Lyme disease as a cause of acropapular dermatitis of childhood.

Acropapular dermatitis of childhood is a symmetric self-limited papulovesicular exanthem that classically occurs on the cheeks, extensor extremities, and buttocks in young children. The eruption of acropapular dermatitis of childhood represents a reaction to a variety of infections usually of viral origin. We present a child with typical findings of acropapular dermatitis of childhood whose serologic workup revealed an acute Lyme infection.

Methicillin-resistant Staphylococcus aureus colonization in children with atopic dermatitis.

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.

Acne arising in an epidermal nevus.

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.

Methicillin-resistant Staphylococcus aureus skin abscesses in a pediatric patient with atopic dermatitis: a case report.

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.

Two novel TP63 mutations associated with the ankyloblepharon, ectodermal defects, and cleft lip and palate syndrome: a skin fragility phenotype.

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.

Rice nightmare: Kwashiorkor in 2 Philadelphia-area infants fed Rice Dream beverage.

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.

The clonal nature of pityriasis lichenoides.

BACKGROUND: Pityriasis lichenoides et varioliformis acuta (PLEVA) and pityriasis lichenoides chronica (PLC) are benign lymphocytic infiltrates of the skin that classically present as either a recurrent papulonecrotic eruption (PLEVA) or a persistent, scaling, papular eruption (PLC). Observations of both types of lesions present on individual patients have led to speculation that both entities are related. Previous studies evaluating the DNA of biopsy specimens from patients with PLEVA and PLC revealed clonal T-cell receptor beta gene rearrangements. OBJECTIVE: To analyze and compare the T-cell populations between lesions of PLEVA and PLC. DESIGN: Retrospective and prospective analysis of patient tissue samples, classified by histologic analysis. Extracted DNA from 13 skin biopsy specimens with the diagnosis of PLC and 14 skin biopsy specimens with the diagnosis of PLEVA was analyzed by polymerase chain reaction/denaturing gradient gel electrophoresis (PCR/DGGE). SETTING: Molecular diagnostic laboratory at an academic medical center. PATIENTS: Twenty-seven tissue samples were obtained from patients with a histologic diagnosis of PLEVA or PLC. These samples were analyzed by PCR/DGGE. MAIN OUTCOME MEASURE: The presence or absence of T-cell receptor gene rearrangements on PCR/DGGE analysis corresponding to a clonal population of T cells. RESULTS: Of 14 PLEVA specimens, 8 (57%) demonstrated monoclonal T-cell receptor gene rearrangements; 1 (8%) of 13 PLC specimens showed a gene rearrangement (P =.008, Fisher exact test). CONCLUSIONS: Our results demonstrate the polyclonal nature of the lymphocytic infiltrate found in almost all of the PLC specimens, which contrasts with the monoclonal nature found in most of the PLEVA specimens. These differences may represent different stages of the clinical evolution of a single entity that results from varying host immune responses to pathogenic factors. Specifically, we propose that PLEVA is a benign clonal T-cell disorder in which the clone arises from a subset of T cells in lesions of PLC. The host immune response to this clone determines the clinical and histologic findings in PLEVA.

The safety and efficacy of pimecrolimus, 1%, cream for the treatment of Netherton syndrome: results from an exploratory study.

BACKGROUND: Impaired skin integrity in patients with Netherton syndrome (NS) results in significant systemic absorption of topically applied medications. Some have advocated the administration of pimecrolimus, 1%, topical cream for the treatment of patients with NS. Insufficient data exist with regard to its safety, systemic absorption, and efficacy. OBSERVATIONS: An exploratory study was conducted involving 3 children with NS who received twice-daily application of pimecrolimus, 1%, cream over 18 months. There were no notable abnormalities in hematologic or chemistry profiles. Blood levels of pimecrolimus ranged from 0.625 to 7.08 ng/mL, with peak levels reached during the first month in all 3 patients. Dramatic reductions were observed in the Netherton Area and Severity Assessment, Eczema Area and Severity Index, Investigator Global Evaluation of Disease, and pruritus scores compared with baseline levels. CONCLUSIONS: Use of pimecrolimus, 1%, cream was well tolerated and demonstrated marked improvements in nearly all of the parameters evaluated. Patients treated with pimecrolimus responded rapidly, within the first month of treatment, and improvement persisted throughout the study period. In adult patients receiving oral pimecrolimus, blood levels as high as 54 ng/mL for 3 months have not shown clinically significant immunosuppression. Absorption of pimecrolimus, 1%, cream was detectable, but levels were much lower than expected even when applied to 50% of total body surface area. Larger studies are warranted to determine the safety and efficacy of pimecrolimus, 1%, cream in the treatment of NS. TRIAL REGISTRATION: clinicaltrials.gov Identifier: NCT00208026.

"Urticaria multiforme": a case series and review of acute annular urticarial hypersensitivity syndromes in children.

Acute annular urticaria is a common and benign cutaneous hypersensitivity reaction seen in children that manifests with characteristic annular, arcuate, and polycyclic urticarial lesions in association with acral edema. It is mistaken most often for erythema multiforme and, occasionally, for a serum-sickness-like reaction. Although these 3 entities may present in a similar manner, specific clinical features help to distinguish them, and it is important for the clinician to be able to differentiate among them. We present herein a series of 18 patients who were given a diagnosis of acute annular urticaria and review the clinical distinctions between acute annular urticaria, serum-sickness-like reactions, and erythema multiforme. Because of the frequency of its clinical confusion with erythema multiforme, we propose the term "urticaria multiforme" as a more apt description to highlight the distinctive clinical features of this urticaria variant.

Congenital infantile fibrosarcoma: a masquerader of ulcerated hemangioma.

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.

Neonatal cutaneous fungal infections.

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.

Streptococcal intertrigo: an underrecognized condition in children.

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.

Necrolytic acral erythema: response to combination therapy with interferon and ribavirin.

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.

Nonpseudomonal ecthyma gangrenosum.

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.