPOSE OF REVIEW: Neonatal skin acclimates rapidly to dry, aerobic conditions at birth and skin function gradually matures throughout infancy. Gentle skin care practices support the ongoing development and function of newborn skin. This article reviews research updates and current skin care recommendations for full-term infants, premature infants, and infants born with severe cutaneous manifestations of genetic skin disorders. RECENT FINDINGS: Although safe early bathing of full-term infants with environmental controls is possible, delaying the first newborn bath for 12-24 h of life offers benefits of increased parental bonding and breastfeeding success. Swaddled bathing every 4 days is an effective bathing strategy for premature neonates. Among infants with a family history of atopic dermatitis, regular application of bland skin moisturizers reduces their risk of developing the disease. For newborns with erosive or blistering genetic skin conditions, use of specialized wound dressings and emollients promotes wound healing and helps limit skin damage. Environmental control with humidified incubators helps prevent life-threatening hypernatremic dehydration among babies born with collodion membranes; however, affected infants can tolerate breaks outside of the humidifier to promote parent-infant bonding. SUMMARY: This article reviews infant skin care recommendations relevant to pediatric practice. Research to further optimize newborn skin care is ongoing, particularly, for the special populations of premature neonates and infants born with severe skin disease.

Malignant melanoma incidence among children and adolescents in Texas and SEER 13, 1995-2013.

BACKGROUND: Epidemiological knowledge and predictors of melanoma among children and adolescents in multiethnic populations are limited. PROCEDURE: Using data from the Texas Cancer Registry (TCR) and the Surveillance, Epidemiology, and End Results (SEER) 13 database, we identified incident melanoma cases diagnosed at 0-20 years old during 1995-2013 in Texas and the United States, respectively. Using negative binomial regression, associations between demographic factors and melanoma incidence rates (IR) were evaluated by calculating incidence rate ratios (IRR) and 95% confidence intervals (CI). Annual percent change in IRs was assessed with joinpoint regression. RESULTS: Overall, the melanoma IR was 4.16 (TCR, n = 634) and 4.84 (SEER, n = 1260) per million. Females, adolescents, non-Hispanic (NH) whites, and Hispanics had higher IRs compared with other groups (P < 0.05). In adjusted analyses, Hispanics had a higher incidence of melanoma than NH non-whites (Texas IRR = 2.17; 95% CI, 1.30-3.61; SEER IRR = 2.88; 95% CI, 1.97-4.21). In Texas, NH whites with melanoma were more likely to live in low poverty areas, whereas the opposite trend was observed in Hispanics. Melanoma IRs increased throughout 1995-2004 followed by an average annual decrease of 7.6% (95% CI, -12.6%, -2.2%) in Texas and 6.0% (95% CI, -8.5%, -3.4%) in SEER during 2005-2013 (P < 0.05). However, these decreasing trends were not observed among Hispanics or those <10 years old. CONCLUSION: Although the overall melanoma IR in children and adolescents appears to be decreasing, this trend is not evident among Hispanics and young children, implicating the need for further research investigating the etiologies and risk factors in these groups.

Classic pseudoxanthoma elasticum in a girl with sickle cell disease.

A pseudoxanthoma elasticum (PXE)-like phenotype develops in a subset of patients with inherited hemoglobinopathies. Although PXE tissue changes are thought to develop in the absence of ABCC6 mutations in patients with beta-thalassemia, ABCC6 mutations have not been well evaluated among sickle cell disease patients with PXE-like disease. To our knowledge, we describe the first patient with sickle cell disease, PXE skin findings, and two confirmed pathogenic ABCC6 mutations. This case suggests that ABCC6 testing is warranted for sickle cell disease patients with the PXE-like phenotype and that the pathogenesis of PXE manifestations in beta-thalassemia and sickle cell disease may differ.

Allergic contact dermatitis related to homemade slime: a case and review of the literature.

Slime has become extremely popular as a children's toy in recent years and is typically made with various household substances. Although reports of slime causing skin irritation are not uncommon, case reports of slime-induced allergic contact dermatitis have only recently surfaced. We present a case of a child with hand dermatitis, history of exposure to slime, and positive allergen patch testing to two ingredients found in slime. The case underscores the need for clinicians to be aware of slime as a possible cause of allergic contact dermatitis in children. Given the trend of newly-reported cases, we briefly review the current literature to date.

Bumps in the Road: Panniculitis in Children and Adolescents Treated with Vemurafenib.

Vemurafenib is increasingly being used to treat nonmelanoma tumors that are positive for the BRAF V600E mutation. We report three children who presented with panniculitis induced by vemurafenib while undergoing treatment for central nervous system tumors and review the literature.