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P8232 A case of epidermal nevus syndrome Anabella Pascucci, MD, UC Davis School of Medicine, Department of Dermatology, Sacramento, CA, United States; Nasim Fazel, MD, DDS, UC Davis School of Medicine, Department of Dermatology, Sacramento, CA, United States Introduction: Epidermal nevus syndromes encompass a wide range of disorders. These syndromes consist of an epidermal nevus associated with underlying organ involvement. There may be defects in the CNS, ocular, skeletal, renal, or cardiovascular systems. We describe a case of a newborn male with a linear epidermal nevus associated with neurologic and ocular abnormalities. Case report: A newborn male was born at 40 weeks and 3 days via emergent C- section because of failure to progress. The mother had prenatal care and the pregnancy was uncomplicated. The baby’s mother is 17 years old and had a miscarriage at age 16. There is no known family history of genetic abnormalities. The baby was noted on initial examination to have a salmon/yellow velvety fairly well demarcated plaque with associated alopecia on the vertex scalp that extended linearly down the right lateral frontal scalp and ended on the right medial eyebrow. He also had salmon/yellow firm papules on the right upper eyelid with an associated right eye corneal opacity. Ophthalmology evaluation showed severe right-sided anterior segment dysgenesis. Further work-up was significant for a negative newborn screen and negative chromosomal microarray analysis. Skeletal survey was negative. Renal ultrasound showed bilateral mild grade I hydronephrosis. Brain MRI showed nonobstructive culpocephaly with abnormalities of gray matter migration and cortical development with leptomeningeal angiomatosis of the right hemisphere. Discussion: Epidermal nevus syndrome is a term used to describe the association of an epidermal nevus with underlying systemic abnormalities. It is likely a catch all term that includes many disorders. The main well defined syndromes are Schimmelpenning syndrome, phacomatosis pigmentokeratotica, nevus comedoni- cus syndrome, angora hair nevus syndrome, Becker nevus syndrome, Proteus syndrome, type 2 segmental Cowden disease, fibroblast growth factor receptor 3 epidermal nevus syndrome, and CHILD syndrome. The male newborn in our case does not seem to fit the definition of any the previously described syndromes, but clearly has a related epidermal nevus syndrome. The clinical findings of a linear epidermal nevus following the lines of Blaschko on the right scalp and face with associated right-sided brain anomalies and right eye abnormalities suggest a genetic developmental defect. Commercial support: None identified. P8085 Access to pediatric dermatology in California: A specialty in severe shortage Alexander L. Fogel, Stanford University School of Medicine, Stanford, CA, United States; Joyce Teng, MD, PhD, Stanford University School of Medicine, Department of Dermatology, Division of Pediatric Dermatology, Stanford, CA, United States Pediatric patients are estimated to comprise [27% of patients seen in dermatologic practices and many pediatric skin diseases are treated by nondermatologists. While research indicates that children are dermatologicly underserved, the availability of pediatric dermatologic care has not been assessed. This study uses a demographic analysis and a survey of physician practice patterns to describe pediatric dermatologic care in California, home to more than 9.2 million children and the largest pediatric population in the US. Board-certified pediatric dermatologists (BCPDs) in California were identified using data from the American Board of Medical Specialties and medical license records, and population data were obtained from the US Census Bureau. 34 BCPDs actively practice in California, a state-wide average of 0.36 per 100,000 children (county level range, 0-3.81). 88% (30/34) of BCPDs are female. 41% (14/34) of BCPDs practice in academic settings, and 26% (9/34) practice at Kaiser Permemente. 38% (13/34) of BCPDs are affiliated with a nonprofit children’s hospital, and 50% (4/8) of such hospitals have an affiliated BCPD. To assess the availability of care, a telephone survey of all BCPD offices in California was conducted. Respondents consisted of physicians or office representatives. 59% (20/34) of BCPDs in California responded, representing physicians of all practice types. 95% (19/20) of BCPDs are accepting new patients, 85% (15/20) accept new patients without a referral, and the average wait-time for a new patient appointment is 22.5 days (range, 1-105). On average, BCPDs in California treat 78 patients per week (range, 4-155). Pediatric patients account for 67% of all BCPD office visits, and 30% (6/20) of BCPDs exclusively treat pediatric patients. 80% (16/20) of BCPDs accept some form of Medicaid, though restrictions often apply. These findings indicate that access to BCPDs is severely limited in California. Many areas are not served by a BCPD, and appointment wait times vary considerably by location. BCPDs treat 44% fewer patients per week than general dermatologists, and most do not exclusively treat pediatric patients. Also, many BCPDs are accessible only to patients with Kaiser insurance. Therefore, recommendations to improve access to BCPD-provided care are indicated. Future research will assess additional measures of access, including a study of telemedicine usage among BCPDs, and will produce recommendations to improve access. Commercial support: None identified. P8175 Alopecia areata in childhood Alexandra Katsarou, MD, PhD, A. Sygros University Hospital, Athens, Greece; Charitomeni Vavouli, A. Sygros University Hospital, Athens, Greece; George Kontochristopoulos, MD, A. Sygros University Hospital, Athens, Greece; Ioanna Verriou, A. Sygros University Hospital, Athens, Greece; Vasiliki Vosynioti, MD, A. Sygros University Hospital, Athens, Greece Introduction: Alopecia areata (AA) is characterized by round plaques hairless, nonscarring, that can be located in any hairy region. In pathogenesis are involved immunologic, genetic, environmental, and psychogenic factors. The AA is associ- ated with atopic dermatitis, autoimmune, endocrinologic diseases, and vitiligo. Children are affected frequently. Purpose: To study the epidemiologic, clinical, laboratory, and therapeutic data of AA in the outpatients of Pediatric Dermatology Department of ‘‘A. Sygros’’ University Hospital during a 6-year period. Methods: In total, 15,280 children were examined, with 335 AA cases identified (2.2%) from December 2005 to June 2012, aged from birth to 12 years. We studied the extent of the lesions, personal and family history of AA, the coexistence with other diseases, the treatment, and the disease course. Results: The 50.4% of the examinees were boys and 49.6% girls. Aged 0-4 years was 23.9%, 5-8 years was 43.9% and 9-12 years was 32.2%. Solitary plaque ( \25% of the surface of the scalp) was observed in 71.6%, multiple plaques (¼ 2) in 17.9% (¼ 50% of the surface of the scalp), and diffuse alopecia in 2.7%, ophiasis in 2.1%, total in 0.6% ( [50% of the surface of the scalp). Nail changes were observed in 8.1%. Personal history of AA had 8.4% and family 4.2%. A relationship with thyroid abnormalities was found in 12%, with atopic personal history 12.6% and family 3.15%. Vitiligo had 0.9%, psoriasis 0.3% and rheumatoid arthritis 0.6%. The 1.2% suffered from Down syndrome. In 8.4% of children were found anemia by iron deficiency. The total IgE was increased in 20.45%. Treatment consisted of topical (70.8%) and systemic corticosteroids (2.1%), minoxidil (1.2%), anthralin (0.6%), local calcineurin inhibitors (0.9%) and combinations of them (20.3%). The disease course was assessed in 3, 6, and 12 months. Regrowth was showed in 42.3% in the 3rd month, from the third month up to sixth month was added another 30% and up to the first year another 5.8%. Approximately 21.9% was not responded to the therapy. Relapses in the third month were 3.6%, in the sixth month 8.7%, and in the first year 14.5%. Conclusion: AA is a relatively common disease. The majority of children (71.6%) have mild disease (size \25%), the most common age of onset is 5 to 8 years that almost affects males and females equally. Thyroid testing is recommended. The course of the disease varies. The response to the treatment is difficult to estimate because of automatic regrowth. Commercial support: None identified. P7730 Analysis of pediatric atopic dermatitis: The relationship between disease progression and total IgE/percent positive immunocaps, year 2 experi- ence, combined results Courtney Tobin, MD, Saint Louis University School of Medicine Department of Dermatology, Saint Louis, MO, United States; Elaine Siegfried, MD, Cardinal Glennon Children’s Hospital, Saint Louis, MO, United States; Eric Armbrecht, PhD, Saint Louis University, Saint Louis, Missouri, United States Objective: To evaluate the predictive value of commercially available biomarkers for progressive extrinsic atopic dermatitis (AD): total IgE and % positivity of the Atopic March Immunocaps Panel. Methods: In this 3.6-year retrospective single center chart review, 74 children aged 9 months to 18 years with mild to severe AD were characterized as intrinsic versus extrinsic based on age-adjusted norms of total IgE. AD patients were included if they had 2 sets of total IgE and immunocaps panels and at least 2 visits to the dermatology clinic. Patients who failed to attend clinic visits within 3 months of the laboratory draws were excluded. Results: The average age was 7.0 yrs with a range of 9 months to 18 years, and the average interval to follow-up was 0.9 yr. AD was intrinsic in 18% (n ¼ 8) and extrinsic in 82% (n ¼ 37). Demographics and comorbities were similar among the intrinsic and extrinsic groups. At baseline, 69% (n ¼ 42) of the extrinsic AD patients had severe disease compared to 31% (n ¼ 4) of intrinsic patients (P ¼.019). Among the subset of severe extrinsic patients whose disease improved at follow-up, there was no significant baseline difference in total IgE (IU/mL) compared to those whose disease remained poorly controlled (9.235 vs. 7.467; P ¼ .66). However, total IgE decreased significantly in those patients whose disease became controlled but increased in poorly controlled patients (4,798 vs. 12,916; P ¼.01: a 4.437 decrease vs. an 5.449 increase; P ¼ .001). Total IgE decreases were similar among patients treated with topical therapy versus systemic therapy patients [absolute change of total IgE, -5,238.8 vs. -3,125.4; P ¼ .500]. Eczema herpeticum and failure to thrive occurred more frequently among the extrinsic poorly controlled group versus the controlled group [eczema herpeticum (n ¼ 7) 24% vs. (n ¼ 8) 62%; P ¼.04: failure to thrive, (n ¼ 1) 3% vs. (n ¼ 6) 46%; P ¼.002]. Limitations: The study was a retrospective chart review performed on a small sample size at a single center. No severity scoring system was used. Conclusions: This study supports the use of total serum IgE as a readily available laboratory test that may be a prognostic indicator of AD progression. It supported a relationship between poorly controlled extrinsic AD and progressively increasing total IgE and correlated a decrease in total IgE with disease control in extrinsic AD whether treated with systemic or topical therapy. Commercial support: None identified. AB142 JAM ACAD DERMATOL MAY 2014

A case of epidermal nevus syndrome

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Page 1: A case of epidermal nevus syndrome

P8232A case of epidermal nevus syndrome

Anabella Pascucci, MD, UC Davis School of Medicine, Department ofDermatology, Sacramento, CA, United States; Nasim Fazel, MD, DDS, UC DavisSchool of Medicine, Department of Dermatology, Sacramento, CA, United States

Introduction: Epidermal nevus syndromes encompass a wide range of disorders.These syndromes consist of an epidermal nevus associated with underlying organinvolvement. There may be defects in the CNS, ocular, skeletal, renal, orcardiovascular systems. We describe a case of a newborn male with a linearepidermal nevus associated with neurologic and ocular abnormalities.

Case report: A newborn male was born at 40 weeks and 3 days via emergent C-section because of failure to progress. The mother had prenatal care and thepregnancy was uncomplicated. The baby’s mother is 17 years old and had amiscarriage at age 16. There is no known family history of genetic abnormalities. Thebaby was noted on initial examination to have a salmon/yellow velvety fairly welldemarcated plaque with associated alopecia on the vertex scalp that extendedlinearly down the right lateral frontal scalp and ended on the right medial eyebrow.He also had salmon/yellow firm papules on the right upper eyelid with an associatedright eye corneal opacity. Ophthalmology evaluation showed severe right-sidedanterior segment dysgenesis. Further work-up was significant for a negativenewborn screen and negative chromosomal microarray analysis. Skeletal surveywas negative. Renal ultrasound showed bilateral mild grade I hydronephrosis. BrainMRI showed nonobstructive culpocephaly with abnormalities of gray mattermigration and cortical development with leptomeningeal angiomatosis of the righthemisphere.

Discussion: Epidermal nevus syndrome is a term used to describe the association ofan epidermal nevus with underlying systemic abnormalities. It is likely a catch allterm that includes many disorders. The main well defined syndromes areSchimmelpenning syndrome, phacomatosis pigmentokeratotica, nevus comedoni-cus syndrome, angora hair nevus syndrome, Becker nevus syndrome, Proteussyndrome, type 2 segmental Cowden disease, fibroblast growth factor receptor 3epidermal nevus syndrome, and CHILD syndrome. The male newborn in our casedoes not seem to fit the definition of any the previously described syndromes, butclearly has a related epidermal nevus syndrome. The clinical findings of a linearepidermal nevus following the lines of Blaschko on the right scalp and face withassociated right-sided brain anomalies and right eye abnormalities suggest a geneticdevelopmental defect.

AB142

cial support: None identified.

Commer

P8085Access to pediatric dermatology in California: A specialty in severeshortage

Alexander L. Fogel, Stanford University School of Medicine, Stanford, CA, UnitedStates; Joyce Teng, MD, PhD, Stanford University School of Medicine,Department of Dermatology, Division of Pediatric Dermatology, Stanford, CA,United States

Pediatric patients are estimated to comprise[27% of patients seen in dermatologicpractices and many pediatric skin diseases are treated by nondermatologists. Whileresearch indicates that children are dermatologicly underserved, the availability ofpediatric dermatologic care has not been assessed. This study uses a demographicanalysis and a survey of physician practice patterns to describe pediatricdermatologic care in California, home to more than 9.2 million children and thelargest pediatric population in the US. Board-certified pediatric dermatologists(BCPDs) in Californiawere identified using data from the American Board of MedicalSpecialties andmedical license records, and population data were obtained from theUS Census Bureau. 34 BCPDs actively practice in California, a state-wide average of0.36 per 100,000 children (county level range, 0-3.81). 88% (30/34) of BCPDs arefemale. 41% (14/34) of BCPDs practice in academic settings, and 26% (9/34)practice at Kaiser Permemente. 38% (13/34) of BCPDs are affiliated with a nonprofitchildren’s hospital, and 50% (4/8) of such hospitals have an affiliated BCPD. Toassess the availability of care, a telephone survey of all BCPD offices in Californiawasconducted. Respondents consisted of physicians or office representatives. 59%(20/34) of BCPDs in California responded, representing physicians of all practicetypes. 95% (19/20) of BCPDs are accepting new patients, 85% (15/20) accept newpatients without a referral, and the average wait-time for a new patient appointmentis 22.5 days (range, 1-105). On average, BCPDs in California treat 78 patients perweek (range, 4-155). Pediatric patients account for 67% of all BCPD office visits, and30% (6/20) of BCPDs exclusively treat pediatric patients. 80% (16/20) of BCPDsaccept some form of Medicaid, though restrictions often apply. These findingsindicate that access to BCPDs is severely limited in California. Many areas are notserved by a BCPD, and appointment wait times vary considerably by location.BCPDs treat 44% fewer patients per week than general dermatologists, and most donot exclusively treat pediatric patients. Also, many BCPDs are accessible only topatients with Kaiser insurance. Therefore, recommendations to improve access toBCPD-provided care are indicated. Future researchwill assess additional measures ofaccess, including a study of telemedicine usage among BCPDs, and will producerecommendations to improve access.

cial support: None identified.

Commer

J AM ACAD DERMATOL

P8175Alopecia areata in childhood

Alexandra Katsarou, MD, PhD, A. Sygros University Hospital, Athens, Greece;Charitomeni Vavouli, A. Sygros University Hospital, Athens, Greece; GeorgeKontochristopoulos, MD, A. Sygros University Hospital, Athens, Greece; IoannaVerriou, A. Sygros University Hospital, Athens, Greece; Vasiliki Vosynioti, MD, A.Sygros University Hospital, Athens, Greece

Introduction: Alopecia areata (AA) is characterized by round plaques hairless,nonscarring, that can be located in any hairy region. In pathogenesis are involvedimmunologic, genetic, environmental, and psychogenic factors. The AA is associ-ated with atopic dermatitis, autoimmune, endocrinologic diseases, and vitiligo.Children are affected frequently.

Purpose: To study the epidemiologic, clinical, laboratory, and therapeutic data of AAin the outpatients of Pediatric Dermatology Department of ‘‘A. Sygros’’ UniversityHospital during a 6-year period.

Methods: In total, 15,280 children were examined, with 335 AA cases identified(2.2%) from December 2005 to June 2012, aged from birth to 12 years. We studiedthe extent of the lesions, personal and family history of AA, the coexistence withother diseases, the treatment, and the disease course.

Results: The 50.4% of the examinees were boys and 49.6% girls. Aged 0-4 years was23.9%, 5-8 years was 43.9% and 9-12 years was 32.2%. Solitary plaque (\25% of thesurface of the scalp) was observed in 71.6%, multiple plaques (¼ 2) in 17.9% (¼ 50%of the surface of the scalp), and diffuse alopecia in 2.7%, ophiasis in 2.1%, total in0.6% ([50% of the surface of the scalp). Nail changes were observed in 8.1%.Personal history of AA had 8.4% and family 4.2%. A relationship with thyroidabnormalities was found in 12%, with atopic personal history 12.6% and family3.15%. Vitiligo had 0.9%, psoriasis 0.3% and rheumatoid arthritis 0.6%. The 1.2%suffered from Down syndrome. In 8.4% of children were found anemia by irondeficiency. The total IgE was increased in 20.45%. Treatment consisted of topical(70.8%) and systemic corticosteroids (2.1%), minoxidil (1.2%), anthralin (0.6%),local calcineurin inhibitors (0.9%) and combinations of them (20.3%). The diseasecourse was assessed in 3, 6, and 12 months. Regrowth was showed in 42.3% in the3rd month, from the third month up to sixth month was added another 30% and upto the first year another 5.8%. Approximately 21.9% was not responded to thetherapy. Relapses in the third month were 3.6%, in the sixth month 8.7%, and in thefirst year 14.5%.

Conclusion: AA is a relatively common disease. The majority of children (71.6%)have mild disease (size\25%), the most common age of onset is 5 to 8 years thatalmost affects males and females equally. Thyroid testing is recommended. Thecourse of the disease varies. The response to the treatment is difficult to estimatebecause of automatic regrowth.

cial support: None identified.

Commer

P7730Analysis of pediatric atopic dermatitis: The relationship between diseaseprogression and total IgE/percent positive immunocaps, year 2 experi-ence, combined results

Courtney Tobin, MD, Saint Louis University School of Medicine Department ofDermatology, Saint Louis, MO, United States; Elaine Siegfried, MD, CardinalGlennon Children’s Hospital, Saint Louis, MO, United States; Eric Armbrecht,PhD, Saint Louis University, Saint Louis, Missouri, United States

Objective: To evaluate the predictive value of commercially available biomarkers forprogressive extrinsic atopic dermatitis (AD): total IgE and % positivity of the AtopicMarch Immunocaps Panel.

Methods: In this 3.6-year retrospective single center chart review, 74 children aged 9months to 18 years with mild to severe AD were characterized as intrinsic versusextrinsic based on age-adjusted norms of total IgE. AD patients were included if theyhad 2 sets of total IgE and immunocaps panels and at least 2 visits to the dermatologyclinic. Patients who failed to attend clinic visits within 3 months of the laboratorydraws were excluded.

Results: The average age was 7.0 yrs with a range of 9 months to 18 years, and theaverage interval to follow-upwas 0.9 yr. ADwas intrinsic in 18% (n¼ 8) and extrinsicin 82% (n ¼ 37). Demographics and comorbities were similar among the intrinsicand extrinsic groups. At baseline, 69% (n ¼ 42) of the extrinsic AD patients hadsevere disease compared to 31% (n ¼ 4) of intrinsic patients (P ¼.019). Among thesubset of severe extrinsic patients whose disease improved at follow-up, there wasno significant baseline difference in total IgE (IU/mL) compared to those whosedisease remained poorly controlled (9.235 vs. 7.467; P ¼ .66). However, total IgEdecreased significantly in those patients whose disease became controlled butincreased in poorly controlled patients (4,798 vs. 12,916; P ¼ .01: a 4.437 decreasevs. an 5.449 increase; P ¼ .001). Total IgE decreases were similar among patientstreated with topical therapy versus systemic therapy patients [absolute change oftotal IgE, -5,238.8 vs. -3,125.4; P ¼ .500]. Eczema herpeticum and failure to thriveoccurred more frequently among the extrinsic poorly controlled group versus thecontrolled group [eczema herpeticum (n¼ 7) 24% vs. (n¼ 8) 62%; P¼.04: failure tothrive, (n ¼ 1) 3% vs. (n ¼ 6) 46%; P ¼ .002].

Limitations: The studywas a retrospective chart review performed on a small samplesize at a single center. No severity scoring system was used.

Conclusions: This study supports the use of total serum IgE as a readily availablelaboratory test that may be a prognostic indicator of AD progression. It supported arelationship between poorly controlled extrinsic AD and progressively increasingtotal IgE and correlated a decrease in total IgE with disease control in extrinsic ADwhether treated with systemic or topical therapy.

cial support: None identified.

Commer

MAY 2014