An investigation of vascular anomalies centers' transition of care practices.

This study aims to examine transition of care (TOC) practices of multidisciplinary vascular anomalies centers (VACs). Thirty-seven of 71 VAC leaders to whom the survey was sent completed the questionnaire. TOC and transfer practices varied with only 16% of VACs having TOC programs. The most frequently cited barriers to developing a TOC program were lack of resources and difficulty finding expert adult providers.

Characteristics and complications of anogenital infantile hemangiomas: A multicenter retrospective analysis.

BACKGROUND: Infantile hemangiomas (IHs) of the anogenital region remain poorly characterized. OBJECTIVE: To examine the distribution, ulceration rate, and associated congenital anomalies of anogenital IHs. METHODS: Retrospective study at 8 tertiary referral centers. RESULTS: A total of 435 infants with an IH of the anogenital region were enrolled (of which, 319 [73%] were girls). Congenital anomalies were present in 6.4% (n = 28) of infants with an anogenital IH. Segmental or partial segmental anogenital IHs ulcerated in 72% (n = 99 of 138) of infants, whereas 45% (n = 133 of 297) of focal anogenital IHs experienced ulceration (P < .001). In a multivariable logistic regression analysis, segmental or partial segmental morphology (adjusted odds ratio [aOR], 2.70; 95% CI, 1.60-4.64), mixed type (aOR, 3.44; 95% CI, 2.01-6.07), and perianal (aOR, 3.01; 95% CI, 1.53-6.12) and buttocks location (aOR, 2.08; 95% CI, 1.17-3.76) had increased odds of ulceration. Segmental or partial segmental IHs of the genitalia were confined to distinct anatomic territories and were predominantly distributed unilaterally, with a linear demarcation at the perineal raphe. LIMITATIONS: Possible selection bias, given recruitment at tertiary referral centers. CONCLUSION: This study improves our understanding of high-risk features of anogenital IHs and demonstrates that genital segmental or partial segmental IHs develop within distinct anatomic territories.

Lumps and bumps: What not to miss.

The evaluation of pediatric patients with subcutaneous nodules remains a diagnostic challenge. Pediatric dermatologists are regularly confronted with patients who have a nonspecific nodule. Though most masses that require evaluation are ultimately benign, the possibility of a more aggressive process, including borderline or malignant neoplasms, underscores the pivotal role of the pediatric dermatologist in recognizing these lesions. The aim of this review is to provide an overview of lumps and bumps that are important to recognize to prevent delay in diagnosis or treatment of a serious underlying condition. Clinical clues that may lead the pediatric dermatologist to have a higher index of suspicion for more aggressive lesions are reviewed. Suggestions for evaluation and workup, as well as tips for the difficult to discern lesion, are proposed.

Acquired epidermodysplasia verruciformis (AEV) in three children after cardiac transplantation: A case series and review of the literature.

Acquired epidermodysplasia verruciformis (AEV) describes epidermodysplasia verruciformis developing in an immunocompromised host. There is limited information in the literature regarding AEV in the pediatric population; of the patients reported, most patients described had HIV, with only two reported cases of children who developed AEV post-transplantation. This case series describes three pediatric patients who developed AEV on immunosuppressant therapy following cardiac transplantation. We review risk factors, treatment options, and prognosis of AEV in the pediatric population.

Lymphadenopathy as a Prodrome for Systemic Lupus Erythematous.

BACKGROUND: Lupus lymphadenopahy (LL) has an estimated incidence of 1% at diagnosis. Here, we report a case of systemic lupus erythematous which presented with a prodrome of generalized lymphadenopathy and fevers. CASE PRESENTATION: A 41-year-old woman presented to the hospital with one month of fevers, chills, lymphadenopathy, abdominal pain, a bilateral upper extremity rash, and malaise. Physical exam was notable for tender, palpable posterior cervical lymph nodes that were mobile and about 1 cm in maximum diameter. After extensive infectious, hematologic, and autoimmune evaluations, a diagnosis of systemic lupus erythematous (SLE) was made and treatment with high-dose steroids and hydroxychloroquine which resulted in gradual improvement in symptoms. CONCLUSION: Systemic lupus erythematous can present with a subtle prodrome of generalized lymphadenopathy. It is important for medical professionals to consider SLE in the differential in a patient with diffuse lymphadenopathy.

An evaluation of the psychosocial impact of epilepsy on marriage in the United Kingdom.

OBJECTIVES: The primary objective of this study was to measure the psychosocial burden for persons with epilepsy (PWEs) and for their spouses and to compare and correlate this with the clinical burden of seizures. A secondary objective was to examine the presence of gender-specific differences in the perception of psychosocial burdens by both PWE and their spouses, as well as in the factors that may influence this perception. We also sought to delineate differences in perceived stigmatization if the onset of epilepsy was within matrimony or if seizure onset was prior to marriage. METHODS: A questionnaire was constructed from previously validated instruments to measure stigma and was administered to 50 PWE-spouse pairs. A copy was applied to the PWE, and another was administered separately to the spouse. The medical notes were scrutinized by a Consultant Neurologist to enable an assessment of seizure severity for each type of seizure that the PWE experienced. Pearson correlation significance was examined at 95% level of significance. RESULTS: Higher seizure severity over the month prior to data collection correlated with smaller reporting differences in psychosocial outcome between spouses and the PWE (p = 0.005), an effect that maintained significance when the period over which seizure severity was evaluated was extended to one year (p = 0.021). Regarding gender-specific differences, low mood over the month prior to administration of the questionnaire was associated with worse psychosocial scores in females only (p = 0.001). Significant impairment in driving was correlated with worse outcomes in males only (p = 0.008). Male spouses' judgment on the 'overall health' of their wife correlated to seizure severity (p = 0.003). However, the psychosocial scores reported by male spouses were inversely correlated to those of the PWE (p = 0.042). Finally, in PWE with seizure onset within marriage, a high degree of perceived stigmatization (p = 0.025) and low mood (p = 0.004) was correlated to worse psychosocial functioning. This group also tended to be more anxious when the PWE was experiencing severe seizures (p = 0.013). CONCLUSION: Although severe seizures in this sample of couples were correlated with a smaller discrepancy in perceived seizure burden, gender-specific differences in perception of epilepsy-related psychosocial burden exist. This is true for both PWE and their spouses. Irrespective of gender, onset of epilepsy within matrimony was correlated with higher levels of anxiety and stigma. These factors need to be considered during efforts to reduce epilepsy-related stigmatization, as well as in tailoring therapies that aim to support the spouse as well as the PWE.

The psychosocial impact of epilepsy on marriage: A narrative review.

There have been many studies exploring quality of life as well as the impact of epilepsy on the affected individual. However, epilepsy affects more than the patients themselves, and there seems to be a paucity of data regarding the impact of epilepsy beyond the person with epilepsy (PWE). In particular, it is uncertain what the impact of epilepsy on marriage may be. We therefore performed a narrative review to evaluate work measuring the psychosocial effect of epilepsy on marriage. We reviewed the literature on epilepsy and marriage by searching PubMed (Medline) and EMBASE and thoroughly examining relevant bibliographies. Forty-two papers were identified that addressed the issue of the psychosocial effect of epilepsy on marriage. The different approaches used to assess the impact of epilepsy on marriage can be broadly grouped into three categories: assessment of the social effect of living with epilepsy, which includes the marital prospects of PWEs and how changes in martial status associate with seizure frequency; assessment of quality of life (QOL) of PWEs; assessment of the association of social support with the disease burden of epilepsy. Within each of these approaches, different research methods have been employed including questionnaires, qualitative methods, and scales. The studies reviewed indicate that epilepsy has a severe impact on individuals and their families. While many quality-of-life surveys do comment on the marital status of the patient, there is little expansion beyond this. The impact that seizures may have on the partner of a patient with epilepsy is barely addressed. With increasing incidence of epilepsy in older populations, potential changes in the dynamic of a long-term marriage with the development of epilepsy in older age are not known. Similarly, the impact of marriage on concordance with medication or proceeding to, for example, surgical treatment for pharmacoresistant epilepsy has not been studied in detail. We suggest ways in which to address these aspects in order to better deliver holistic care to patients with epilepsy and their partners.