bluuebunny/crossref_metadata_2025 huggingface dataset:
Necrobiosis Lipoidica Diabeticorum in Children and Adolescents: A Clue for Underlying Renal and Retinal Disease
Authors: Verrotti Atberto , Chiarelli Francesco , Amerio Pierluigi , Morgese Guido
Published: March 2008

Abstract: The prevalence of persistent microalbumlnuria, retinopathy, and peripheral and autonomic neuropathy was assessed In 18 children and adolescents with type 1 (insulin‐dependent) dlabetes mellitus (IDDM) who suffered from necrobiosls llpoldlca diabeticorum (NLD) and in 40 dlabetics without NLD, matched for sex, age, duration of disease, and metabolic control. The mean ± SD age ot the patients was 15.1 ± 8.6 years (range 7.9–23.9 yrs) and their duration of IDDM was 10.9 ± 8.1 years (range 7.1–21.0 yrs). Their mean glycosylated hemoglobin level was 9.9 ± 5.0% (7.3–16.6%) and their fructosamine level was 274 ± 180 μmol/L (199–466 μmol/L). Patients with NLD had a higher frequency of persistent microalbumlnuria (p < 0.001) and retlnopathy (p < 0.001) than those without NLD. Our study suggests that children as well as adult diabetics with NLD can be at high risk for nephropathy and retinopathy; NLD can be a clue for diabetic nephropathy and retinopathy.
Henoch‐Schonlein Purpura Presenting as Hemorrhagic Vesicles and Bullae: Case Report and Literature Review
Authors: Wananukul Siriwan , Pongprasit Prapai , Korkij Wiwat
Published: March 2008

Abstract: Henoch‐Schonlein purpura is a syndrome characterized by nonthrombocytopenic purpura accompanied by arthralgia, gastrointestinal symptoms, or renal involvement. We report a 5.5‐year‐old boy with high fever and hemorrhagic vesicles and bullae varying in size from 2 to 50 mm in diameter, on both pinnas, the hard palate, gums, dorsa of the hands, buttocks, and both legs, and review the pertinent literature.
Childhood Subacute Cutaneous Lupus Erythematosus Associated with Homozygous Complement 2 Deficiency
Authors: Buckley Deirdre , Barnes Louise
Published: March 2008

Abstract: We describe a 9‐year‐old boy with an Ro‐positive, subacute, cutaneous lupus rash associated with homozygous C2 deficiency. His response to a mild topical steroid and sunscreen was excellent. Hereditary complement deficiency and its association with childhood lupus erythematosus are discussed.
Late, Painful, Subungual Hyperkeratosis in Incontinentia Pigmenti
Authors: Abimelec P. , Rybojad M. , Cambiaghi S. , Moraillon I. , Cavelier‐Balloy B. , Marx C. , Morel P.
Published: March 2008

Abstract: The postpubertal appearance of subungual, painful keratotic tumors is a rare feature of incontinentia pigmenti. A patient affected by incontinentia pigmenti developed subungual, painful, nontumoral, hyperkeratotic lesions of the hands at 10 years of age. The mildness of the subungual lesions may be explained by the early stage of the disorder, but it is difficult to correlate the severity of the fingertip pain with the absence of true tumoral swelling. To our knowledge this is the youngest patient reported so far and the only one with a prepubertal expression of this puzzling disorder.
Solid Alveolar Rhabdomyosarcoma of the Hand in Adolescence: A Clinical, Histologic, Immunologic, and Ultrastructural Study
Authors: Bianchi L. , Orlandi A. , Iraci S. , Spagnoli L. G. , Nini G.
Published: March 2008

Abstract: Rhabdomyosarcoma (RMS), a high‐grade, malignant, skeletal muscle tumor, represents approximately 5% of neoplasms in children. The poorly differentiated forms of RMS are often not easily diagnosed and classified. Among the four histologic variants, alveolar RMS is the least frequently reported subtype. A poorly differentiated solid variant of alveolar RMS occurred on the right hand of a 16‐year‐old girl. Because of the tumor size, local invasiveness, and occurence of cutaneous and breast metastases at presentation, the clinical staging was group IV (T2/N0/M1). Surgical excisions of the primary and metastatic locations were performed and chemotherapy with vincristine, dactinomycin, cyclophosphamide, and doxorubicin was administered. Light and electron microscopy studies revealed a solid proliferation with a focal alveolar pattern of monomorphous, small, round neoplastic cells without easily detectable muscular morphologic features. The skeletal muscle origin was revealed by the positive immunostaining for desmin, α‐sarcomeric actin, muscle‐specific actins, and enolase, and confirmed by immunoblotting for desmin. Despite the age of our patient, which is considered by some authors an independent predictor of outcome, all prognostic variables were unfavorable. However, a disease‐free interval during three years of follow‐up underlines the importance of multidisciplinary regimens for the treatment of this rare solid tumor of childhood and adolescence.
The Prevalence of Skin Disease Among School Children in Rural Ethiopia—A Preliminary Assessment of Dermatologic Needs
Authors: Figueroa Jose L , Fuller L. Claire , Abraha Aynalem , Hay Rod J.
Published: March 2008

Abstract: It Is always assumed that the prevalence of skin diseases in developing countries is very high, and that infestations and skin Infections are highly endemic in poor rural communities; however, very few epidemiologlc reports verify these assumptions. As part of a continuing Study of dermatologic needs in southwestern Ethiopia, and to estimate the prevalence of treatable skin disease in children, a school survey was undertaken in Shebe. In October 1992,112 children were examined by a team of dermatologists and their conditions were recorded. Twenty‐two children (19.6%) were considered healthy and 90 (80.4%) had one or more skin diseases. A total of 140 conditions were identified and considered treatable in 98% of children. Infestations were the most prevalent skin pathology, 81.2%, followed by fungal infections, 13.4%.
Primary Anetoderma in Children: Report of Two Cases and Literature Review
Authors: Karrer Sigrid , Szeimies Rolf‐Markus , Stolz Wilhelm , Landthaler Michael
Published: March 2008

Abstract: Two boys, age 7 and 9 years, with the diagnosis of primary anetoderma are presented, in one patient a growing number of indolent lesions developed for one year on the neck. The other boy complained of Single lesions appearing over four months on the arms, teet, and chest wall. Individual lesions measured up to 1 cm In diameter and showed a palpable herniation phenomenon and wrinkled surface. The lesions did not have an inflammatory onset. Histologically, in both patients the diagnosis of anetoderma was verified by loss of elastic fibers and a lympho‐histiocytic infiltration in the middermis. Administration of oral penicillin for three weeks did not result in marked improvement, and atrophic macules continued to appear in the younger boy. Although no autoimmune logic abnormalities or other associated diseases have arisen in our patients, long‐term follow‐up is mandatory to detect autoimmune disorders that are reported to occur In the course of the disease.
Electronmicroscopic Observation of the Basement Membrane Zone in Focal Dermal Hypoplasia
Authors: Lee Joon , Cha Myung Soo , Kim Soo Chan , Bang Dongsik
Published: March 2008

A 3‐month‐old boy had reticulated achromic and atrophic skin lesions on the right buttock and thigh, as well as partial syndactyly of the third and fourth toes of the right foot. With hematoxylin and eosin stain, an epidermal defect, abnormally located fat cell lobules, and absence of the upper and midportions of the dermis were observed, conforming to a typical histopathologic picture of focal dermal hypoplasia. Electron microscopic examination of the atrophic site showed loose collagen bundles, collagen fibers with loss of regular bands, abnormal fibroblasts, and disruption of the basement membrane zone. These defects in the basement membrane zone strongly suggest that abnormal formation of type IV collagen is associated with focal dermal hypoplasia, and that this abnormal formation of collagen is correlated with the clinical sign of skin atrophy.
Changes in Skin pH after the Use of Baby Wipes
Authors: Priestley G. C. , MeVittie E. , Aldridge R. D.
Published: March 2008

We recently made a double‐blind safety‐in‐use comparison of four different brands of baby wipes using a panel of 302 infants over a period of 10 weeks. For the first two weeks, only soap and water was used to clean the babies’ skin after each diaper change, and then for the eight‐week test phase each baby was allocated one or other of the products for normal home use. The wipes differed in cleansing lotion formulation (emollients, preservative, pH) and fibrous composition. There were no clinically detectable differences in the effects of the wipes in terms of erythema, frequency of rashes, edema, and desquamation, but we recorded significant changes in the pH of pubic and buttock skin inside the diaper area. In particular, the brand of wipes with the lowest pH (2.8) in the lotion reduced the mean skin pH from 5.6 to 5.0 (p < 0.01), and those with a pH of 5.5 had no significant effect. Wipes of intermediate pH (3.7) gave a final skin pH of 5.4‐but the downward trend was not statistically significant. These data indicate that skin pH can be depressed by such topical application, although the trial lasted only a fraction of the total time wipes might be used on each infant. Further research is necessary to evaluate the implications of these findings.
Basal Cell Carcinoma in a Girl after Cobalt Irradiation to the Cranium for Acute Lymphoblastic Leukemia: Case Report and Literature Review
Authors: García‐Silva Jesús , Velasco‐Benito Juán Agustin , Peña‐Penabad Carmen , Armijo Miguel
Published: March 2008

A 12‐year‐old girl had a lesion located on the upper part of the scalp that was clinically interpreted as a melanocytic nevus and corresponded histologically to a basal cell carcinoma. At age 2 years she had been diagnosed as having acute lymphoblastic leukemia L1, for which she was treated with systemic chemotherapy. She also received telecobalt therapy to the whole cranium for prophylaxis of meningeal leukosis. Having rejected other possible causes that can favor the appearance of basal cell carcinoma during childhood (basal cell nevus syndrome, nevus sebaceus, albinism, etc.), we believe that this case should be added to the five previously reported in the literature as basal cell carcinoma developing at an early age in cranial radiation portals with megavoltage radiotherapy. We draw attention to the need to explore radiated areas of skin as part of the long‐term follow‐up of these patients, and to the advisability of obtaining a biopsy from any persistent lesion in these areas.
International Summit on Cutaneous Antifungal Therapy, Focus on Tinea Capitis, Boston, Massachusetts, November 11–13, 1994
Authors: Elewski Boni E. , Hay Roderick J.
Published: March 2008

This article reports the highlights of presentations made at an international symposium held on November 11–13, 1994, in Boston, Massachusetts, on the subject of cutaneous antifungal therapy. Some of the key points pertaining to the epidemiology, etiology, pathogenesis, presentation, and management of tinea capitis are discussed.
Subungual Exostosis: Case Report and Review of the Literature
Authors: Davis Daniel A. , Cohen Philip R.
Published: March 2008

Abstract: Subungual exostosis is typically a benign, acquired tumor of cartilaginous bone occurring on the medial surface of the distal hallux. A 16‐year‐old boy with subungual exostosis of his left great toe is presented. We evaluated the English language literature on this topic from 1857 to 1994; including our patient, 312 cases have been described. The diagnostic characteristics (histologic and radiologic), etiologic features, and therapeutic management were reviewed. Subungual exostosis more commonly affects women than men In a ratio of 2:1. Most lesions occur in the early twenties; however, a minimum of 51 (16%) of the 312 cases of subungual exostoses appeared in children 18 years of age or younger. Seventy percent of lesions occurred on the first hallux. There was frequently an association with trauma. The diagnosis of subungual exostosis may be suspected from the clinical presentation and confirmed with radiographic examination. The treatment is surgical.
Cutis Marmorata Telangiectatica Congenita or Neonatal Lupus?
Authors: Carrascosa Jafse M. , Ribera Miquel , Bielsa Isabel , Coroleu Wenceslao , Ferrándiz Carlos
Published: March 2008

Abstract: Cutis marmorata telangiectatica congenita (CMTC) is a cutaneous vascular abnormality characterized by a persistent cutis marmorata pattern, spider nevuslike telangiectasia, and superficial ulceration. An infant girl was noted at birth to have characteristic features of CMTC as well as anti‐Ro/SSA antibodies and a positive autonuclear antibody (ANA) test. Serologic studies carried out on her mother were positive for Ro/SSA antibodies as well as ANA (titer 1:2560). Therefore the diagnosis of neonatal lupus was made. We propose that livedo patterns mimicking CMTC might sometimes represent the residual phase of neonatal lupus active during intrauterine life.
Papular Urticaria in Children
Authors: Howard Renée , Frieden Ilona J.
Published: March 2008

Abstract: Papular urticaria is a common pruritic hypersensitivity reaction to the bites of insects of many different types, including fleas, mosquitoes, chiggers, and, more rarely, avian or rodent mites. Which particular insect is the cause varies with geographic location. In San Francisco, children with papular urticaria due to flea bites are part of dermatologic practice year round. With its relatively warm winters and wet, foggy summers, San Francisco is an environment where fleas thrive, and their bites afflict children at day care centers and park playgrounds, as well as in homes with pets. We review the clinical approach to papular urticaria in general, and focus on flea bite prevention and management.
Aerobic and Anaerobic Microbiology of Necrotizing Fasciitis in Children
Authors: Brook Itzhak
Published: March 2008

Abstract: Specimens obtained from eight children with necrotizing fasciitis (NF) were cultured for aerobic and anaerobic bacteria. A total of 21 isolates were recovered, 13 anaerobic and 8 aerobic or facultatives. The facultative organism Streptococcus pyogenes was present alone in two (25%) instances, and mixed aerobic and anaerobic bacteria were isolated in six (75%). The predominant isolates were Peptostreptococcus spp. (6 isolates, including 3 Peptostreptococcus magnus). S. pyogenes (4), Bacteroides fragilis group (3), Clostridium perfringens (2), Escherichia coli (2), and Prevotella spp. (2). Organisms similar to the ones isolated from the NF aspirates were recovered in the blood of all patients except one. These included S. pyogenes (3 isolates). B. fragilis group (2), E. coli (1), and P. magnus (1) and Clostridium perfringens (1). All patients underwent surgical fasciotomy, and four required skin grafting. Antimicrobials were administered to all children. Despite extensive resection and intense supportive therapy, three patients died from sepsis accompanied by shock acidosis and disseminated intravascular coagulation. These findings illustrate the polymicrobial aerobic‐anaerobic flora of NF in children.
Staphylococcal Blistering Dactylitis: Report of Two Patients
Authors: Woroszylski Alejandro , Durán Carola , Tamayo Lourdes , de la Luz Orozco Ma. , Ruiz‐Maldonado Ramon
Published: March 2008

Abstract: Two children with staphylococcal blistering distal dactylitis (BDD), a condition usually caused by streptococci, are reported. Etiologic distinction in BDD can be made only bacteriologically. Staphylococci are increasingly the cause of bacterial cutaneous infections.
Neonatal Lupus Erythematosus in an Infant with Turner Syndrome
Authors: Ruas E. , Moreno A. , Tellechea O. , Rocha G. , Raptista A. Poires
Published: March 2008

Abstract: Neonatal lupus erythematosus (NLE) is characterized by transient, annular cutaneous lesions, congenital heart block, and a variety of systemic or hematologic abnormalities. We describe a white infant girt with onset of skin lesions on the face and scalp at 4 days of age. At age 4 weeks she had generalized, erythematous, scaly, annular skin lesions that underwent spontaneous regression at age 5 months. Her mother had no cutaneous or other lesions, but complement examinations revealed the presence of anti‐Ro(SSA) and anti‐La(SSB) antibodies, and absence of anti‐Sm and anti‐RNP antibodies. Karyotyping revealed Turner syndrome (TS) with 45,XO sex chromosome constitution. Ro(SSA) and La(SSB) antibodies were found, and direct immunofluorescence testing on healthy skin was positive. At age 5 months, follow‐up immunologic examination of the infant had normal results but the mother still had anti‐Ro(SSA) and anti‐La(SSB) antibodies. We believe that this is the first reported case of NLE in association with TS.
Melanoma in Children and Adolescents
Authors: Wu Sandra Jones , Lambert David R.
Published: March 2008

Abstract: Melanoma is a life‐threatening and rare malignancy in childhood and adolescence. Because it is so uncommon, melanoma may not be recognized readily or considered seriously in a differential diagnosis, resulting in delays in definitive treatment. We review melanoma occurring in 13 patients under 20 years of age who presented to a single tertiary care children's hospital. Five patients had nonmetastatic primary cutaneous melanoma, with Breslow thickness measurement ranging from 0.84 to 7.8 mm. Three patients had metastatic primary cutaneous melanoma, three had metastatic melanoma arising in a congenital giant nevus, and two had primary leptomeningeal melanoma. All of the patients were given definitive surgical treatment; some received adjunctive radiation therapy and chemotherapy. Overall 5‐year survival rate was 40.0% for all cutaneous melanomas, which included 100% for clinical stage I disease, and 0% for clinical stage II and III disease. Both patients with primary leptomeningeal melanoma died an average of 6 months following diagnosis. Nonmetastatic primary cutaneous melanoma is a survivable disease if detected early and treated by surgical excision; metastatic and leptomeningeal disease were uniformly fatal. Types of melanoma, risk factors for melanoma, and the role of the dermatologist in primary prevention and detection are discussed.
Nonlipidized Juvenile Xanthogranuloma: A Histologic and Immunohistochemical Study
Authors: Newman Cathy C. , Raimer Sharon S. , Sanchez Ramon L.
Published: March 2008

Abstract: Among all patients with a pathologic diagnosis of juveniie xanthogranuloma (JXG) seen at our institution from 1983 to 1994, we identified five patients with an unusual histologic pattern that differed from the classic juvenile xanthogranuioma (CJXG) with foamy cells and Touton giant cells. Four of these five cases, which we termed nonlipidized juvenile xanthogranuloma (NJXG), were seen in infants. The histologic features include a monomorphic infiltrate with absent or few foam cells and Touton giant cells. There is little inflammation, and mitotic figures are easily found. Four cases exhibit a diffuse sheetiike pattern while one is trabecular. Immunoperoxidase staining was done. Ail lesions are consistently positive for factor Xllla as opposed to only focally positive or negative in CJXG and negative in Langerhans cell histiocytosis (LCH). The S‐100 was negative. NJXG represents an atypical histologic variant of JXG, which may suggest a malignant or aggressive tumor. The followup, however, indicates that these lesions behave in a fashion similar to those of CJXG. The differential diagnosis should be made with LCH, intradermal nevus, and reticulohistiocytosis. The immunoperoxidase findings help to differentiate NJXG from these entities.
Neurologic Abnormalities in Two Patients with Facial Hemiatrophy and Sclerosis Coexisting with Morphea
Authors: Menni Silvano , Marzano Angelo Valerio , Passoni Emanuela
Published: March 2008

Abstract: Progressive facial hemiatrophy or Parry‐Romberg syndrome is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue, and the underlying bony structures. This syndrome has many features of linear scleroderma en coup de sabre but is distinguished by more extensive involvement of the lower face and by only slight cutaneous sclerosis. We describe two unusual children with both atrophic and sclerotic changes of half of the face coexisting with multiple plaques of typical morphea. Both children developed neurologic disturbances with cranial magnetic resonance imaging (MRI) abnormalities 2 years and 15 years, respectively, after the onset of cutaneous lesions. Thus considering that it may not be possible to correlate impairment in neurologic function and cutaneous disease, as illustrated by our patients, we emphasize the importance of an accurate follow‐up.
A Case of Bloom Syndrome with Conjunctival Telangiectasia
Authors: Sahn Eleanor E. , Hussey Richard H. , Christmann Linda M.
Published: March 2008

Abstract:Bloom syndrome is a rare genodermatosis characterized by photosensitivity, telangiectasias, growth retardation and malignancies. Eye findings have rarely been mentioned in case reports of this syndrome. We report a child with Bloom syndrome who had pronounced bulbar conjunctival telangiectasia originally diagnosed as episcleritis. Bulbar telangiectasia are frequently described in other genodermatoses such as ataxia telangiectasia and hereditary hemorrhagic telangiectasia, but are Infrequently noted in Bloom syndrome. Previously described eye findings in Bloom syndrome are reviewed and the differential diagnosis of bulbar telangiectasia is discussed.
Neonatal Lupus Erythematosus Related to Maternal Leukocytoclastic Vasculitis
Authors: Borrego Leopoldo , Rodrìguez Julio , Soler Elena , Jiménez Antúnez , Hernández Buenaventura
Published: March 2008

Abstract: Neonatal lupus erythemalosus (NLE) is an autoimmune disease whose major findings are skin lesions and congenital heart block. Affected infants have maternal, transplacentally acquired, autoantibodies to Ro/SSA, La/SSB, or U,‐RNP antigens. Anti‐Ro/SSA is the predominant autoantibody, present in about 95% of cases. Mothers of babies with NLE may be asymptomatic initially or may have Sjógrent syndrome, lupus erythematosus, overlap syndrome or, uncommonly, leukocytoclastic vasculitis. When evaluating a young woman with a cutaneous leucocytoclastic vasculitis, dermatologists should be aware of the possible presence of antibodies related to NLE. If any patient suffering a disorder related to NLE becomes pregnant, testing for autoantibodies and close obstetric prenatal care with fetal echocardiogram is necessary. In cases of fetal bradycardia, treatment with dexamethasone or betamethasone should be considered, as these drugs are accessible to the fetal circulation.
Acne Fulminans Treated with Isotretinoin and “Pulse” Corticosteroids
Authors: Allison Matthew A. , Dunn Cary L. , Person Donald A.
Published: March 2008

Abstract: Acne fulminans (AF) is a rare and severe form of acne that occurs almost exclusively in white adolescent boys. We report AF in a 16‐year‐old white boy developing 14 months after the onset of uncomplicated acne vulgaris. The symptomatology, etiology, treatment, and prognosis of AF are discussed.
Lichen Striatus: Simultaneous Occurrence in Two Nonrelated Siblings
Authors: Smith Sidney B. , Smith Jeffrey B. , Ellis Leighton E. , Hogan Daniel J.
Published: March 2008

Abstract: Lichen striatus is an uncommon, self‐limited eruption of unknown etiology. What role genetic or environmental influences play in its pathogenesis is unclear. We report two nonrelated, adopted children living in the same household who simultaneously developed lichen striatus.
Congenital Constriction Band of the Trunk
Authors: Bahadoran Ph. , Lacour J.‐Ph. , Terrisse A. , Ortonne J.‐P.
Published: March 2008

Abstract: Constriction band of the trunk is a rare congenital malformation. A 2‐year‐old girl with a congenital circumferential constriction band around the waist is described. Six other cases of congenital constriction band of the trunk are reviewed. Like constriction band of the limbs, this malformation is probably related to the amniotic band syndrome. Surgical correction should be considered carefully because natural evolution is usually benign and the postoperative esthetic benefit may be questionable. There is no evidence of any risk of familial recurrence.
Ear Lobe Bilateral Necrosis by Levamisole‐Induced Occlusive Vasculitis in a Pediatric Patient
Authors: Menni Silvano , Pistritto Grazia , Gianotti Raffaele , Ghio Luciana , Edefonti Alberto
Published: March 2008

Abstract: We describe a bullous‐hemorrhagic reaction of the ear lobes in a child with nephrosis after levamisole intake. This drug is used in children as an alternative treatment of corticosteroid‐responsive nephrotic syndrome. Histologic evaluation of a lesion revealed necrotizing vasculitis involving mainly the large vessels of the deep plexus. The lesions disappeared 25 days after discontinuation of treatment.
Misdiagnosis, Treatments, and Outcomes in the Immunobullous Diseases in Children
Authors: Weston William L. , Morelli Joseph G. , Huff J. Clark
Published: March 2008

Abstract: As a group, the immunobullous diseases are very uncommon in childhood. We analyzed all cases of immunobullous diseases in individuals under age 18 years which we had evaluated at the University of Colorado and examined their age of onset, diagnostic difficulties, treatments, and outcomes. This was considered to be a representative North American population within a single referral area which contained a diverse ethnic population mixture. Further, we believe this study to be uniquely uniform in that all the patients were examined by us using the same diagnostic strategy and the immunofluorescent (IF) diagnostic tests were performed by the same individual (J. C. Huff) using uniform diagnostic criteria and consistent IF techniques.
Herlitz Junctional Epidermolysis Bullosa:A Case Report and Review of Current Diagnostic Methods
Authors: Basarab T. , Dunnill M. G. S. , Eady R. A. J. , Russell‐Jones R.
Published: March 2008

Abstract: We report an infant with Herlitz junctional epidermolysis bullosa (JEB) presenting at birth with erosions on the scalp, thigh, and periumbilical area in addition to nail abnormalities. Ultrastructural studies demonstrated a split through the lamina lucida with poorly formed hemidesmosomes and no clearly defined subbasal dense plates. Indirect immunofluorescence staining with antibodies GB3 (antiiaminin 5) and 19‐DEJ‐1 (antiuncein) was totally absent. These findings, in combination with the clinical picture, favor a diagnosis of Herlitz JEB, Immunohistochemistry findings greatly facilitated an accurate diagnosis, which is essential in view of the poor prognosis for patients with this form of junctional epidermoiysis bullosa.
Diffuse Whitening of the Oral Mucosa in a Child
Authors: Hernández‐Martin Angela , Fernández‐López Emilia , Unamuno Pablo , Arnrjijo Miguel
Published: March 2008

Abstract: We report a healthy 16‐year‐old Caucasian boy, who consulted us for white, asymptomatic lesions in the mouth. The lesions were stable and had been present for 6 years. On physical examination, there were diffuse white, soft, corrugated plaques involving the buccal and labial mucosa, oral commissures, and floor of the mouth. No other mucosae were affected and there were no skin or nail abnormalities. The histologic findings revealed epidermal hyperplasia with parakeratosis and Intracellular edema in the squamous cell layer. No nuclear atypia was observed. A differential diagnosis of three entities is proposed: white sponge nevus, leukoedema, and focal epithelial hyperpiasia.
Use of an Emollient As a Steroid‐Sparing Agent in the Treatment of Mild to Moderate Atopic Dermatitis in Children
Authors: Lucky Anne W. , Leach Alan D. , Laskarzewski Peter , Wenck Horst
Published: March 2008

Abstract: The effectiveness of an emollient as an adjunct to topical corticosteroid therapy for the treatment of mild to moderate atopic dermatitis was studied for 3 weeks in 25 children 3 to 15 years of age in comparison with corticosteroid therapy alone. The adjunctive regimen of a once‐daily application each of hydrocortisone 2.5% cream and of a water‐in‐oil cream was equivalent in efficacy to the comparative regimen of twice‐daily applications of hydrocortisone 2.5% cream. Both treatment regimens elicited significant improvement in skin condition by day 7 (p < 0.005) and further significant improvement by day 14 (p < 0.005). No significant differences between the two treatment regimens were observed in the rates of improvement (p > 0.545) or in the reductions in mean lesion size (p > 0.9B). No differences were observed in parental evaluations, except for ease of application where a slight preference was expressed for the hydrocortisone 2.5% cream preparation (p < 0.038). We conclude that emollient adjunct i ve therapy offers a steroid‐sparing alternative to topical corticosteroids alone in the treatment of mild to moderate atopic dermatitis.
Menkes' Kinky Hair Syndrome: Ultrastructural Cutaneous Alterations of the Elastic Fibers
Authors: Martins C. , Gonçalves C. , Moreno A. , Gonçalves O. , Baptista A. Poiares , Bairos V.
Published: March 2008

Abstract: Menkes' kinky hair syndrome is associated with the defecive functioning of several copper‐dependent enzymes due to impaired copper absorption, transport, or metabolism. Lysyl oxidase is a copper‐requiring enzyme that catalyzes the oxidative deamination of lysyl residues linking two adjacent chains of tropoelastin polypeptides into an insoluble network, Elastin of the connective tissue is the responsible protein for the elastic properties of the skin. We report transmission electron microscopy findings concerning elastic fiber alterations of the skin in three patients with Menkes' syndrome. The reticular dermis showed marked changes in the elastic fibers with a paucity of the central amorphous component while retaining normal microfibrillary material. These ultrastructural observations, to the best of our knowledge, are reported for the first time in skin from these patients and may be readily interpreted In terms of a specific biochemical defect in elastogenesls.
Epidermolysis Bullosa Associated with Congenital Localized Absence of Skin, Fetal Abdominal Mass, and Pyloric Atresia**
Authors: Puvabanditsin Surasak , Garrow Eugene , Samransamraujkit Rujipat , Lopez Louis Alfonso , Lambert W. Clark
Published: March 2008

Abstract: 2320‐g male infant was delivered at 35 weeks gestation to a mother who had polyhydramnios. He had a combination of congenital localized absence of skin, unilateral hydronephrosis, and hydroureterdue to ureterovesical obstruction, and nonbilious vomiting due to pyloric atresia. Blistering of the skin developed after birth. Epidermolysis bullosa simplex was confirmed by electron microscopy of a skin biopsy specimen. We describe this patient, who had three unusual manifestations of epidermolysis bullosa.
Neonatal Dermal Hematopoiesis Associated with Diffuse Neonatal Hemangiomatosis
Authors: Evole‐Buselli Montserrat , Hernandez‐Marti Miguel J. , Gascó‐Lacalle Blanca , Menor Carlos Esquembre , Mascuñan‐Diaz Ignacio , Sorni‐Valls Guillermo
Published: March 2008

Abstract: This report describes a neonate with dermal hematopoiesis associated with diffuse hemangiomatosis. The cutaneous lesions consisted of multiple red papules and bluish subcutaneous nodules scattered over his body. The nodules were bluish due to the presence of hematopoietic tissue within the hemangiomas. Although neonatal dermal hematopoiesis has been described with viral infections or hematologic dyscrasias, the association with diffuse hemangiomatosis has not been previously described.
Perineal Median Raphe Cyst
Authors: LeVasseur Major John G. , Perry Lt. Col. Van E.
Published: March 2008

Abstract: Median raphe cysts occur as a developmental anomaly along the midline male perineum, A perineal median raphe cyst arising in a 3‐year‐old boy was successfully treated with primary excision. The possible origins and histologic features are briefly reviewed.
Constipation in Epidermolysis Bullosa: Successful Treatment with a Liquid Fiber‐Containing Formula
Authors: Haynes Lesley , Atherton David , Clayden Graham
Published: March 2008

Abstract: in epidermolysis bullosa (EB), chronic constipation, painful defecation, and fecal impaction frequently contribute to malnutrition and growth fallure. Standard treatments for constipation, such as Increased intake of conventional dietary fiber and fluids and/or the use of laxatives and stool softeners, are largely unsuccessful. We evaluated by questionnaire the use of a fiber‐containing liquid formula (Enrich®) in 20 chronically constipated children with dystrophic EB. All derived substantial improvement in constipation when taking 250 to 750 ml Enrich® per day. We recommend that such a fiber‐containing food be prescribed for chronic constipation in EB. In cases of fecal impaction, this should be preceded by bowel cleansing.
APPLICATION OF THE PDMPO TECHNIQUE IN STUDYING SILICA DEPOSITION IN NATURAL POPULATIONS OF FRAGILARIA CROTONENSIS (BACILLARIOPHYCEAE) AT DIFFERENT DEPTHS IN A EUTROPHIC RESERVOIR1
Authors: Znachor Petr , Nedoma Jiří
Published: March 2008

At weekly intervals from July to October 2006, we measured silica deposition in the summer diatom assemblage at various depths in the eutrophic Římov Reservoir (Czech Republic) using PDMPO, the 2‐(4‐pyridyl)‐5{[4‐(2‐dimethylaminoethyl‐aminocarbamoyl)‐methoxy]phenyl}oxazole labeling technique. Fluorescence microscopy coupled with image analysis allows quantifying silicon (Si) deposition over time and a simple distinction between cells that are actively depositing Si and those that are not. Diatom assemblage was exclusively dominated by Fragilaria crotonensis Kitton, which formed pronounced subsurface maxima (2–6.5 m). Concentrations of the main nutrients (Si and phosphorus, P) were low over the whole season; however, at depth, the nutrient availability was higher than at the surface. Fragilaria silica deposition rates were eight times higher at the surface than at depth. Half the population was involved in silica deposition at the surface, while only 20% active cells were doing so at depth. At the surface, silica deposition was limited by P deficiency; the effect of dissolved Si (DSi) was not statistically significant. Silica deposition at depth was significantly constrained by low light availability despite the 1% average light attenuation at depth, which is supposed sufficient for photosynthesis. This study represents the first attempt to employ the PDMPO technique coupled with quantitative image analysis of PDMPO fluorescence in freshwater ecology. On the basis of our results, PDMPO probe appears to be an appropriate proxy for the study of resource limitation in natural diatom populations.
Psychosocial Differences Between High‐Risk Acute vs. Chronic Low Back Pain Patients
Authors: Gatchel Robert J. , Bernstein Dana , Stowell Anna W. , Pransky Glenn
Published: March 2008

▪ Abstract:  The present study was designed to evaluate the relative degree and type of emotional distress in high‐risk acute low back pain (ALBP) subjects (defined as less than 3 months since initial injury) vs. high‐risk chronic low back pain (CLBP) subjects (defined as greater than 3 months since initial injury). It is an extension of earlier findings that demonstrated the significant role that such emotional distress may play in the development of CLBP disability if not appropriately treated in the acute phase. This work stems from a conceptual three‐stage model, which characterizes the progression from acute to chronic pain. Several psychosocial measures were administered that included information allowing for the classification of subjects as high‐risk based upon an earlier developed screening algorithm. The ancova procedure in SPSS was used to compare groups, controlling for gender, ethnicity, and age. Results revealed that CLBP subjects had higher rates of certain measures of emotional distress and depression relative to ALBP subjects. These findings further support the importance of effectively managing emotional distress factors early, when treating musculoskeletal disorders such as low back pain. ▪
PERSPECTIVE: How Honda Innovates*
Authors: Meyer Marc H.
Published: March 2008

This article describes how Honda leverages its engine and other key subsystem technologies into new products uniquely designed for new target users and their new special uses. In this profile of the development of the Honda Element sports utility vehicle, we see how firm can identify new target applications, dive deeply into user research, and translate its acquired insights into specific designs and features. The feasibility and profitability of the effort is maximized by leveraging robust, fully operational platforms within new product lines.
New Product Success: Is It Really Controllable by Managers in Highly Turbulent Environments?
Authors: Droge Cornelia , Calantone Roger , Harmancioglu Nukhet
Published: March 2008

This research proposes and tests a model of direct and indirect effects linking four antecedents to new product success: (1) a proactive strategic orientation along with enabling (2) organic organizational structures should lead to more (3) innovativeness and (4) market intelligence. Innovativeness and market intelligence should in turn lead to greater new product success. The relationships among the four antecedents are not hypothesized to be moderated by environmental turbulence because their domain is intraorganizational. However, the relationships from intraorganizational constructs to new product success are hypothesized to be moderated by environmental turbulence because success depends in part on the environment in which the new product must compete. The model was tested using a sample composed of 202 small business units of manufacturers on the Fortune 500. The sample was heavily involved in new product development: Their average annual research and development budget was $360.4 million, and approximately 8.2% of sales came from products introduced in the last five years. A two‐group structural equation model analysis supports the moderation model overall and reveals the pattern of direct, indirect, and total effects. The results show that innovativeness (but not market intelligence) directly predicts new product success when turbulence is high, whereas market intelligence (but not innovativeness) directly engenders new product success in low turbulence. Environmental turbulence also affects the total indirect impact of strategy proactiveness and organizational organicity on new product success. These indirect effects operate through innovativeness and market intelligence as complete mediators.
A Two‐Stage Probabilistic Approach to Multiple‐Community Similarity Indices
Authors: Chao Anne , Jost Lou , Chiang S. C. , Jiang Y.‐H. , Chazdon Robin L.
Published: March 2008

Summary A traditional approach for assessing similarity among N (N > 2) communities is to use multiple pairwise comparisons. However, pairwise similarity indices do not completely characterize multiple‐community similarity because the information shared by at least three communities is ignored. We propose a new and intuitive two‐stage probabilistic approach, which leads to a general framework to simultaneously compare multiple communities based on abundance data. The approach is specifically used to extend the commonly used Morisita index and NESS (normalized expected species shared) index to the case of N communities. For comparing N communities, a profile of N− 1 indices is proposed to characterize similarity of species composition across communities. Based on sample abundance data, nearly unbiased estimators of the proposed indices and their variances are obtained. These generalized NESS and Morisita indices are applied to comparison of three size classes of plant data (seedling, saplings, and trees) within old‐growth and secondary rain forest plots in Costa Rica.
Affective Match in Leadership: Leader Emotional Displays, Follower Positive Affect, and Follower Performance1
Authors: Damen Frederic , Van Knippenberg Barbara , Van Knippenberg Daan
Published: March 2008

Leader emotions may play an important role in leadership effectiveness. Extending earlier research on leader emotional displays and leadership effectiveness, we propose that the affective match between follower positive affect (PA) and leaders’ emotional displays moderates the effectiveness of leader emotional displays. Leader display of emotions has more positive effects on follower behavior if the match between the valence of leader emotion and follower PA is strong rather than weak. Support for this hypothesis was found in 2 experiments. Congruency between leader emotional displays and follower PA determined follower task performance and extra‐role compliance. Results from the second experiment indicated that this effect is a due to affective aspects of leader behavior and not to the valence of message content.
The Role of Cognitive Ability in Self‐Efficacy and Self‐Assessed Test Performance1
Authors: Truxillo Donald M. , Seitz Rainer , Bauer Talya N.
Published: March 2008

Research has shown that test takers are often unable to assess their own test performance accurately. However, the role of cognitive ability in assessing one's test performance has not been explored. We examined whether high cognitive ability participants were better than low cognitive ability participants in assessing their performance on a video‐based situational judgment test (SJT) of customer‐service skills. Results indicated a strong relationship between actual and perceived SJT performance for high cognitive ability participants, but no relationship for those low in cognitive ability. The discussion focuses on implications for metacognitive theory, test perceptions, and providing feedback to applicants.
Ambivalence and Unprotected Sex: Failure to Predict Sexual Activity and Decreased Condom Use1
Authors: MacDonald Tara K. , Hynie Michaela
Published: March 2008

Using a prospective design, we assessed whether ambivalence toward sexual activity is associated with decreased condom use. Undergraduates predicted whether they would have intercourse and use condoms in the next week. A week later, they reported their sexual and contraceptive behavior. Among 65 individuals who had intercourse and planned to use condoms, ambivalence toward sexual activity was negatively associated with condom use. Moreover, whether intercourse was planned mediated the relationship between ambivalence and condom use, such that ambivalence was negatively associated with accuracy in planning sex, and unplanned sex was less likely to be protected. Individuals who are ambivalent about sex may intend to use condoms, but fail to do so because they cannot predict when they will have intercourse.
The Endocannabinoid System: A Promising Novel Mechanistic Pathway in the Cardiometabolic Syndrome
Authors: Al‐Jaghbeer Eshraq , Khraisat Ahmad , Singh Sant P.
Published: March 2008

The endocannabinoid system (ECS) is a neuroendocrine system that modulates several cardiometabolic processes. An overactive ECS is implicated as a significant contributor to the cardiometabolic syndrome and obesity, in addition to a large number of other physiologic processes. Endocannabinoid receptors have been detected centrally and peripherally, regulating appetite, food intake, metabolism, and storage. ECS blockade is thought to be a promising new pharmacologic modality of improving the unfavorable metabolic risk profile in patients with the cardiometabolic syndrome and obesity.
Effects of Angiotensin‐Converting Enzyme Inhibitor Therapy on Levels of Inflammatory Markers in Response to Exercise‐Induced Stress: Studies in the Metabolic Syndrome
Authors: Vaccari Christopher S. , Rahman Syed T. , Khan Qamar A. , Cheema Faiz A. , Khan Bobby V.
Published: March 2008

The authors sought to determine whether the angiotensin‐converting enzyme (ACE) inhibitor perindopril has beneficial effects on vascular markers of inflammation in patients with the metabolic syndrome when exposed to exercise‐induced stress. Thirty patients with the metabolic syndrome were randomized to perindopril (4 mg/d) or placebo in a double‐blind fashion for 4 weeks. Prior to treatment, the patients underwent an exercise treadmill study to a level of 8 metabolic equivalents. Circulating monocyte CD11b expression, levels of soluble interleukin 6 (sIL‐6), and levels of vascular cell adhesion molecule‐1 (VCAM‐1) were measured. After the treatment period, exercise treadmill study and measurement of markers were repeated. Treatment with perindopril reduced sIL‐6 levels at pre‐exercise by 22% and at 1 and 30 minutes by 30% and 33%, respectively (P<.005). Levels of soluble VCAM‐1 in perindopril‐treated patients were reduced at pre‐exercise by 25% and at 1 and 30 minutes by 31% and 37%, respectively. Treatment with perindopril reduced monocyte CD11b expression by 25%. In response to exercise‐induced physical stress, the addition of an ACE inhibitor differentially regulates markers of inflammation, thereby providing potential vascular protection in the metabolic syndrome.
Bayesian Meta‐Analysis of Tissue Angiotensin‐Converting Enzyme Inhibitors for Reduction of Adverse Cardiovascular Events in Patients with Diabetes Mellitus and Preserved Left Ventricular Function
Authors: Lang Christopher D. , Arora Rohit R. , Saha Sandeep A. , Molnar Janos
Published: March 2008

The role of angiotensin‐converting enzyme (ACE) inhibitors in diabetic patients with preserved ventricular function is uncertain. Tissue ACE inhibitors have been defined by increased lipophilicity and structural characteristics that result in greater tissue‐specific ACE binding when compared with plasma ACE inhibitors. A Bayesian meta‐analysis of randomized trials was conducted to evaluate tissue ACE inhibitors in prevention of cardiovascular disease among patients with diabetes mellitus and preserved left ventricular function. Four trials were selected that evaluated 2 different ACE inhibitors and included 10,328 patients (43,517 patient‐years). The Perindopril Substudy in Coronary Artery Disease and Diabetes (PERSUADE) and the Perindopril Protection Against Recurrent Stroke Study (PROGRESS) compared the effects of perindopril vs a placebo, and the Heart Outcomes Prevention Evaluation (HOPE) and the Non‐Insulin‐Dependent Diabetes, Hypertension, Microalbuminuria, Proteinuria, Cardiovascular Events, and Ramipril (DIABHYCAR) study investigated the impact of ramipril vs a placebo. Bayesian meta‐analysis of sequential trials and sensitivity analysis of therapeutic response were subsequently computed. Bayesian meta‐analysis determined reduced risk of cardiovascular mortality (PB=.991), myocardial infarction (PB=.999), and the need for invasive coronary revascularization (PB=.995) when compared with placebo. Total mortality was also decreased (PB=.967), while the risk of stroke (PB=.907) and hospitalization for heart failure (PB=.923) were impacted. Bayesian meta‐analysis of randomized trials suggests that tissue ACE inhibitors decrease the probability that diabetic patients with preserved left ventricular function will experience myocardial infarctions and cardiovascular death and reduce overall mortality.