Infantile hemangiomas (IHs) are the most common tumors of childhood. Unlike other tumors, they have the unique ability to involute after proliferation, often leading primary care providers to assume they will resolve without intervention or consequence. Unfortunately, a subset of IHs rapidly develop complications, resulting in pain, functional impairment, or permanent disfigurement. As a result, the primary clinician has the task of determining which lesions require early consultation with a specialist. Although several recent reviews have been published, this clinical report is the first based on input from individuals representing the many specialties involved in the treatment of IH. Its purpose is to update the pediatric community regarding recent discoveries in IH pathogenesis, treatment, and clinical associations and to provide a basis for clinical decision-making in the management of IH.

OBJECTIVE: To characterize the atypical cutaneous presentations in the coxsackievirus A6 (CVA6)–associated North American enterovirus outbreak of 2011–2012. METHODS: We performed a retrospective case series of pediatric patients who presented with atypical cases of hand, foot, and mouth disease (HFMD) from July 2011 to June 2012 at 7 academic pediatric dermatology centers. Patients were included if they tested positive for CVA6 or if they met clinical criteria for atypical HFMD (an enanthem or exanthem characteristic of HFMD with unusual morphology or extent of cutaneous findings). We collected demographic, epidemiologic, and clinical data including history of skin conditions, morphology and extent of exanthem, systemic symptoms, and diagnostic test results. RESULTS: Eighty patients were included in this study (median age 1.5 years, range 4 months–16 years). Seventeen patients were CVA6-positive, and 63 met clinical inclusion criteria. Ninety-nine percent of patients exhibited a vesiculobullous and erosive eruption; 61% of patients had rash involving >10% body surface area. The exanthem had a perioral, extremity, and truncal distribution in addition to involving classic HFMD areas such as palms, soles, and buttocks. In 55% of patients, the eruption was accentuated in areas of eczematous dermatitis, termed “eczema coxsackium.” Other morphologies included Gianotti-Crosti–like (37%), petechial/purpuric (17%) eruptions, and delayed onychomadesis and palm and sole desquamation. There were no patients with serious systemic complications. CONCLUSIONS: The CVA6-associated enterovirus outbreak was responsible for an exanthem potentially more widespread, severe, and varied than classic HFMD that could be confused with bullous impetigo, eczema herpeticum, vasculitis, and primary immunobullous disease.

Many subjects with CARD14 mutations display characteristics of both psoriasis and PRP. We propose the term CARD14-associated papulosquamous eruption to describe this spectrum of disease. Subjects with clinical features suggestive of CAPE should undergo CARD14 sequencing and may benefit from treatment with ustekinumab.

IMPORTANCE Topical corticosteroid (TCS) phobia refers to the negative feelings and beliefs related to TCSs experienced by patients and patients' caregivers. This phenomenon may be a major contributing factor in treatment failure in patients with atopic dermatitis, yet it has been sparsely described in the literature.OBJECTIVE To systematically assess the nomenclature, prevalence, origins, and effect on treatment adherence of TCS phobia in atopic dermatitis.EVIDENCE REVIEW A literature search was conducted using specific eligibility criteria across electronic databases, including Ovid (MEDLINE, EMBASE), PubMed, and Web of Science, for articles published from January 1, 1946, to October 31, 2016. Included articles must have assessed TCS phobia in patients with atopic dermatitis or their caregivers. Quality ratings of studies were based on a modified version of the Oxford Centre for Evidence-Based Medicine quality rating scheme for individual studies.FINDINGS Of the 490 articles identified by literature search, 16 met the eligibility criteria. All studies were cross-sectional. Topical corticosteroid phobia prevalence ranged from 21.0% (95% CI, 15.8%-26.2%) to 83.7% (95% CI, 81.9%-85.5%). There was significant variation in how phobia was defined, ranging from concern to irrational fear. Questionnaires used to assess for TCS phobia included 1 to 69 questions. In the 2 studies that compared nonadherence between a phobia group and a nonphobia group, patients in both phobia groups were found to have a significantly higher rate of nonadherence (49.4% vs 14.1% and 29.3% vs 9.8%). The sources from which patients were receiving information about corticosteroids included physicians, friends and relatives, broadcast media, print media, and the internet.CONCLUSIONS AND RELEVANCE Features of TCS phobia are commonly reported by patients across cultures and may be associated with a higher rate of nonadherence. Patients with TCS phobia and the sources from which patients are receiving information about corticosteroids may be targetable for intervention to increase adherence to treatment regimens. The nomenclature and assessment methods for TCS phobia used in studies, however, lack standardization, precluding quantitative comparison and extrapolation of data. Additional research, using standardized definitions and methods of assessment, is needed to better characterize this phenomenon and evaluate the efficacy of potential interventions.

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Vascular tumors are among the most common neoplasms in infants and children; 5%-10% of newborns present with or develop lesions within the first 3 months of life. Most are benign infantile hemangiomas that typically regress by 5 years of age; other vascular tumors include congenital tufted angiomas (TAs), kaposiform hemangioendotheliomas (KHEs), and childhood lobular capillary hemangiomas (LCHs). Some of these lesions can become locally invasive and unresponsive to pharmacologic intervention, leading to significant complications. Recent investigation has revealed that activating mutations in HRAS, KRAS, NRAS, GNAQ, and GNA11 can cause certain types of rare childhood vascular tumors, and we have now identified causal recurrent somatic activating mutations in GNA14 by whole-exome and targeted sequencing. We found somatic activating GNA14 c.614A>T (p.Gln205Leu) mutations in one KHE, one TA, and one LCH and a GNA11 c.547C>T (p.Arg183Cys) mutation in two LCH lesions. We examined mutation pathobiology via expression of mutant GNA14 or GNA11 in primary human endothelial cells and melanocytes. GNA14 and GNA11 mutations induced changes in cellular morphology and rendered cells growth-factor independent by upregulating the MAPK pathway. Our findings identify GNA14 mutations as a cause of childhood vascular tumors, offer insight into mechanisms of oncogenic transformation by mutations affecting Gaq family members, and identify potential targets for therapeutic intervention.

Background: Hidradenitis suppurativa (HS) and pyoderma gangrenosum (PG) are both rare inflammatory skin conditions that are associated with systemic inflammatory diseases. We performed a retrospective medical chart review of patients with an overlap of HS and PG.Observations: We identified 11 cases of PG lesions presenting in patients with HS. Ten of the patients were women, and 9 were obese. All the patients developed HS lesions first, a median of 2.5 years (range, 0-15 years) preceding the appearance of PG lesions. All patients required multiple therapeutic agents because their diseases were often poorly responsive to standard therapies. Two patients received tumor necrosis factor inhibitors;

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