Clinical spectrum of capillary malformation-arteriovenous malformation syndrome presenting to a pediatric dermatology practice: a retrospective study.

Capillary malformation-arteriovenous malformation syndrome (CM-AVM) is an autosomal dominant disorder caused by RASA1 mutations. The prevalence and phenotypic spectrum are unknown. Evaluation of patients with multiple CMs is challenging because associated AVMs can be life threatening. The objective of this study was to describe the clinical characteristics of children presenting with features of CM-AVM to an academic pediatric dermatology practice. After institutional review board approval was received, a retrospective chart review was performed of patients presenting between 2009 and 2012 with features of CM-AVM. We report nine cases. Presenting symptoms ranged from extensive vascular stains and cardiac failure to CMs noted incidentally during routine skin examination. All demonstrated multiple CMs, two had Parkes Weber syndrome, and two had multiple infantile hemangiomas. Seven patients had family histories of multiple CMs; three had family histories of large, atypical CMs. Six had personal or family histories of AVMs. Genetic evaluation was recommended for all and was pursued by six families; four RASA1 mutations were identified, including one de novo. Consultations with neurology, cardiology, and orthopedics were recommended. Most patients (89%) have not required treatment to date. CM-AVM is an underrecognized condition with a wide clinical spectrum that often presents in childhood. Further evaluation may be indicated in patients with multiple CMs. This study is limited by its small and retrospective nature.

The "biker-glove" pattern of segmental infantile hemangiomas on the hands and feet.

BACKGROUND: Infantile hemangiomas (IH) on the extremities have not been systematically studied. OBJECTIVE: We sought to describe the clinical characteristics and distribution patterns of IH affecting acral surfaces and to explore the relationship among these patterns, limb development, and IH pathogenesis. METHODS: This was a retrospective multicenter cohort study. Photographic archives from 4 tertiary pediatric dermatology referral centers were searched for patients with IH larger than 1 cm and involving 1 or more digit. Hemangioma location, distribution, and morphologic subtype were recorded. Medical records were reviewed for demographic and clinical data. RESULTS: In all, 73 patients were identified. The most common IH pattern resembled that of a "biker glove" (73%), followed by localized IH on the distal digits (14%), segmental IH extending over the distal digits (8%), and intermediate patterns (5%). Overall, 63% of acral IH were segmental, 26% indeterminate, and 11% localized. Five patients had associated structural anomalies. Complications were noted in 33% of cases. LIMITATIONS: Limitations were retrospective study design; selection bias based on recall and photography; documentation and follow-up were not standardized across institutions; and treatment information may not reflect current approaches. CONCLUSION: Acral IH display specific patterns and are associated with a relatively high risk of ulceration.

Evaluation of wound care options in patients with recessive dystrophic epidermolysis bullosa: a costly necessity.

Recessive dystrophic epidermolysis bullosa (RDEB) is a genetic disorder in which mutations in collagen VII, the main component of the anchoring fibril, lead to skin fragility and to the development of acute and chronic wounds. Wound care and dressing changes are an important part of the daily lives of individuals with RDEB. Ideal wound care should improve wound healing, minimize pain, and improve quality of life. The objective of the current study was to review wound care options that might be used in a patient with RDEB and calculate the cost of these various options based on publicly available pricing of wound care products. There is a wide range of costs for wound care options in patients with RDEB. For example, a 1-day supply of dressing for a neonate boy with RDEB ranges from $10.64 for the least expensive option to $127.54 for the most expensive option. Wound care in patients with severe, generalized RDEB has not only a significant economic effect, but also directly affects quality of life in this patient population. Although randomized controlled trials evaluating different wound care products in patients with RDEB are lacking, small studies and expert opinion support the use of specialized nonadherent dressings that minimize skin trauma and promote wound healing. Until there is a cure, prospective studies are needed to assess pain, quality of life, and wound healing associated with the use of specialized wound care products for this life-altering condition.

Congenital cutaneous hemangioma causing cardiac failure: a case report and review of the literature.

We present a case of a large congenital hemangioma (CH) on the neck causing cardiac failure and thrombocytopenia in a female neonate. A trial of medical therapy with corticosteroids and propranolol was attempted, but the patient ultimately underwent definitive treatment with embolization and surgical resection with a positive outcome. A review of the English language literature revealed 16 previously reported cases of CHs complicated by congestive heart failure. This series supports known demographic features of CHs, including a lack of gender discrepancy and a predilection to affect the head and neck. These CHs are rarely diagnosed in utero; most patients present with a mass at birth. Cardiac failure is identified prenatally or in the first days of life. A mild to moderate thrombocytopenia and coagulopathy, which is likely transient and distinct from classic Kasabach-Merritt phenomenon, accompanies many of these cases. There is a 30% associated mortality rate. Both medical and interventional treatment modalities have been reported. Steroids are the most commonly used medication, but without any clear benefit. We hypothesize that, based on its possible mechanisms of action,propranolol may be a more effective treatment for CHs requiring treatment. As surgical intervention may be necessary, we recommend a multidisciplinary approach to treating patients with problematic CHs.

Management of Infectious Aspects of Atopic Dermatitis in Primary Care: A Resident Survey.

Atopic dermatitis (AD) is the most common skin disease encountered by pediatric primary care providers. To describe the knowledge, attitudes, and practices of primary care residents in the management of infectious aspects of pediatric AD, an anonymous web-based survey was offered to all residents in the pediatric and family medicine departments at 3 New York City hospitals. Eighty residents responded. Most (62%) reported seeing 5 to 14 patients with AD monthly. Twenty-seven percent reported obtaining cultures prior to prescribing oral antibiotics most of the time, while 8% reported doing so before giving topical antibiotics. Most respondents (60%) reported never/rarely recommending dilute bleach baths, and family medicine residents were significantly more likely to report never doing so (67% vs 16%, P < .001). Greater education on the use of cultures to guide treatment and potential benefits of dilute bleach baths is needed, especially given increasing antibiotic resistance.

Chlorhexidine gluconate­impregnated central access catheter dressings as a cause of erosive contact dermatitis: a report of 7 cases.

BACKGROUND: Chlorhexidine gluconate-impregnated dressings have become widely adopted as a means to reduce the risk for catheter-associated bloodstream infections. These dressings release antiseptic under occlusion onto the skin surrounding catheter insertion sites. Although chlorhexidine gluconate is a known cause of contact dermatitis, the phenotypic range of this adverse effect of chlorhexidine gluconate­impregnated dressings in critically ill patients has not been described. OBSERVATIONS: We report 7 cases of erosive irritant contact dermatitis due to chlorhexidine gluconate-impregnated transparent dressings. Six of these patients were children (age range, 4 months to 2 years); the adult was a critically ill 62-year-old man. Four patients were immunosuppressed after solid organ transplant and all were receiving blood pressure support at the time of this reaction. The insertion sites of femoral catheters were involved in all but 1 case; 3 catheter sites were involved in the adult patient. Results of extensive infectious workups were negative. All lesions resolved with discontinuation of the chlorhexidine gluconate-containing dressings, local wound care, and alternative antimicrobial dressings. CONCLUSIONS: Erosive contact dermatitis is an under-recognized complication of chlorhexidine gluconate-impregnated dressings. Health care providers should be aware of this risk, particularly in young children and immunosuppressed and/or critically ill patients, who may be more susceptible to the irritant effects of these dressings. When the dressings are used, patients should be monitored closely for skin breakdown.