translation and validation into brazilian portuguese of a quality of life instrument for...

P7699Subcutaneous fat necrosis of the newborn and hypercalcemia: A casereport

Ignacio Valenzuela Salas, Hospital Universitario Virgen de las Nieves, Granada,Spain; Andrea Fern�andez Miralbell, Hospital Universitario Virgen de las Nieves,Granada, Spain; Carmen Mart�ınez Peinado, Hospital Universitario Virgen de lasNieves, Granada, Spain; Cristina Garrido Colmenero, Hospital UniversitarioVirgen de las Nieves, Granada, Spain; Eliseo Mart�ınez Garc�ıa, HospitalUniversitario Virgen de las Nieves, Granada, Spain; Jes�us Tercedor S�anchez,Hospital Universitario Virgen de las Nieves, Granada, Spain; Jos�e AneirosFern�andez, Hospital Universitario Virgen de las Nieves, Granada, Spain

Introduction: Subcutaneous fat necrosis (SCFN) is a rare, benign, self-limiteddisorder that can affect apparently healthy infants. It is sometimes associated withhypercalcemia. We report the case of a neonate with subcutaneous fat necrosisassociated with uncomplicated hypercalcemia.

Case report: A baby girl was born by cesarean section at a gestational age of 41weeksbecause of fetal distress with meconium-stained amniotic fluid. The mother hadgestational diabetes. At 12 days of age she developed skin lesions on back andshoulders. Physical examination revealed an erythematous, indurated, well definedplaque located in the back and 2 smaller lesions on both shoulders. A skin biopsywas performed, showing lobular panniculitis with focal necrosis and a mixedinflammatory infiltrate with foreign body giant cells, findings consistent with SCFNthe newborn. Blood test showed an albumin-corrected calcium of 12.2 mg/dL(normal, 8.5-10.5 mg/dL). Markers of renal function and levels of phosphorus andparathyroid hormone were normal. Skin lesions regressed until total resolution 2weeks after its appearance. Calcium levels remained high in subsequent blood testsand finally returned to normal after 1 year of life. During follow-up, the patient hadno symptoms associated with hypercalcemia or required treatment beyondadequate hydration and restriction of vitamin D.

Discussion: SCFN of the newborn is a rare, benign, self-limited panniculitis, whichusually occurs in term infants during the first weeks of life. It has been associatedwith meconium aspiration, hypothermia, hypoxemia, and cesarean, although theexact etiology remains uncertain. It is characterized by $ 1 erythematous topurplish, indurated plaques or subcutaneous nodules, the most frequent on cheeks,back, buttocks, arms, and thighs. Hypercalcemia is an infrequent association ofunknown etiology that has been related to the overproduction of vitamin D bymacrophageswithin the granulomas of fat necrosis. Differential diagnosis of SCFN ofthe newborn should include bacterial cellulitis, erysipelas, and sclerema neo-natorum. The prognosis is excellent, with resolution of skin lesions withoutsequelae over a period of weeks to months. Hypercalcemia may have a delayedstart and persist for months. Treatment is usually conservative, with hydration andrestriction of vitamin D, but sometimes need a more aggressive treatment, with theuse of glucocorticoids, furosemide, or bisphosphonates.

MAY 201

cial support: None identified.

P7892Systemic congenital Langerhans cell histiocytosis mimicking diffuseneonatal hemangiomatosis

Bel�en Rubio-Gonz�alez, MD, Hospital Universitario 12 de Octubre, Madrid, Spain;Beatriz Garc�ıa-Bracamonte, MD, Hospital Univeristario 12 de octubre, Madrid,Spain; Francisco Vnaclocha-Sebasti�an, MD, Hospital Universitario 12 de Octubre,Madrid, Spain; Jos�e Luis Rodr�ıguez-Peralto, MD, PhD, Hospital Universitario 12 deOctubre, Madrid, Spain; Lara Angulo-Mart�ınez, Hospital Universitario 12 deOctubre, Madrid, Spain; Mar�ıa Garrido-Ruiz, MD, Hospital Universitario 12 deOctubre, Madrid, Spain; Pablo Luis Ortiz-Romero, MD, PhD, Hospital 12 deOctubre, Madrid, Spain

Largerhans cell histiocytosis (LCH) is a rare condition defined by an aberrantproliferation of Langerhans cell which is characterized by S100 and CD1a positive.There are several diseases included as LCH depending of clinical presentation,histologic findings, and systemic involvement as LetterereSive disease, Hand-Schuller-Christian disease, congenital self-healing reticulohistiocytosis(HashimotoePritzker), and eosinophilic granuloma. We report a 26-day-old girlwas born at 40 weeks’ gestation to a healthy 33-year-old woman after an uncom-plicated pregnancy. At 2 to 3 days after birth, the infant started with vascular lesionslooking in abdominal area and involvement of upper and lower extremities and oralmucosa. Dermoscopic examination showed large vascular gaps in skin lesions.Systemic involvement with lymphadenopathy, hepatosplenomegaly, weight loss,diarrhea, anemia, thrombocytopenia, and disturbance of liver enzymes assays wasnoted. A presumptive diagnosis of diffuse neonatal hemagiomatosis was made, and askin biopsy of an abdominal lesion was reported as Langerhans cell histiocytosis(LCH) with S100 and CD1 positive. With the diagnosis of LCH with systemicinvolvement, the patient started treatment with vinblastine and prednisone withmarked improvement. Our case is unique in that the lesions mimicked diffuseneonatal hemangiomatosis clinically. This fact has been described in patients withcongenital self-healing reticulohistiocitosis (CSRH) but not in patients with neonataland systemic involvement (LetterereSive disease). Thus it is important to includeLCH in the differential diagnosis of infantile hemangiomas or diffuse hemangioma-tosis and making a correct diagnosis to beginning a treatment as soon as possible.Infants born with cutaneous lesions of LCH lacking organ involvement also shouldhave a long-term follow-up for possible recurrence.

cial support: None identified.

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P7673Tolerance assessment of cosmetic products dedicated to baby’s skin:Innovative approaches using stinging test and infant epidermis model

Caroline Baudouin, Laboratoires Expanscience, Epernon, France; Clarence deBelilovsky, Labortoires Expanscience, Epernon, France; Franck Menu,Laboratoires Expanscience, Epernon, France; Nad�ege Lachmann, LaboratoiresExpanscience, Epernon, France; Philippe Msika, Laboratoires Expanscience,Epernon, France; St�ephanie Br�edif, Laboratoires Expanscience, Epernon, France

Introduction: The skin evolution process continues after birth until the first years oflife with an impact on barrier organization and quality. We have developed specificguidelines on formulation and safety evaluation of products dedicated to childrenunder the age of 3 years, including a specific stinging test and the use of our modelsof infants’ epidermis.

Modified stinging test: Cosmetics’ sensory irritation potentials are independent ofobjective irritation. We developed a modified stinging test to increase thechallenging conditions and applied it to screen different preformulations of thesame baby product (A and B) which obtained similar objective irritation scores.Modified test has increased ambient temperature and relative humidity and earliermeasures. Fifteen stinger women were included in a 2-phase single blind study.Perceived degrees of stinging were recorded at T0, 1, 2.5, and 5 minutes. Clinicalrelevance of Global Score was: \10 very slight, 11-24 slight to moderate, [25strong.

Results: Global Scores were respectively 4 and 11. Therefore, stinging propensitywas considered as ‘‘very slight’’ for preformulation A which was further developed.

Age-specific infant epidermis-patented model: During in vitro studies, we havemodeled age-specific infants and children epidermis and characterized theirgenomic signature. We used 1 month-aged reconstructed epidermis to assess thetolerance of cosmetic baby products and we applied a validated ‘‘irritation skin’’protocol for the read-out parameters. After 16 h of applications with 2 products,tolerance was evaluated by analyzing 3 parameters: epidermis viability assessmentby MTT test; IL-8 measurement into the supernatants by ELISA assay; and histologyanalysis by hematoxylineeosin coloration of the paraffin-embedded tissue.

Results: The treatment of 1-month-aged reconstructed epidermis with the babyproducts did not induce any signs of toxicity, revealing their good tolerance.

Conclusion: In order to respect baby’s skin, it is important to use specific skin careproducts which have been formulated but also assessed under specific conditions.We developed 2 tolerance tools adapted to baby skin. Modified stinging test isroutinely added to our tolerance pack and a characterization of ‘‘very slight stinger’’is mandatory before final formulation and use-tests may be initiated. The patentedinfant epidermis models are now included in the safety evaluation process of all ourbaby cosmetic products.

cial support: None identified.

P7965Translation and validation into Brazilian Portuguese of a quality of lifeinstrument for epidermolysis bullosa patients (QOLEB)

Tania Cestari, MD, PhD, University of Rio Grande do Sul School of Medicine,Hospital de Clinicas de Porto Alegre-Brazil, Porto Alegre, Brazil; Clarissa Prati,MD, MS, University of Rio Grande do Sul School of Medicine, Hospital de Clinicasde Porto Alegre-Brazil, Porto Alegre, Brazil; Dedee Murrell, MD, PhD, Universityof New South Wales School of Medicine, St George Hospital Sydney-Australia,Sidney, Australia; Doris Menegon, RN, MS, University of Rio Grande do Sul Schoolof Medicine, Hospital de Clinicas de Porto Alegre-Brazil, Porto Alegre, Brazil;Maria Cecilia Machado, MD, PhD, University of S~ao Paulo School of MedicineHospital das Clinicas de S~ao Paulo-Brazil, S~ao Paulo, Brazil; Zilda Prado deOliveira, MD, PhD, University of S~ao Paulo School of Medicine Hospital dasClinicas de S~ao Paulo-Brazil, S~ao Paulo, Brazil

The Quality of Life Evaluation in Epidermolysis Bullosa (QOLEB) questionnaire wasdeveloped for English-speaking individuals. The aims of this study were to translatethe QoLEB to Brazilian Portuguese, culturally adapt it to our population, and to verifyits reliability and validity. The study followed the steps proposed by the WHO forQoL questionnaires: translation, evaluation by a panel of experts and EB patients,back translation, second evaluation by the original authors, and final linguistic andcultural adaptation. All subjects (58) were clinically evaluated and their QoLassessed by both the QOLEB and the Dermatology Life Quality Index (DLQI) or theChildren Dermatology Life Quality Index (CDLQI) instruments. Translation andcultural/linguistic adaptationwas conducted by the conjoinedwork of 10 patients, 3translators, and the English- and Portuguese-speaking authors. The population wascomposed of 40 children (average age, 8.8) and 18 adults. The average QOLEB for EBsimplexwas 9.6 (SD¼ 6.6) and 12.7 (SD¼ 8.7) for dystrophic EB (no difference: P¼.26), even when stratified for adults and children. The QOLEB-PB showed highinternal consistency (Cronbach alfa, 0.88) and high testeretest reliability (intraclasscorrelation coefficient, 0.70) confirming the internal consistency and reproduc-ibility of the Portuguese version. There was a significant correlation betweenQOLEB scores and CDLQI (rs ¼ 0.807; P\.001) or DLQI (rs ¼ 0.707; P\.003)scores. EB impacts patients’ and their families’ lives, strongly correlating with thedisease severity. The Brazilian-Portuguese version of QOLEB is validated for ourpopulation, and could be recommended as a measure to assess the impact of thedisease on the patients’ QoL, allowing the application and use of Brazilian data oninternational studies.

cial support: None identified.

J AM ACAD DERMATOL AB149