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Background: The pediatric dermatology workforce has not been systematically evaluated since recent changes in board certification requirements., Objective: To quantify and characterize the workforce of academic pediatric dermatologists and examine issues related to training, hiring, and retention., Methods: Dermatology chairpersons and residency directors in the United States and Canada completed a 30-question survey., Results: Eighty of 132 programs (61%) responded to the survey. More than two thirds of programs (56/80) employed a pediatric dermatologist, and 34 programs were recruiting a pediatric dermatologist. The number of residents that pursue careers in pediatric dermatology is significantly associated with the number of pediatric dermatologists on faculty at their institution., Limitations: Self-reported data, which may have been reflected by recall bias, and 61% response rate., Conclusions: At a majority of academic centers, the current pool of pediatric dermatology faculty is neither adequate to meet academic nor clinical demands. Methods to increase exposure to pediatric dermatology among medical students and residents must be sought.

Children with atopic dermatitis are more frequently colonized with Staphylococcus aureus than children without atopic dermatitis. However, little epidemiological data exist regarding the prevalence of methicillin-resistant S. aureus among children with atopic dermatitis. Recent studies have revealed an increasing prevalence of community-associated methicillin-resistant S. aureus among patients presenting to hospitals with serious bacterial infections, particularly those with cutaneous and soft tissue infections. As many atopic dermatitis patients are treated empirically with antibiotics for secondary skin infections, an understanding of the epidemiology of bacterial colonization and superinfection is essential for directing proper treatment in the atopic patient population. This study investigates the prevalence of risk factors for community-associated, methicillin-resistant S. aureus colonization among pediatric atopic dermatitis patients encountered at an academic pediatric dermatology clinic. An observational cross-sectional study was conducted at the Children's Hospital of Philadelphia in which 54 patients previously diagnosed with atopic dermatitis were enrolled. A detailed patient questionnaire, a complete cutaneous examination, and an evaluation of eczema severity according to the Eczema Area and Severity Index were completed at the time of enrollment. Bacterial cultures from the skin and nares were obtained to determine the frequency of colonization with either methicillin-sensitive S. aureus or methicillin-resistant S. aureus. Although most atopic dermatitis patients studied were colonized with S. aureus (43/54 [80%]), methicillin-resistant S. aureus was isolated from only seven atopic dermatitis patients (7/43 [16%]). Patients colonized with S. aureus were more likely to be male, to have been previously hospitalized, to have used a topical calcineurin inhibitor in combination with a topical steroid, and less likely to have used topical antibiotics. Bivariable analysis, however, revealed that only previous hospitalization was independently associated with an increased risk of methicillin-resistant S. aureus colonization. We observed that 80% of atopic dermatitis patients were colonized with S. aureus, and that of these patients, 16% of colonized patients were colonized with a methicillin-resistant strain. Methicillin-resistant S. aureus colonization was found to be significantly associated with previous hospitalization. Evidence also indicates that topical calcineurin inhibitors used in conjunction with topical steroids is associated with increased S. aureus colonization, while topical antibiotic use appears to decrease S. aureus colonization.

The management of acne vulgaris is a long-term process that must be individualized to each patient. Relevant factors influencing treatment include the age and gender of the patient, the severity and extent of disease, the efficacy and tolerability of prior interventions, and the degree of compliance with recommended therapies. The follow-up phase of acne management requires a framework for approaching treatment modification that may include concepts such as up-titration, add-on, or switching of available agents to improve efficacy and tolerability. Whenever oral antibiotics are used, abrupt discontinuation in favor of topical maintenance therapy rather than gradual tapering is recommended. Strategies for assessing and optimizing medical adherence are reviewed.

We report two cases of focal preauricular dermal dysplasia and review the available literature. Focal preauricular dermal dysplasia is a form of aplasia cutis congenita in which atrophic skin lesions occur in a stereotypical bilateral distribution in the preauricular region. Although focal preauricular dermal dysplasia and membranous cutis aplasia of the scalp share clinical similarities, focal preauricular dermal dysplasia represents a form of aplasia cutis congenita that is not typically associated with extracutaneous anomalies.

Background: Collodion phenotype is a term applied to the condition affecting a newborn involving a parchmentlike membrane covering the whole body surface (collodion membrane). This presentation is common to several different forms of autosomal recessive congenital ichthyoses, including nonbullous congenital ichthyosiform erythroderma (NCIE), lamellar ichthyosis (LI), and harlequin ichthyosis (HI). Recent years have seen considerable advances in our understanding of the molecular basis of autosomal recessive forms of congenital ichthyosis. Several genetic loci have been identified for LI and NCIE., Observations: We describe the clinical and molecular features of 2 cases of self-healing newborns of collodion phenotype developing mild NCIE. A dramatic improvement of the skin was observed in the first few weeks after birth in both cases. The molecular analysis of the ALOX12B gene demonstrated that both patients were compound heterozygous for previously unreported mutations., Conclusions: Both patients were compound heterozygous for novel ALOX12B mutations, underscoring the concept that mutations in at least 2 different genes, ALOX12B and TGM1, may result in this unusual clinical phenotype.

We present a child with new-onset nephrotic syndrome, acrodermatitis, low serum zinc levels and decreased serum alkaline phosphatase. A diagnosis of acquired zinc deficiency acrodermatitis was made. Oral zinc supplementation led to rapid clinical resolution. The etiology of zinc deficiency in nephrotic syndrome remains unknown.

Skin findings in childhood sarcoidosis vary greatly, but only a few occurrences have been published in which the histopathology has been characterized well. We describe a child with sarcoidosis in whom the cutaneous findings were atypical, resembling granuloma annulare. Histologic examination of these cutaneous lesions, however, revealed areas of sarcoid-like epithelioid cell granulomas, a palisading granulomatous process with features of granuloma annulare, as well as palisading neutrophilic and granulomatous dermatitis and interstitial granulomatous dermatitis. This underscores the variability of skin findings in childhood sarcoidosis--even within the same patient--and suggests that sarcoidosis should be considered in the differential diagnosis of children initially diagnosed with granulomatous skin lesions, such as granuloma annulare, palisading neutrophilic, and granulomatous dermatitis or interstitial granulomatous dermatitis, who demonstrate associated signs of systemic disease.

Epidermal nevi are benign congenital lesions that follow Blaschko lines. Epidermal nevi can demonstrate a variety of histologic patterns and contain hamartomatous hyperplasia of any epidermal or adnexal structure. We present the first reported instance of acne arising strictly within a preexisting keratinocytic epidermal nevus during puberty, which is a demonstration of genetic mosaicism in pilosebaceous units.

Hay-Wells/ankyloblepharon-ectodermal dysplasia-clefting syndrome is a rare autosomal dominant disorder characterized by ankyloblepharon, ectodermal dysplasia, and cleft lip and/or cleft palate. Mutations in the p63 gene recently have been shown to be etiologic in the majority of cases of ankyloblepharon-ectodermal dysplasia-clefting syndrome. To date, there have been no reports to document wound healing after cleft lip and/or palate repair in ankyloblepharon-ectodermal dysplasia-clefting patients. We describe two patients with ankyloblepharon-ectodermal dysplasia-clefting syndrome and provide a review of the literature. There have been no reported instances of wound healing complications in affected patients. Seventeen percent (3/18) of reported patients required revisions or repair of oronasal fistulae. Cleft lip and palate repair can be performed safely in patients with Hay-Wells syndrome.

Combined hepatocellular cholangiocarcinoma (cHCC-CC) is an uncommon histological variant of primary liver cancer. This combined type of tumour lesion follows an aggressive clinical course with an unfavourable prognosis. Although the role of orthotopic liver transplantation in the management for hepatocellular carcinoma is well defined, to date, there has been no report on its treatment value for cHCC-CC. We report our experience of orthotopic liver transplantation in three patients with cHCC-CC. One patient succumbed to distant metastases 16.5 months after liver transplantation whereas the other two patients continued to survive 35 and 25 months after transplantation, respectively.

Acne vulgaris is a nearly universal phenomenon among adolescents in the western world and continues to remain problematic for a significant proportion of adults. During adolescence, emotional and physical changes must be successfully integrated into the emerging sense of self, and skin disorders such as acne, which alter that self-image, may engender distressing feelings of embarrassment, shame, and disgust. While most patients eventually achieve spontaneous remission, approximately one quarter of teenagers will show evidence of permanent acne scarring by 18 years of age. This article reviews current information regarding the pathophysiology, clinical manifestations, differential diagnosis, and therapy of the adolescent patient who has acne, and emphasizes recent advances in acne management.

Recent trends indicate an increasing incidence of community-acquired methicillin-resistant Staphylococcus aureus (CAMRSA) in the general population, which is especially worrisome for clinicians caring for patients with atopic dermatitis (AD). Patients with AD are heavily colonized with S aureus and have impaired skin integrity and abnormal immune responses, which predisposes them to more invasive cutaneous infections (eg, cellulitis, furuncles, abscesses). In this report, we describe a child with severe AD who presented with CAMRSA skin abscesses. The presence of an atypical skin infection in patients with AD, particularly those unresponsive to conventional penicillinase-resistant penicillins and cephalosporins, should alert the clinician to the possibility of MRSA as the underlying etiology, and intervention should be directed accordingly.

Congenital infantile fibrosarcoma, a rare malignant tumor of childhood, may present as a highly vascularized mass that is clinically difficult to distinguish from a hemangioma. When ulcerated, significant hemorrhage, anemia, and thrombocytopenia may occur in children with these lesions. This report describes three infants with ulcerated congenital infantile fibrosarcomas of the hand. As appropriate medical and surgical management hinges on timely and appropriate diagnosis, we review the clinical manifestations of these lesions.

Objective: To analyze the effectiveness of methotrexate (MTX) for the therapy of pediatric localized scleroderma (LS)., Methods: A retrospective chart review was performed for 17 pediatric patients with LS who failed topical therapy and were subsequently treated with MTX (12.5-25 mg weekly) with or without oral corticosteroids. A structured followup telephone call to the families was used to assess patient satisfaction., Results: Skin findings improved in 16 of 17 patients with a median time to improvement of 2.25 and 2.0 months for MTX alone or in combination with corticosteroids. Only one patient had active lesions at the most recent followup visit. Fifteen of 17 families reported improvement in their child's lesions after beginning MTX. Twelve of 17 patients were treated with MTX and oral corticosteroids. There were no major adverse events., Conclusion: MTX appears to be a safe and effective therapy for pediatric LS.

Background: Ankyloblepharon, ectodermal defects, and cleft lip and palate (AEC) syndrome is a rare autosomal dominant disorder caused by mutations in the sterile alpha motif region of TP63, a homologue of the tumor suppressor TP53. Recent structure-function studies have identified complexities in the genotype-phenotype correlation of the p63 syndromes., Observations: We report 2 sporadic cases of AEC syndrome in infants. Both patients demonstrated skin erosions with prominent scalp involvement. Histologic studies demonstrated mild basal layer vacuolization and rare dyskeratotic keratinocytes, with evidence of both acantholysis and cytolysis at the blister edge. Immunohistochemistry using anti-p63 monoclonal antibody demonstrated basal epidermal nuclear staining in both healthy control and patient tissue samples. Ultrastructural studies showed focal disruption of anchoring fibrils near the blister edge of one patient and normal desmosomes, hemidesmosomes, and basement membrane zone in the nonblistered skin of the other patient. The DNA analysis of each patient revealed 2 novel missense mutations in the TP63 gene that resulted in L514S and R555P amino acid substitutions within the sterile alpha motif region of the p63 protein., Conclusions: We report 2 novel TP63 mutations resulting in AEC syndrome. The R555P mutation is the most carboxy-terminal of all the reported AEC missense mutations of p63. The presence of skin fragility, manifested as erosive skin lesions in body areas in addition to the scalp, is postulated to be an important diagnostic feature of AEC syndrome.

Hemangiomas of infancy are common in the general pediatric population, are usually easily diagnosed, and generally do not require treatment. However, a small but significant percentage of hemangiomas of infancy may develop complications, including infection or ulceration. In addition, hemangiomas located in some anatomic regions may be associated with other anomalies and therefore require more careful monitoring and earlier intervention to prevent permanent sequelae. This review focuses on distinguishing hemangiomas from vascular malformations and delineates the natural history of hemangiomas of infancy, with an emphasis on identifying those hemangiomas that require additional evaluation and closer follow-up. Current treatment modalities, including the use of systemic steroids and the pulsed-dye laser, are discussed. In addition, several conditions that often present with cutaneous hemangiomas are described, including PHACES syndrome and neonatal hemangiomatosis. Finally, an assessment is made of the current understanding of the biology of hemangioma proliferation and involution, including the role of endothelial growth factors and GLUT1, a new marker for hemangiomas of infancy., (Clin Pediatr. 2005;44:747-766.)

Cutaneous pigmentation is a hallmark of Addison disease. When present, the hyperpigmentation generally localizes to sun-exposed surfaces. This case highlights a less well-recognized cutaneous feature that is pathognomonic for the disease: oral mucous membrane hyperpigmentation. We describe this unique type of discoloration in detail and contrast it with other forms of oral pigmentation.

Purpose of Review: Cutaneous fungal infections are not uncommon in newborns and are seen in premature or otherwise immunocompromised neonates as well as in healthy full-term neonates. Healthy newborns can develop clinical manifestations as a result of infection with Candida species or as a result of skin colonization with Malassezia species; cutaneous infection with other fungal pathogens is rare. Immunocompromised and premature neonates, however, are susceptible to infection with opportunistic pathogens and are also at higher risk for invasive infection with common pathogens such as Candida. This review discusses the fungal species associated with cutaneous fungal infection in neonates, emphasizes the relevant clinical features, and also reviews the use of newer antifungal agents, including lipid-associated amphotericin B, voriconazole, and caspofungin., Recent Findings: Neonatal cutaneous infections with opportunistic fungal pathogens, including Aspergillus and the Zygomycetes, have been reported with increasing frequency as advances in neonatal care have improved the survival rate in very low birthweight neonates. Although these infections are frequently fatal, survival in some neonates has been reported with the use of aggressive surgical debridement and systemic antifungal therapy. Newer antifungal agents, including voriconazole and caspofungin, show promise in the treatment of potentially fatal fungal infections in neonates., Summary: Cutaneous fungal infections in neonates range from generally benign conditions such as congenital candidiasis and neonatal cephalic pustulosis to potentially fatal infections with opportunistic pathogens in very low birthweight or immunocompromised neonates. The prompt recognition and appropriate treatment of cutaneous fungal disease in neonates is critical to the prevention of adverse outcomes.

A 14-month-old girl and a 7-month-old boy each presented with a diffuse dermatitis, whole body edema, and hypoalbuminemia. The diets of both infants consisted almost entirely of Rice Dream, a rice-based, protein-poor beverage. Both infants were diagnosed with kwashiorkor, which resolved with protein supplementation. Clues from the physical examination, a diet history, appropriate laboratory examinations, and an index of suspicion are crucial in promptly diagnosing and treating infants with kwashiorkor. Manufacturers of rice beverages should appropriately warn parents about the dangers of using their products as infant nourishment.

Background: Aquagenic wrinkling of the palms (AWP) is a rare condition characterized by the rapid and transient formation of edematous whitish plaques on the palms on exposure to water (the so-called hand-in-the-bucket sign). The changes may be asymptomatic or accompanied by pruritic or burning sensations. First described in 1974 in patients with cystic fibrosis--and still primarily reported in these patients--this condition has been previously described only in females. Specific mutations in CFTR, the gene responsible for cystic fibrosis, have not been reported previously in patients with AWP., Observations: We describe 2 patients with AWP, both of whom are homozygous for the delta F508 mutation in CFTR: a 17-year-old boy--the first male reported to have this condition--who has had AWP for 3 years and a 13-year-old girl who has had AWP for 6 months., Conclusions: Cystic fibrosis should be considered in patients with AWP, and patients with cystic fibrosis should be asked about symptoms of this condition. Although the etiology of AWP is unknown, the association with cystic fibrosis, and with marasmus and cyclooxygenase-2 inhibitors, suggests that exposure of the skin to abnormally high concentrations of salt may play a role in its pathogenesis.

Most biopolymer drugs to date have been proteins. However, the ability to select nucleic acid binding species (aptamers) has led to the development of protein inhibitors and modulators that are small, readily synthesized nucleic acids. The techniques for optimizing, stabilizing, and delivering nucleic acid therapies are just beginning to be developed, but the same engineering flexibility that has so far allowed the generation of multiple, high affinity and specificity binding species appears to also apply to the methods for adapting nucleic acids to clinical applications. We review the selection and characterization of various aptamers and their applications to a variety of disease states, and then focus on the hurdles that must be overcome for the use of aptamers as both exogenously delivered drugs and as gene therapies.

Warts and molluscum contagiosum are common skin diseases in children and are usually self-limiting. The decision of whether to treat children with molluscum or warts should be individualized to the patient and his or her family. Considerations include how symptomatic the lesions are, the extent and duration of disease, the ability of the child and the parents to tolerate and comply with treatment recommendations, and any underlying medical conditions (Table, see page 219). Recurrences of molluscum contagiosum and especially warts are common, and realistic expectations regarding the potential for treatment failure and recurrence should be discussed with the child and his or her family prior to initiating any therapy. As pediatric practitioners, we all remain acutely aware of our patients' physical and psychological development and the potential for any intervention to influence this development. Although various treatment modalities now exist for the treatment of these viral diseases, any intervention should be balanced against these considerations.

Purpose of Review: Acne vulgaris is a common skin disorder among children and young adults that carries enormous financial and psychosocial impact. Contemporary therapies attempt to address factors underlying acne as a disorder of the pilosebaceous unit. These longstanding paradigms regarding pathogenesis and treatment continue to evolve in light of recent work on this ubiquitous disease., Recent Findings: This review focuses on new literature that has emerged regarding the biology of the folliculosebaceous unit, the identification of particular mediators responsible for inflammatory acne, the use of topical and systemic retinoids in acne therapy, and approaches to address the emergence of antibiotic-resistant Propionibacterium acnes strains. In addition, the use of several novel therapeutic avenues is discussed, including combination therapies, lipoxygenase inhibitors, and lasers., Summary: As the understanding of the factors that initiate and exacerbate acne vulgaris continues to increase, so does the diversity of therapeutic options. Rational use of available treatment options based on the type and severity of acne lesions is a key component of successful acne therapy and allows the physician who treats adolescents with acne to provide optimum care.

Ecthyma gangrenosum is a cutaneous infection associated most commonly with pseudomonal sepsis in the patient who is immunocompromised. We describe an 8-month-old girl with acute myelocytic leukemia who developed perineal ecthyma gangrenosum caused by Citrobacter freundii, a gram-negative pathogen that has been rarely associated with cutaneous disease. We also review the literature to categorize the range of pseudomonal and nonpseudomonal pathogens associated with ecthyma gangrenosum.

Necrolytic acral erythema is a papulosquamous and sometimes vesiculobullous eruption bearing clinical and histologic similarity to other necrolytic erythemas such as necrolytic migratory erythema, pseudoglucagonoma, and nutritional deficiency syndromes. Necrolytic acral erythema is distinguished by its association with hepatitis C infection and its predominantly acral distribution. We describe a pediatric patient with necrolytic acral erythema whose eruption resolved with hyperalimentation and combination interferon and ribavirin therapy, despite the persistence of detectable viral load and continued hepatic and renal insufficiency.

The presence of multiple café au lait spots (CALSs) has been well described and associated with several neurocutaneous and genetic syndromes including, most commonly, neurofibromatosis. However, scant literature exists regarding the clinical significance of the large solitary CALS. We describe 5 patients with congenital large solitary CALSs without associated abnormalities. Our cases and review of the literature suggest that large solitary CALSs are uncommon benign lesions that most likely are not associated with any neurocutaneous syndromes or developmental anomalies.

Group A beta-hemolytic streptococci have been implicated in a variety of common childhood cutaneous infections. Infants and young children may be particularly susceptible to a form of streptococcal intertrigo that has heretofore been underrecognized in this population. Manifesting as intense, fiery-red erythema and maceration in the intertriginous folds of the neck, axillae, or inguinal spaces, the condition is characterized by a distinctive foul odor and an absence of satellite lesions. Specific clinical features help differentiate this condition from its clinical mimics. Topical and oral antibiotic therapy with or without concomitant low-potency topical steroid application is generally curative.

Aquagenic palmoplantar keratoderma is an acquired condition characterized by burning and edema limited to the hands after brief immersion in water. The 3 patients described possess a striking similarity to those with transient reactive papulotranslucent acrokeratoderma. All 3 patients manifested the "hand-in-the-bucket" sign, having presented to a physician with a hand immersed in a bucket of water to more promptly demonstrate the physical findings. Aluminum chloride hexahydrate represents a potentially valuable therapeutic option for this unusual condition.

Bupivacaine is a commonly used local anesthetic in dental, ophthalmologic, and simple surgical procedures. Its current popularity derives from its potency and relatively long half-life. Widespread use of bupivacaine has resulted in sometimes severe adverse reactions when significant systemic absorption has occurred. This report documents a life-threatening event following use of bupivacaine, briefly reviews its neurotoxic and cardiotoxic effects, and raises questions about current management strategies when toxicity occurs.