bluuebunny/crossref_metadata_2025 huggingface dataset:
Children adopted from China: a prospective study of their growth and development
Authors: Cohen Nancy J. , Lojkasek Mirek , Zadeh Zohreh Yaghoub , Pugliese Mirella , Kiefer Heidi
Published: March 2008

Background:  China has become a lead country for international adoption because of the relatively young age of the children and reported positive conditions of the orphanages. This study examined the process and outcome of growth and development of children adopted from China over their first two years with their adoptive families.

Method:  Seventy infant girls adopted from China at 8 to 21 months of age (Mean age = 13 months) were examined on arrival in Canada and 6, 12, and 24 months later. Comparisons were made with non‐adopted Canadian girls of similar age and from a similar family background as adoptive parents on indices of growth and standardized measures of mental, psychomotor, and language development.

Results:  At arrival, children adopted from China were smaller physically and exhibited developmental delays compared to current peers. Children adopted from China were functioning in the average range on physical and developmental measures within the first 6 months following adoption. However, they were not performing as well as current peers until the end of their second year after adoption. Even then, there was developmental variation in relation to comparison children and continuation of relatively smaller size with respect to height, weight, and head circumference. Physical measurement was related to outcomes at various points on all developmental measures.

Conclusions:  Deprivation in experience in the first year of life has more long‐lasting effects on physical growth than on mental development. The variable most consistently related to development was height‐to‐age ratio. As a measure of nutritional status, the findings reinforce the critical importance of early nutrition.
Research Review: The importance of callous‐unemotional traits for developmental models of aggressive and antisocial behavior
Authors: Frick Paul J. , White Stuart F.
Published: March 2008

The current paper reviews research suggesting that the presence of a callous and unemotional interpersonal style designates an important subgroup of antisocial and aggressive youth. Specifically, callous‐unemotional (CU) traits (e.g., lack of guilt, absence of empathy, callous use of others) seem to be relatively stable across childhood and adolescence and they designate a group of youth with a particularly severe, aggressive, and stable pattern of antisocial behavior. Further, antisocial youth with CU traits show a number of distinct emotional, cognitive, and personality characteristics compared to other antisocial youth. These characteristics of youth with CU traits have important implications for causal models of antisocial and aggressive behavior, for methods used to study antisocial youth, and for assessing and treating antisocial and aggressive behavior in children and adolescents.
Amino acids outside of the loops that define the agonist binding site are important for ligand binding to insect nicotinic acetylcholine receptors
Authors: Liu Zewen , Han Zhaojun , Liu Shuhua , Zhang Yixi , Song Feng , Yao Xiangmei , Gu Jianhua
Published: March 2008

Nicotinic acetylcholine (ACh) receptors (nAChRs) are the targets of several kinds of insecticides. Based on the mutagenesis studies of Torpedo californica nAChRs and solved structure of a molluscan, glial‐derived soluble ACh‐binding protein, a model of the agonist site was constructed with contributing amino acids from three distinct loops (A, B, and C) of the α subunits and another three loops (D, E, and F) of the non‐α subunits. According to this model, most insect nAChR subunits can form the functional heteromeric or homomeric receptors. Actually, insect subunits themselves did not form any functional receptor at various combinations as yet, and only part of them can form the functional receptors with vertebrate non‐α subunits. These findings suggested that the agonist binding for insect nAChRs was not only contributed by those key amino acids in six loops, but also some unidentified amino acids from other regions. In our previous studies on nAChRs for Nilaparvata lugens, a target‐site mutation (Y151S) was found within two α subunits (Nlα1 and Nlα3). In Drosophila S2 cells and Xenopus oocytes, Nlα1 can form functional receptors with rat β2 subunit. However, the same thing was not observed in Nlα3. In the present paper, by exchanging the corresponding regions between Nlα1 and Nlα3 to generate different chimeras, amino acid residues or residue clusters in the regions outside the six loops were found to play essential roles in agonist binding, especially for the amino acid clusters between loop B and C. This result indicated that the residues in the six loops could be necessary, but not enough for the activity of agonist binding.
Tyrosinemia II: Lessons in Molecular Pathophysiology
Authors: Goldsmith Lowell A.
Published: March 2008

Tyrosinemia II is caused by a deficiency of hepatic tyrosine amino‐transferase. With the deficiency of this key enzyme of tyrosinc catabolism there is an increase in plasma tyrosine and then an increase in tyrosinc metabolites in the urine. The increased plasma tyrosinc causes tyrosine to crystallize in the cornea, producing corneal ulcerations and sometimes proliferation of corneal epithelium. In the epiderm is of the palms and soles, tyrosine leads to erosions, crusting, and then hyperkeratosis. The human disease is due to an autosomal recessive gene, and similar genetic diseases have been found in mink and in dogs. A nutritional model for the disease, in which a high‐tyrosine low‐protein diet is ted to rats, produces almost identical features. The features of this disorder and some of the implications of this disease for the study of other genetic diseases is discussed in this review.
Cerebriform Intradermal Nevus
Authors: Lasser Alan E.
Published: March 2008

Abstract: Tumors make up almost 25% of the causes of cutis verticis gyrata. An underlying dermal nevus or neurofibroma is the tumor most often found. Two new cases of cutis verticis gyrata caused by an intradermal nevus cell nevus (cerebriform intradermal nevus) are presented. The importance of early diagnosis and aggressive surgical excision and plastic reconstruction in an attempt to minimize the emotional trauma of the required surgery is stressed. Cerebriform intradermal nevi are a type of large congenital nevi and should be evaluated with this in mind.
The Biology of Human Fetal Skin at Ages Related to Prenatal Diagnosis
Authors: Holbrook Karen A.
Published: March 2008

Abstract: It is the goal of the present article to review the state of knowledge of human skin development and to indicate some of the questions being approached in current studies of human fetal skin. Because of the breadth of the topic, the discussion will be limited to two ages during gestation that correspond to periods when samples of fetal skin or amniotic fluid cells (which in part are derived from the skin) are used to evaluate the condition of the fetus‐ These periods correspond to ages when amniocentesis (14–16 weeks) and fetoscopy accompanied by fetal skin biopsy (19–20 weeks) are performed.
The Rise and Fall of Fluorescent Tinea Capitis
Authors: Prevost Elena
Published: March 2008

Abstract: The epidemiology of tinea capitis has had a remarkable change in the past 20 years. It is important for physicians to realize that most tinea capitis in the United States is caused by Trichophyton tonsurans and that these lesions cannot be diagnosed by the Wood's lamp. Trichophyton tonsurans tinea capitis is frequently misdiagnosed because the lesions mimic such common scalp conditions as dandruff and seborrhea. Further, this organism can cause chronic tinea capitis in women that may become an infectious reservoir for other family members. A negative potassium hydroxide preparation will not rule out infection with T tonsurans, and cultures are necessary. Scalp lesions in children should be considered tinea capitis until culturally proved otherwise.
Halo Scalp Ring: A Form of Localized Scalp Injury Associated with Caput Succedanum
Authors: Neal Patricia R. , Merk Philip F. , Norins Arthur L.
Published: March 2008

Abstract: Caput succedaneum is a common birth injury attributed to cervical, uterine, or vaginal pressure on the fetal presenting part. It usually consists of diffuse soft tissue swelling and bruising, which resolve in a few days without sequelae. We have seen two patients who had an unusual alopecic “halo” ring on the scalp associated with a caput succedaneum.
Epidermodysplasia verruciformis in a Setting of Common Variable Immunodeficiency
Authors: Goldes Jeffry A. , Filipovich Alexandra H. , Neudorf Steve M. , Bender Mitchell E. , Ostrow Ronald S. , Faras Anthony , Goltz Robert W.
Published: March 2008

Abstract: A 14‐year‐old native American female with common variable immunodeficiency was admitted for bone marrow transplantation. Preoperative evaluation showed a generalized lichenoid papular eruption present for several years. Light microscopy revealed expansion of the epidermis by atypical keratinocytes; electron microscopy showed intranuclear papillomavirus inclusions within the granular keratinocytes; DNA hybridization revealed a type 5‐related human papilloma virus homology. Four days after bone marrow transplantation the lichenoid papules blackened and began to disappear. Within 30 days after bone marrow transplantation the distribution and appearance of the papules was similar to that of the pretransplantation evaluation. One year after transplantation the patient showed evidence of a successful T lymphocyte graft. No transformation to squamous cell carcinoma had occurred. Epidermodysplasia verruciformis has been associated with deficient cell‐mediated immunity, the varying severity of which does not predict the tendency to neoplasm formation (2, 5). Several distinct human papillomavirus genomes have been recovered with DNA hybridization techniques in these patients. It is hoped that the bone marrow transplantation might be associated with diminished transformation to squamous cell carcinoma.
Cutis Marmorata Telangiectatica Congenita: A Case Report
Authors: Altman Andrew R. , Tschen Jaime A. , Wolf John E.
Published: March 2008

Cutis marmorata telangiectatica congenita (CMTC) is a rare cutaneous vascular disorder that was first described in 1922 by Van Lohuizen (1). The clinical presentation is one of persistent cutis marmorata, phlebectasia, telangiectasia, and areas of ulcerations. The disorder shows slow clinical improvement over time.

Previously, CMTC has also been described as congenital generalized phlebectasia (2–7), congenital phlebectasia (8), nevus vascularis rcticularis (9), congenital livedo reticularis (10), and livedo telangiectatica (11). The following is a case report.
Bullous Mastocytosis: Diffuse Cutaneous Mastocytosis with Extensive Blisters Mimicking Scalded Skin Syndrome or Erythema Multiforme
Authors: Golitz Loren E. , Weston William L. , Lane Alfred T.
Published: March 2008

Abstract: Bullous mastocytosis (diffuse cutaneous mastocytosis) is a rare form of mast cell disease that begins during the first month of life and causes extensive blisters that mimic scalded skin syndrome or bullous erythema multiforme. Discrete pigmented macules, papules, and nodules arc absent and the characteristic leathery induration of skin may not develop until 6 months of age. Skin biopsy shows a subepidermal blister with mast cells at the base. The most serious complications are gastrointestinal hemorrhage and shock. The symptoms of bullous mastocytosis may be modified by a number of new therapeutic agents.
Blindness, Anonychia, and Oral Mucosal Scarring as Sequelae of the Stevens‐Johnson Syndrome
Authors: Hansen Ronald C.
Published: March 2008

Abstract: In its most severe form, bullous erythema multiforme is known as the Stevens‐Johnson syndrome (SJS). A case of SJS is reported in which ophthalmic complications caused blindness and ultimate enucleation, and in which there occurred permanent loss of nails as well as scarring of the oral mucosa.
Acquired Cutis Laxa: Case Report and Review of Disorders of Elastolysis
Authors: Koch Susan E. , Williams Mary L.
Published: March 2008

Abstract: A 10‐year‐old boy developed cutis laxa while receiving isoniazid therapy; no systemic manifestations occurred. There are several well‐documented cases of acquired cutis laxa. We propose a classification of the elastolysis syndromes, including inherited, neonatal, and acquired forms of cutis laxa.
Localized Vulvar Pemphigoid in a Child
Authors: DeCastro Patricia , Jorizzo Joseph L. , Rajaraman Srinivasan , Solomon Alvin R. , Briggaman Robert A. , Raimer Sharon S.
Published: March 2008

Abstract: Bullous pemphigoid often affects the elderly but is not common in children. At least two localized variants, cicatricial pemphigoid and localized, chronic pemphigoid of Brunsting‐Perry, have been described. A case of bullous pemphigoid in a 9‐year‐old girl was unusual because erosions were limited to the vulvar area. Other bullous dermatoses were considered in the differential diagnosis. Histopathologic examination and immunofluorescence microscopic studies, as well as immunoelectron microscopic studies, which yielded unique results, were performed to confirm the ciagnosis.
Fish Odor Syndrome: Trimethylaminuria with Milk as Chief Dietary Factor
Authors: Rothschild James G. , Hansen Ronald C.
Published: March 2008

Abstract: A 10‐year‐old boy had a two‐year history of a rotten fish odor, noted particularly in the summer after exercise. The odor was partially controlled by frequent bathing. Dietary history was unremarkable except for large daily milk intake. Milk elimination resulted in a prompt disappearance of the odor. Studies of the patient's urine after choline loading established the diagnosis of trimethylaminuria. Restriction of dietary milk as well as foods containing choline and trimethylamine oxide eliminated the offensive odor.
Keratoderma Hereditaria Mutilans (Vohwinkel's Syndrome): A Trial of Isotretinoin
Authors: Goldfarb Michael T. , Woo Thomas Y. , Rasmussen James E.
Published: March 2008

Abstract: An 8‐year‐old girl with the classic findings of keraloderma heredilaria mutilans (Vohwinkel's syndrome) was seen. Treatment with isotretinoin was instituted to decrease the hyperkeratosis and to prevent further autoamputation. After a 12‐week course at 2 mg per kg per day, the patient had only minimal decrease in the amount of hyperkeratosis. Because of Ihe well‐known long‐term risks of systematic retinoids and her suboptimal improvement, therapy was discontinued.
A Solitary Variant of Congenital Self‐healing Reticulohistiocytosis: Solitary Hasimoto‐Prltzker Disease
Authors: Berger Timothy G. , Lane Alfred T. , Headington John T. , Hartmann Kip , Berrish Gene , Levin Marc W. , Disantagnese P. Anthony , Schwartz Joel L. , Lambert John S.
Published: March 2008

Abstract: Four neonates had solitary, congenital, rapidly growing, spontaneously ulcerating tumors of the face, trunk, and extremities. No extracutaneous involvement was found, and all lesions spontaneously involuted. Mononuclear cells of the cutaneous infiltrate were Langerhans'cells. These findings expand the spectrum of congenital self‐healing reticulohistiocytosis.
Primary Cutaneous Amyloidosis: Identically Distributed Lesions in Identical Twins
Authors: LeBoit Philip E. , Greene Ira
Published: March 2008

Abstract: Male HLA‐identical twins with numerous congenital abnormalities were discovered to have lichen amyloidosus in similar distribution on their chests and abdomens. Pruritus was absent in both. The finding of identical distribution of nonpruritic lesions was previously reported in familial lichen amyloidosus. This suggests that a subset of these patients have a nevoid condition in which a circumscribed patch of keratinocytes undergoes filamentous degeneration and may appear in identical distribution in family members in the absence of pruritus.
Facial Sporotrichosis in Children
Authors: Prose Neil S. , Milburn Peter B. , Papayanopulos David M.
Published: March 2008

Abstract: We examined two patients with facial lesions of fixed cutaneous sporotrichosis. That condition should be considered in the differential diagnosis of persistent nodules or plaques in this anatomic location.
Acute Miliary Tuberculosis in a Child with Anhidrotic Ectodermal Dysplasia
Authors: Frix Carl D. , Bronson Darryl M.
Published: March 2008

Abstract: A 20‐month‐old boy with anhidrotic ectodermal dysplasia developed fever and papulopustular skin lesions. A skin biopsy showed the histologic changes of acute miliary tuberculosis. Anhidrotic ectodermal dysplasia is characterized by an absence of seromucous glands in the oropharynx and tracheobronchial tree, making children with this disease prone to viral and bacterial respiratory infections. Mild defects in their immunologic system have also been reported. We suggest that these abnormalities may have predisposed the patient to this rare form of cutaneous tuberculosis.
Congenital Self‐healing Reticulohistiocytosis: Report of a Case
Authors: Jordaan H. Francois , Drusinsky Sarina F.
Published: March 2008

Abstract: A newborn male infant presented with a single crateriform nodule in the region of the right iliac fossa. No extracutaneous involvement was found and the lesion involuted spontaneously without scarring in one month. Histology showed histiocytelike cells of which 10% to 15% contained Birbeck granules. SlOO protein could not be demonstrated. This case possibly represents a solitary variant of congenital self‐healing reticulohistiocytosis.
Chronic Bullous Dermatosis of Childhoo Immonopathologic Studies
Authors: Roberts Lynne J. , Sontheimer Richard D.
Published: March 2008

Abstract: Chronic bullous dermatosis of childhood (CBDC) is a distinctive subepidermai olistenng disorder that characteristicaliy involves the lower Face, neck, lower trunk, and diaper area. Nearly ail affected patients have a conlinuous linear band of igA at the dermoepidermal junction by direct immunoflu‐orescence and many have circulating IgA antibasement membrane antibodies. Few uitrastructural studies have been performed to determine the location of these IgA autoantibodies. We examined an infant with the typical clinical, his‐topathologic. and immunopathologic features of CBDC in whom a circulating, lamina lucida‐reactive IgA autoantibody was identified wilh a new, more sensitive, split‐skin indirect immunofluorescence technique.
Pediatric Human Immunodeficiency Virus Infection and its Cutaneous Manifestations
Authors: Prose Neil S. , Mendez Herman , Menikoff Howard , Miller Harvey J.
Published: March 2008

Abstract: Acquired immunodeficiency syndrome was first observed in children in 1982. Human immunodeficiency virus (HIV) is now known to be the etiologic agent of this disease complex. Children acquire the viral infection in utero or perinatalty, or by receiving contaminated blood products. The cutaneous manifestations include persistent oral thrush, herpes simplex, herpes zoster, molluscum contagiosum, and a variety of fungal and bacterial skin infections. Vasculitis, unusual drug eruptions, and cutaneous manifestations of nutritional deficiencies are also seen.
Kawasaki Disease and its Diagnosis
Authors: Bligard Carey A.
Published: March 2008

Abstract: Mucocutaneous lymph node syndrome, or Kawasaki disease, is a febrile, exanthematous disease of children that has potentially fatal complications. The most important complication is the development of aneurysms in the coronary arteries, which may thrombose or occasionally, rupture and cause severe morbidity or death. Criteria for the diagnosis of Kawasaki disease have been developed that may help the clinician make the diagnosis and prevent complications. The major criteria include fever, skin eruption, ocular changes, oral changes, changes in the extremities, and lymphadenopathy. Prognosis may be evaluated by the clinical picture and cardiac work‐up with echocardiogram.
Eosinophil Fluorescence: A Cause of False Positive Slide Tests for Herpes Simplex Virus
Authors: Detlefs Richard L. , Frieden Dona J. , Berger Timothy G. , Westrom Dale
Published: March 2008

Abstract: Two neonates who had different vesicular eruptions were erroneously diagnosed as having herpes simplex infections because of false positive results of herpes simplex fluorescent antibody slide tests. The infants, who proved to have incontinentia pigmenti and histiocytosis X, respectively, had multiple vesicular lesions containing numerous eosinophils. Eosinophils are known to show nonspecific fluorescence in immunofluorescent techniques because the fluorescein isothiocyanate label of the antiserum binds with the basic proteins in the eosinophilic granules. Clinicians should be aware that nonspecific eosinophil fluorescence may result in false positive results of immunofluorescent slide tests.
Alopecia Areata Symposium
Authors: Thiers Bruce H. , Bergfeld Wiima F. , Fiedler‐Weiss Virginia C. , Weston William L. , Lane Alfred T. , Price Vera H. , Verbov Julian L. , Galen Wesley King
Published: March 2008

Abstract: No dermatologic disorder poses so little threat to health and physical cotnfort and yet is so emotionally devastating as alopecia areata. Fortunately, the rate of spontaneous remission is high; nevertheless, management is problematic because of the considerable cosmetic disability imposed by extensive and visible hair loss, and the unpredictability of response to accepted therapeutic agents. Indeed, opinions vary as to whether or not any of the currently employed therapeutic modalities is effective, and there is remarkable diversity in approach to the patient with this problem.

This symposium includes two lengthier articles on alopecia areata as well as several shorter commentaries on management by dermatologists with a special interest in this disorder.
Linear Porokeratosis in Two Families with Disseminated Superficial Actinic Porokeratosis
Authors: Commens Chris A. , Shumack Stephen P.
Published: March 2008

Abstract: Two case reports of linear porokeratosis occurring in individuals or families with disseminated, superficial, actinic porokeratosis (DSAP) are presented. Linear porokeratosis and DSAP may be different expressions of one dominantly inherited condition. We reviewed the clinical features of linear porokeratosis and its association with other forms of porokeratosis. The linear form has a potential for carcinomatous change.
Uncombable‐hair Syndrome
Authors: Matis Wendy L. , Baden Howard , Green Richard , Boiko Susan , Lucky Anne W. , Homstein Luisa , Ashraf Muhanunad , Hood Antoinette F.
Published: March 2008

Abstract: Four children had short, unmanageable, pale blond hair. They had no associated abnormalities and no family histories of abnormal hair. Light microscopy of the hair was normal in three patients, with piii torti present in the fourth. Electron microscopy of hairs from all four children revealed longitudinal grooves in the hair shaft, diagnostic of uncombable‐hair syndrome.
Chronic Cutaneous Herpes Simplex and X‐Mnked Hypogammaglobulinemia
Authors: Olson Nancy Y. , Hall John C.
Published: March 2008

Abstract: Chronic mucocutaneous or cutaneous herpes simplex is a rare syndrome associated with immunosuppression. We report the first case associated with X‐Iinked hypogammaglobulinemia and illustrate the effectiveness of oral acyclovir in this potentially serious illness.
The Sturge‐Weber Syndrome
Authors: Paller Amy S.
Published: March 2008

Abstract: The Sturge‐Weber syndrome is characterized by angiomas of the leptomeninges overlying the cerebral cortex in association with a facial nevus flammeus. Although frequently included with other neurocutaneous genodermatoses, the syndrome is almost always sporadic in occurrence. The most common associated neurologic abnormality is seizures, which are controlled in more than 50% of patients by the administration of anticonvulsants. Laser therapy is the most promising therapeutic option for cosmetic management of the facial nevus flammeus.
Sarcoidosis in Children: Differentiation from Juvenile Rheumatoid Arthritis
Authors: Mallory Susan B. , Paller Amy S. , Ginsburg Barry C. , McCrossin Ian D. , Abernathy Rosalind
Published: March 2008

Abstract: Sarcoidosis is rare in young children. and is characterized by skin. joint. and eye changes. Differentiated clinically from juvenile rheumatoid ar‐ thritis (JRA) by milder constitutional symptoms and characteristic joint abnor‐ malities. sareoidosis is confirmed by demonstrating noncaseating granulomas in skin. conjunctival, or synovial biopsies. Recent reports have shown children with features of both sarcoidosis and juvenile rheumatoid arthritis. some with similarly affected family members. We cared for four children with sarcoidosis and severe joint manifestations. Two had a personal or family history of JRA. Three of the four children had ichthyosiform cutaneous manifestations. which may suggest an association between severe joint disease and ichthyosiform changes. Because of the difficulty in making a diagnosis on clinical grounds alone. biopsy of cutaneouslesions is recommended in children with these symptoms.
Childhood Bullous Lichen Planus
Authors: Kwee Darlene J. , Dufresne Raymond G. , Ellis Darrel L.
Published: March 2008

Abstract: A child with previous lichenoid lesions had bullae refractory to antistaphylococcal antibiotics. Biopsy of a bulla showed histologic changes of bullous lichen planus. Indirect immunofluorescence using the patient's serum on a perilestional bulla biopsy showed positive staining for IgA and IgG in the stratum granulosum and stratum corneum, confirming the diagnosis. The lesions resolved with dapsone treatment.
Follicular Atrophoderma with Scrotal Tongue
Authors: Tüzün Yalcin , Cem Mat M. , Serdaroglu Server , Kotogyan Agop
Published: March 2008

Abstract: Follicular atrophoderma has been associated with a variety of findings. In our patient's family the disease was present in the mother and all of her living children. Serotal tongue was also found in all but one child. We think that follicular atrophoderma is probably inherited as an autosomal dominant trait.
Umbilical Polyps
Authors: de Luna Margarita Larralde , Cicini Vicente , Herrera Agustin , Casas Josè G. , Magnin Pedro H.
Published: March 2008

Abstract: We report three patients, ages 5 years 3 years. and 4 days, with umbilical polyps. In the third child the polyp was associated with an umbilical enteric fistula. An umbilical polyp is the result of incomplet closure of the omphalomesenteric duct and becomes apparent after the umbilical cord is detached. It is a reddish tumor of a few milmeters: it seldom bleeds or is exudative. We consider it important to study every case in detail in oreder to exclude possible underlying embryologic anomalies such as Meckel's diverticulum and umbilical enteric fistula.
Nutritional and Metabolic Profile of Children with Epidermolysis Bullosa
Authors: Lechner‐Gruskay Donna , Honig Paul J. , Pereira Gilberto , McKinney Shortie
Published: March 2008

Abstract: The clinical features of epidermolysis bullosa (EB) include oral, pharyngeal, gastrointestinal, and total‐body blistering. This results in the potential for decreased oral intake, malabsorption, anemia, and depressed viseral protein stores, and a multifactorial etiology for the development of malnutrition and growth retardation. Thus, it was the purpose of this study to document the nutritional and metabolic profile of seven children with junctional or recessive dystrophic EB as compared to seven age‐ and sex‐matched controls. Each child underwent a comprehensive nutritional assessment, including evaluation of anthropometric, dietary, and biochemical values and determination of resting energy expenditures. This study demonstrated that subjects with EB are statistically different for all anthropometric values studied and represent a population suffering from the effects of acute and chronic malnutrition. Nutrient deficiencies were reported for zinc, magnesium, calcium, potassium, and iron; vitamines A, D, E, B1, B12, and B6; protein, and calories. Comparison of laboratory values revealed significantly lower values for hemoglobin, hematocrit, and zinc. This research illustrates the magnitude of the growth deficits, and nutrient and biochemical deficiencies present in children with EB. The results provide a strong argument for the value of nutritional assessment and intervention and their potential impact in this population. Optimizing autritional status may be one viable method of improving the morbidity and mortality associated with the disease and ultimately improving the overall quality of life.
Follicular Mucinosis of Childhood and Adolescence
Authors: Gibson Lawrence E. , Muller Sigfrid A. , Peters Margot S.
Published: March 2008

Abstract: We cared for nine patients who were less than 21 years old at the time of diagnosis of follicular mucinosis. Four had clearing of the lesions, four did not, and one was lost to follow‐up. Follicular mucinosis, even in childhood, may be persistent and be associated with lymphoma, which can result in death. Because there is no clinical pattern or single histopathologic criterion that correlates with the outcome, clinical follow‐up and assessment of several histopathologic criteria must be relied on when evaluating this condition in children.
Neonatal Job's Syndrome Featuring a Vesicular Eruption
Authors: Kamei Robert , Honig Paul J.
Published: March 2008

Abstract: A new born infant who developed a vesicular eruption, clinically indistinguishable from herpetic lesions, eventually developed the classic features of Job's syndrome. The initial areas of involvement included the hands and feet, then the scalp, face, and suprapubic skin. The clear, tense vesicles varied only slightly in size and appeared as isolated, grouped, or confluent lesions on inflamed skin. Many eventually became umbilicated. The more typical eczematous component appeared over the course of the next several months. Although the child initially had an elevated white blood cell count and eosinophilia, his IgE level did not become dramatically elevated until after 1 year of age. Job's syndrome should be considered as part of the differential diagnosis of a vesicular eruption in the newborn.
Hypohidrotic Ectodermal Dysplasia: A Genealogic, Stereomicroscope, and Scanning Electron Microscope Study
Authors: Norval E. J. G. , van Wyk C. W. , Basson N. J. , Coldrey J.
Published: March 2008

Abstract: This is a report of three patients with hypohidrotic ectodermal dysplasia. or Christ‐Siemens‐Touraine syndrome, their genealogic backgrounds and the stereomicroscope and scanning electron microscopic appearances of the hair, the skin of their fingertips and palms as well as skin studies of members of their families. The skin morphology was recorded by means of silicone monomer rubber impressions and epoxy resin dies. In two of the patients the disease was acquired by X‐linked inheritance, while in the third, a boy. it appeared to follow an autosomal dominant pattern. Defects of the skin of the fingertips and palms of the propositi and members of the families included abnormalities of the morphology and pattern of the epidermal ridges, reduction of sweat pores varying from 13 to 87% of normal, and changed anatomy of the openings of the sweat glands. The openings were shallow and with less whorl‐ing compared to the normal, funnel‐shaped sweat pores. Among the sweat pores, micropores. or openings with an average diameter of 5.3 μm, were observed. One of the propositi and the affected father of another had orifices on their fingertips resembling hair sheaths. Two propositi and the affected father of one exhibited grooving of the hair. The findings confirm the necessity for genealogic investigations in patients with or suspected of having the disease in order to advise parents or prospective parents. They also illustrate the usefulness of stereomicroscopy and scanning electron microscopy in observing skin and hair abnormalities.
Pigmentary Regression in a Giant Nevocellular Nevus: A Case Report and a Review of the Subject
Authors: Zack Lisa D. , Stegmeier Otto , Solomon Lawrence M.
Published: March 2008

Abstract: A giant congenital nevocytic nevus (CNN) on the trunk was followed in a patient from age three months to 23‐years‐oId. During that time the nevus underwent pigmentary regression leaving 2–3 mm lentigines without any evidence of vitiligo or halo formation around the giant CNN. Histopathologi‐cally. nevus cells remained in the dermis.
Two Sisters with Telangiectasia, Spondyloepiphyseal Dysplasia, Hypothyroidism, Neovascularization and Tractional Retinal Detachments: A New Entity?
Authors: Lavin Maureen P. , Olson Judyann C. , Ruttum Mark S. , Esterly Nancy B.
Published: March 2008

Abstract: Two sisters had an unusual constellation of findings, including telangiectasia Involving the face and limbs, unclassifiable spondyloepiphyseal dysplasia, hypothyroidlsm, and tractional retinal detachment preceded by retinal and iris neovascularization. To our knowledge, this combination of defects has not been previously documented in the literature.
Long‐Term Preservation of Permanent Teeth in a Patient with Papillon‐Lefevre Syndrome Treated with Etretinate
Authors: Gelmetti Carlo , Nazzaro Vincenzo , Cerri Danilo , Fracasso Luigi
Published: March 2008

Abstract: Papillon‐Lefevre syndroms (PLS) is a rare autosomal recessive genodermatosis characterized by the association of palmoplantar keratoderma with severe periodontopathy resulting in premature loss of both deciduous and permanent dentition. We treated a patient with PLS with etretinate and followed him for 10 years. Treatment was started at age 9 years, soon after the eruption of secondary dentition. Healing of cutaneous and periodontal lesions was obtained in a few months and maintained during the four years of treatment. Six years after etretinate withdrawal the teeth are still free of periodontal disease and firmly anchored to the alveolar bone. Our case confirms that retinoid therapy can positively influence the development of normal dentition in PLS when it is started during the eruption of the permanent teeth, and suggests that this result can be maintained for a long time even after stopping therapy.
Pemphigoid in Children
Authors: Oranje Arnold P. , Joost Theodoor van
Published: March 2008

Abstract: Pemphigoid is an autolmmune blistering disease that is rare in childhood. A review of the English, German, and French literature published prior to 1989 revealed 31 cases of juvenile bullous pemphigoid and 12 of other forms (10 cases of juvenile cicatricial and 2 of juvenile localized bullous pemphigoid). Childhood pemphigoid, although less frequent, seems no different than its adult counterpart. While oral lesions are more common in the juvenile bullous form, there is no association with malignancy, which is a controversial and presumably incorrectly assumed association in the adult disease. The prognosis for children is good in most cases, and the disease is self‐limiting.

The mainstay of therapy has been oral corticosteroids, but dapsone also often produces a good response. A relatively new approach is to give a combination of erythromycin and niacinamide with dapsone for a steroid‐sparing effect. In some patients the response to erythromycin plus niacinamide alone has been satisfactory; however, controlled therapeutic trials are lacking.

It has been stated that high doses of steroids and immunosuppressive drugs are indicated for the cicatricial, as opposed to juvenile, bullous pemphigoid. From this review of the few cases of childhood cicatricial disease, it seems that the therapeutic approach might be the same for both forms.
Diaper Performance: Maintenance of Healthy Skin
Authors: Wilson Patricia A. , Dallas Merry Jo
Published: March 2008

Abstract: Skin wetness is proportional to diaper wetness, and with increased skin wetness, the potential for diaper dermatitis is increased. This study evaluated a wide range of infant diaper products. Eighty healthy volunteers wore 2‐inch wetted diaper patches on their volar forearm for two hours. An evaporimeter was used to measure excess skin wetness attributed to the patches. The amount of moisture retained in the patch was also calculated. We found that superabsorbent (SA) disposable diapers kept the skin drier and retained more synthetic urine than cloth reusable and conventional disposable brands, and thus have the greatest potential for helping prevent diaper dermatitis. The SA brands evaluated did an equally adequate job in keeping the skin dry and in retaining moisture. Conventional disposable diapers were less able to keep the skin dry than SA diapers and were not superior to cloth products in most instances. Cloth diapers” performance depended on their composition. Those with several layers of the same fabric were less successful in keeping the skin dry than ones that contained middle layers of different nonwoven components.
Giant Juvenile Xanthogranuloma: Exophytic and Endophytic Variants
Authors: Resnick Steven D. , Woosley John , Azizkhan Richard G
Published: March 2008

Abstract: Xanthogranulomas larger than 2 cm are rarely reported variants of this otherwise common, benign lesion usually measuring 1 mm to 1 cm in diameter. The natural history of such macronodular xanthogranulomas is uncertain, but appears to parallel the generally predictable course toward involution seen in micronodular lesions. Two patients with macronodular xanthogranuloma illustrate exophytic and endophytic variants, as well as the problems of managing this unusual tumor. Nonoperative observation is advisable if possible, but complicating factors may necessitate surgical excision in some cases.
Congenital Midline Hamartoma: Case Report with Histochemical and Immunohistochemical Findings
Authors: Elgart George W. , Patterson James W.
Published: March 2008

Abstract: We report the case of a polypoid nodule on the chin of an infant. Microscopically, the lesion featured numerous pilosebaceous units, eccrine sweat glands, arrectores pilorum muscles, mature adipose tissue, and prominent admixtures of skeletal muscle. This lesion has much in common with the accessory tragus, and is similar to the one reported earlier as rhabdomyomatous mesenchymal hamartoma. We feel the more general term congenital midline hamartoma is preferable, as it encompasses both the clinical and microscopic features of the lesion.
Dysplastic Nevi in Children
Authors: Rothman Karen F. , Esteriy Nancy B. , Williams Mary L. , Sagebiel Richard S. , Solomon Alvin R. , Spraker Mary K. , Roth Monique E. , Grant‐Kels Jane M. , Burgdorf Walter H. C. , From Lynn , Rogers Maureen , Rhodes Arthur R. , Koh Howard K. , Bhawan Jag , Grin Caron , Kopf Alfred W.
Published: March 2008

Issues surrounding the diagnosis and management of the dysplastic nevus have sparked considerable controversy in the dermatologic literature. Despite the great amount of attention to this subject, little has been written about the prob‐lem as it relates to the pediatric patient. For this reason, we formulated a set of questions that we thought needed to be addressed, and sought answers from several dermatologists. The replies largely reflect personal opinions, as few data are available on which to base a standard approach to these lesions.
Quantitative Assessment of Scanning Electron Microscope Defects in Uncombable‐Hair Syndrome
Authors: Rest Ellen B. , Fretzin David F.
Published: March 2008

Abstract: Uncombable‐hair syndrome is a rare disorder of scalp hair that starts early in childhood. It is characterized clinically as unruly hair and by scanning electron microscopy as longitudinally grooved hairs with a triangular cross section. Because of the controversy of inheritance and prior reports of similar changes in normal and other hair disorders, we performed a quantitative scanning electron microscopic assessment of four patients with uncombable‐hair syndrome, first‐degree relatives, and nonaffected controls. Our results indicate that longitudinal grooving of scalp hair is a common occurrence, and that for clinical changes to be apparent, approximately 50% of hairs must be affected.
Occurrence of Hereditary Bullous Epidermolyses in Croatia
Authors: Pavičić Željko , Kmet‐Vižintin Paula , Kansky Aleksej , Dobrić Ivan
Published: March 2008

Abstract: To determine the occurrence of hereditary bullous epidermolyses (EB) in Croatia, Yugoslavia, from 1960 to 1987, cases were gathered from the hospital files of dermatologic and pediatric clinics and departments throughout the area. The diagnosis of EB type was made on the basis of clinical features, patients' histories, and light microscopy and electron microscopy findings. Fifty families with 58 patients were registered; 44 patients were examined personally by one of the authors. The most frequent type of EB in Croatia was recessive dystrophic EB Hallopeau‐Siemens, occurring in 35 of the 58 individuals. Regional accumulation of cases within the Varaždin area was noted (13 patients). Prevalence of EB in Croatia is 0.956 cases per 100,000 inhabitants. One case of recessive dystrophic EB Hallopeau‐Siemens occurred in about every 52,000 live births.
Hair Casts: A Clinical and Electron Microscopic Study
Authors: Zhu Wen‐Yuan , Xia Ming‐Yu , Wu Jian‐Hua , Do De‐An
Published: March 2008

Abstract: Hair casts were seen in 22 girls ranging in age from 4 to 13 years. They were localized to the frontal, vertex, and temporal areas. The number of affected hairs varied from one‐tenth to one‐third. Twenty of the 22 girls styled their hair in ponytails or pigtails. Compound root sheath casts were present in six of nine patients using 1% 4‐dimethylaminocinamaldehyde stain. A mass of large polymorphous scales and solitary or clumped spores was seen on the surface of hair casts using scanning and transmission electron microscopy. Energy‐dispersive x‐ray microanalysis showed that elements of silicon, aluminum, and molybdenum were uniquely present in hair casts. We assume that tightly drawn braids, by causing local circulatory disturbances and inflammation of the scalp, may be one etiologic factor in the disorder.
Focal Dermal Hypoplasia with an Initial Inflammatory Phase
Authors: Mann Michael , Weintraub Rosalyn , Hashimoto Ken
Published: March 2008

Abstract: We report a case of focal dermal hypoplasia with an initial inflammatory phase. Although our patient had dark red macules and papules and red linear atrophic lesions at birth, her lesions were somewhat faded at 3 months of age and had become yellowish before 5 months of age. Histologic examination of lesions at 3 months of age showed marked edema in the papillary dermis, mononuclear cell infiltration, a prominent fibroblastic population, and a large cluster of dermal lipocytes, suggesting an association between the inflammation and the development of dermal adipose tissue.
Solitary Langerhans Cell Histiocytoma: An Unusual Form of Hashimoto‐Pritzker Histiocytosis?
Authors: Masouyé I. , Chavaz P. , Salomon D. , Balderer J. , Carraux P. , Saurat J.‐H.
Published: March 2008

Abstract: A 2‐month‐old girt acutely developed a brownish, firm nodule on the left thigh. The lesion was surgically removed when the patient was age 3 months, and histology showed a massive dermal infiltration by large histiocytic cells with abundant ground glass cytoplasm. Most cells were S100 protein positive. Electron microscopic examination revealed the presence of Birbeck granules in about 30% of the histiocytes, as well as laminated dense bodies and wormlike bodies. No recurrence was observed during follow‐up for three years. We believe that this case, like the one previously reported, represents a clinical variant of Hashimoto‐Pritzker disease.
Papular and Papulovesicular Acrolocated Eruptions and Viral Infections
Authors: Patrizi Annalisa , Lernia Vito Di , Ricci Giampaolo , Masi Massimo , Varotti Claudio
Published: March 2008

Twenty–six children with a symmetric papular or papulovesicular acrolocated eruption were studied to evaluate a possible link with viral infection. A history of a recent immunization was present in four patients, two of whom had a polio vaccine virus isolated from their stool samples. There was serologic evidence of a recent Epstein–Barr virus infection in one patient, adenovirus infection in another, a concurrent Epstein–Barr virus and adenovirus Infection in a third, and cytomegalovirus infection in a fourth. Viral cultures were positive in two patients for adenovirus and in two others for rotavirus. Although some of the virologlc findings could have been coincidentai, this study confirms that multiple virai infections can be reiated to most papular and papulovesicular acrolocated eruptions of chiidhood. Virologic investigations in the eariier phases of the eruption are recommended to identify the etiologic factors of these lesions.
A Lichen Striatuslike Eruption Adopting the Morphology of Blaschko Lines
Authors: Jordá Esperanza , Zayas Ana Isabel , Revert Angeles , Donnini Amparo , Ramón Dolores , Torres Vicente
Published: March 2008

Abstract: A 15‐month‐old girl had orange papules that formed V‐shaped lines on her back. The clinical evolution and histology were compatible with the diagnosis of a lichen striatus‐like eruption, adopting a special morphology by following Blaschko lines.
Immunologic Markers of Systemic Scleroderma in Children
Authors: Błaszczyk Maria , Jabfołnska Stefania , Szymańska‐Jagiełło Wanda , Jarzabek‐Chorzelska Maria , Chorzelski Tadeusz
Published: March 2008

Abstract: This study was performed on seven children with systemic scleroderma, three with the diffuse and four with the limited type. All three patients with diffuse scleroderma had high titers of clumpy pattern antinucleolar antibody on HEp‐2 cells. The course of the disease was severe, and two children died. Four children with limited scleroderma had mild disease, and Scl‐70 antibody, an immunologic marker that in adults is associated mostly with diffuse scleroderma. In one child Scl‐70 antibody and anticentromere antibody coexisted, although previously the two were believed to be mutually exclusive. This study shows that limited scleroderma of childhood with slight cutaneous involvement may be associated with the Scl‐70 marker. The findings in 10 adults in whom Raynaud's phenomenon developed in childhood and indurations appeared some years later, point to the sinificance of careful observation of these children, with repeated testing for immunologic markers of SSc. An important new finding is the association of different types of systemic sclerodermas with specific immunologic markers.
Eosinophilic Pustular Folliculitis (Ofuji Disease) in Childhood: A Review of Four Cases
Authors: Giard Françoise , Marcoux Danielle , McCuaig Catherine , Powell Julie , Russo Pierre
Published: March 2008

Abstract: Four children under 2 years of age were treated at our hospital in the last three years with a recurrent vesiculopustular eruption of the scalp, variably extending to the face and limbs. All cases followed a cyclical pattern. Three of the children had a moderate response to topical steroids, and one went into remission after a two‐month course of erythromycin. Ethnic origin may be an important predisposing factor. Histologically, all patients showed a moderate mixed inflammatory infiltrate with numerous eosinophils centered around hair follicles. Peripheral white blood cell count showing leukocytosis with eosinophilia was observed in those cases measured, but no consistent immunologic abnormalities could be identified.
Progressive Early Dermatologic Changes in Hutchinson‐Gilford Progeria Syndrome
Authors: Gillar Patty J. , Kaye Celia I. , McCourt James W.
Published: March 2008

Abstract: We describe evolving dermatologic findings in a male with progeria from age 1 month to 21.5 months. At 18 months of age, irregular pigmentary changes of the abdomen, early occipital aiopecia, superficial scalp veins, glyphic nasal tip, absent ear lobules, coarse hair that stands on end, crowded dentition with delayed tooth development, and dystrophic nails permitted the diagnosis of progeria. Radiographs showed evidence of resorption of the distal ends of the clavicles, attenuation of the terminal phalanges, diffuse osteopenia, and fishmouth vertebral bodies, which are typical of this syndrome. Appreciation of the evolution of early dermatologic findings may permit earlier diagnosis of this condition in infants with skin changes.