bluuebunny/crossref_metadata_2025 huggingface dataset:
Pseudorheumatoid Nodule (Deep Granuloma Annulare) of Childhood: Clinicopathologic Features of Twenty Patients
Authors: Evans Margaret J. , Blessing Karen , Gray Elizabeth S.
Published: March 2008

Abstract: Pseudorheumatold nodule of childhood is seen uncommonly by pathologists and is thought to be a variant of granuloma annoIan. We report a series Of 20 patients (13 M, 7F)age1 to 17 years. Fourteen lesions arose on the front of the tower limb, and 14 children had numerous lesions at diagnosis. Seven developed some form of recurrence, except for one case occurring at the site of the original lesion. Only one patient had a weakly positive rheumatoid factor, and was the patient with remote recurrence. As a significant number of lesions arose around the foot when the child was beginning to walk or starting to wear shoes, It la suggested that trauma plays a role in the development of some of these lesions.
Aquagenic Urticaria in a Child
Authors: Wasserman Dror , Preminger Aviya , Zlotogorski Abraham
Published: March 2008

Abstract: A healthy 3‐year‐old boy daveloped acute urticaria five minutes after exposure to tap water In different areas of Jerusalem. Physical examination and baseline laboratory evaluation were within normal limits. The clinical picture and possible mechanisms of aquagenic urticaria are reviewed. This is the youngest patient to be reported.
Buschke‐Ollendorff Syndrome, Otosclerosis, and Congenital Spinal Stenosis
Authors: Schnur Rhonda E. , Grace Kimberly , Herzberg Arlene
Published: March 2008

Abstract: We report a family with the Buschke‐Oltendorlf syndrome and several unusual features, Including one individual wtth congenital spinal stenosis and another with hearing loss, probably due to otosclerosia. Other reported abnormalities associated with this syndrome, including otosclerosis, are reviewed.
Recurrent Pseudomonas Folliculitis
Authors: Trüeb Ralph M. , Gloor Marianne , Wüthrich Brunello
Published: March 2008

Abstract: Pseudomonas aeruginosa has attracted the attention of the dermatotogic literature through repeatedly reported outbreaks of folliculitis occurring in small epidemics related to contaminated whirlpools, hot tubs, and swimming pools. In contrast, sporadic cases without these recreational exposures may present a diagnostic challenge. Two sisters, age 8 and 4 years, had sporadic Pseudovnonas folliculitis. It is important to recognize the organism as the cause of this recurrent, papulopustubar, follicular eruption on the trunk and gluteal regions, also called nonrecreational Pseudomanas folliculitis, to avoid misdiagnosis and mismanagement. Inadvertent therapy with steroids may result in rapid spread of the lesions. Unless a meticulous Investigation of contaminant sources in the environment leads to the detection of the vehicle of infection, the dermatitis tends to recur and become chronic.
Asymmetric Periflexural Exanthem of Childhood: Report of Two New Cases
Authors: Gelmetti Carlo , Grimalt Ramon , Cambiaghi Stefano , Caputo Ruggero
Published: March 2008

Abstract: A newly described exanthem of infancy consists of an eczematous or scarlatinltorm eruption that starts initially from one axillary fold and spreads unilaterally and centrifugally on the trunk and the proximal part of the upper limb. Minor lesions are infrequently present on the contralaterai side. The rash may be slightly pruritic and sometimes associated with a moderate regional lymphadenopathy. The appearance in small epidemics and the seasonal prevalence imply a viral agent as the probable origin. We report two new cases of this entity, which appears to be not so rare.
Keratosis Lichenoides Chronica in a Child
Authors: Torrelo Antonio , Mediero Imelda G. , Zambrano Antonio
Published: March 2008

Abstract: A young boy developed childhood keratosis lichenoides chronica. The eruption cleared spontaneously after 13 years. This is a rare condition, regarded by some as a variant of lichen planus. The great preponderance of cases occur in adults, whereas the disease in childhood is uncommon.
Treatment of Severe Alopecia Areata with Squaric Acid Dibutylester in Pediatric Patients
Authors: Orecchia Giovanni , Malagoli Pierglorgio , Santagostino Laura
Published: March 2008

Abstract: Topical squaric dibutylester (SADBE) is currently used in Europe to treat alopecia areata In adults. We attempted to determine this drug's effectiveness and convenience in treating children with severe aiopecia areata who are psychologically disturbed and resistant to other therapies. Twenty‐eight children under 13 years of age who had extensive and Song‐standing alopecia areata and were not responsive to conventional therapies were sensitized on the head with 2% SADBE in acetone and treated with weekly applications for 12 months. Nine patients (32.1%) achieved complete or cosmeticaliy acceptable regrowth, and another six (21.4%) had significant regrowth. No correlation was found between response and sex, age of onset of illness, extent, duration, or clinical type of disease. In 14 patients followed for a period ranging from 18 months to 8 years SADBE remained efficacious for reiapses as weSi. This study demonstrates that SADBE is a valid and suitable treatment for children, particuiarly those who are resistant to conventional therapies.
Treatment of Kerions
Authors: Honig Paul J. , Caputo Grace L. , Leyden James J. , McGinley Kenneth , Selbst Steven M. , McGravey Ann R.
Published: March 2008

Abstract: Therapy for kerlons was evaluated by randomly assigning 30 patents to one of four treatment groups: group A griseofulvin, group B grlssofulvln plus erythromycin, group C griseofulvin plus prednisone, and group D griseofulvin, erythromydn, and prednisone. Data lndicate that antibiotic and steroid therapy. In addition to grlseotulvln, may reduce scaling and pruritus, but does not reduce the time It takes for kerions to flatten.
Impetigo: An Overview
Authors: Darmstadt Gary L. , Lane Alfred T.
Published: March 2008

Abstract: This article reviews in detail the pathogenesis, clinical characteristics and management of impetigo in children. Impetigo is the most common bacterial skin infection of children. Most cases of nonbullous impetigo and all cases of bullous impetigo are caused by Staphylocaccus aureus. The remainder of cases of nonbullous impetigo are due to group A beta hemolytic streptococci (GABHS). GABHS colonize the skin directly by binding to sites on f ibronectin that are exposed by trauma. In contrast, S. aureus colonizes the nasal epithelium first; from this reservoir, colonization of the skin occurs. Patients with recurrent impetigo should be evaluated for carriage of S. aureus. Superficial, localized impetigo may be treated successfully in more than 90% of cases with topical application of mupirocin ointment. Impetigo that is widespread or involves deeper tissues should be treated with a beta‐lactamase‐resistant oral antibiotic. The choice of antibiotics is affected by the local prevalence of resistance to erythromycin among strains of S. aureus, antibiotic cost and availability, and issues of compliance.
Sun Exposure of Young Children While at Day Care
Authors: Grin Caron M. , Pennoyer Jennifer W. , Lehrich Deborah A. , Grant‐Kels Jane M.
Published: March 2008

Abstract: Sun exposure in childhood has been implicated as a risk factor for the development of melanoma and nonmelanoma skin cancers. As an increasing number of young children are cared for in day‐care centers, we were interested In examining the sun‐protection practices in this setting. In our study of day‐care centers, we found that while most day‐care center staff were aware of the adverse effect of excess sun exposure and the need for sun protection, the use of sunscreen and protective clothing and avoidance of midday sun were limited. We conclude that intensive education of day‐care center staff and parents regarding sun exposure and sun protection is necessary if we are to attempt to reduce the frequency of melanoma and nonmelanoma skin cancer.
Limited Joint Mobility and Lipodystrophy in Children and Adolescents with Insulin‐Dependent Diabetes Mellitus
Authors: Kakourou Talia , Dacou‐Voetetakis Catherine , Kavadias G. , Bakoula Chrysa , Aroni Kyriaki
Published: March 2008

Abstract: One hundred forty‐eight children, aged 11.9 ± 3.7 years, who had insulin‐dependent diabetes melHtus (IDDM) for 4.5 ± 3.7 years and glycosylated Hb values (HbA1) of 10.5%± 4.5%, were examined for limited joint mobility (LJM) and lipodystrophy. En all diabetics, human biosynthetic insulin was used. Six hundred forty‐eight pupils, aged 11.8 ± 2.5 years, served as controls. LJM was found in 28.4% of the diabetics and in 7.5% of the controls (p < 0.001). The presence and severity of LJM was positively correlated with the duration of diabetes and negatively with height. There was no correlation between the presence of LJM and sex, chronological age, age of diabetes onset, HbA1 values, or retinopathy. Lipodystrophy at insulin injection sites was found in 37.1% of the diabetics. Hypertrophic lesions predominated. Our findings and those of the literature lead to the following conclusions: the prevalence of LJM in EDDM subjects of comparable age using Rosenbloom's criteria is almost uniform in all published studies (28%); the correlation, however, of prevalence and severity to different parameters presents discrepancies which cannot be interpreted at present. Human biosynthetic insulin does not protect from lipodystrophy and alterations of insulin injections still remain the only currently available preventive and therapeutic measure.
Palmar Purpura: An Atypical Presentation of Childhood Dermatitis Herpetiformis
Authors: McGovern Thomas W. , Bennion Scott D.
Published: March 2008

Abstract: Dermatitis herpetiformis (DH) is seen most commonly as a pruritic, papulovesicular eruption in young children or adolescents. Differentiation from other bullous diseases of childhood may be difficult. We report the first case of an adolescent in whom pruritic, palmar, purpuric macules and papules were the only manifestations of DH. The patient later developed typical vesiculobullous extensor lesions and symptomatic gluten‐sensitive enteropathy (GSE). All lesions and GSE symptoms resolved with dapsone and a gluten‐free diet. Our purpose is to illustrate an unusual presentation of pediatric DH.
Bilateral Large‐Cell Calcifying Sertoli Cell Tumor of the Testes with Peutz‐Jeghers Syndrome: A Case Report
Authors: Dreyer Leonora , Jacyk Witold K. , du Plessis Dion J.
Published: March 2008

Abstract: A 13‐year‐old boy with Peutz‐Jeghers syndrome (PJs), gyne‐comastia, and bilateral multifocal testicular tumors is described. Histology of the testicular tissue disclosed large‐cell calcifying Sertoli cell tumors. Females wHBi PJs are known to be at increased risk of developing gonadal tumors. This case and a review of other reported cases suggest that mates with PJs are also at risk for developing gonadal tumors.
Bacillary Angiomatosis in an Immunocompetent Child: The First Reported Case
Authors: Paul Maria A. , Fleischer Alan B. , Wieselthier Janet S. , White Wain L.
Published: March 2008

Abstract: Bacillary angiomatosis, an infectious process associated with Rochalima spp., was thought until recently to be restricted to HIV‐infected or otherwise immunosuppressed patients. In 1993, bacillary angiomatosis was reported in several immunocompetent adults. An extensive literature review failed to find references to bacillary angiomatosis in immunocompetent children. We describe a 6‐year‐old female who presented with a single, rapidly growing, friable, erythematous papule on her neck. Histologic examination of a biopsy specimen confirmed the diagnosis of bacillary angiomatosis. The patient was otherwise healthy, and her physical examination was normal. Laboratory studies, including HIV serology, were normal. The patient was treated with six weeks of oral erythromycin without evidence of recurrence. We present and discuss the implications of the first case of bacillary angiomatosis in an immunocompetent child.
Clinical Analysis of 40 Cases of childhood‐Onset Behçet's Disease
Authors: Kim Dong‐Kun , Chang Sung Nam , Bang Dongsik , Lee Eun‐So , Lee Sungnack
Published: March 2008

Abstract: Behçet's disease is a chronic, relapsing, idiopathic, multisystem condition characterized by mucocutaneous, ocular, gastrointestinal, articular, vascular, urogenital, and neurologic involvement. Usually the onset occurs between 20 and 30 years of age; it is very uncommon in children. We analyzed 40cases of childhood‐onset Behçet's disease. The male: female ratio was 0·67 the most frequent major sign was oral ulceration, appearing inall patients. Other major sings were genital ulcers (33 patients) skin lesions (29), and ocular lesions (11). the average time interval between the initial oral ulceration and the second major manifestation, the third and fourth features rapidly developed within one to two years. The most frequent minor sign was arthritis, occurring in 11of 40 patients. Intestinal, neurologic, and renal involvement was also present. From our results, we concluded that oral ulceration, which is the most common initia manifestation, should not be neglected in children, since it may signal Behçet's disease.
Further Experience With Pemphigus in Children
Authors: Kanwar Amrinder J. , Dhar Sandipan , Kaur Surrinder
Published: March 2008

Abstract: Seven children had pemphigus: six had pemphigus vulgaris and one pemphigus foliaceus. Four who had pemphigus vulgaris were administered dexamethasone pulses. Follow‐up from six months to one year was uneventful; the disease was under control and there were no complications due to therapy. Our observations suggest that the clinical features, course, and principles of therapy for pemphigus in children are essentially the same as those in adults.
Melanocytic Nevi in Turner Syndrome
Authors: Becker Brad , Jospe Nicholas , Goldsmith Lowell A.
Published: March 2008

Abstract: One morphologic feature of Turner syndrome is increased number of melanocytic nevi; however, little attention has been given to their characterization. The development of a melanoma in one of our patients with Turner syndrome prompted this study. We prospectively examined 10 patients with the disease, confirmed by karyotype. all patients underwent full body skin examination noting the number, size distribution, and degree of clinical atypia of melanocytic nevi. Representative and unusual lesions were photographed. An average of 115 nevi were seen, with the majority measuring 1 to 5 mm. Most were located on the back and extremities. Clinical atypia was uncommon. our patients had larger numbers of benign‐appearing nevi than the general population. Large numbers of melanocytic nevi is a risk factor for melanoma, suggesting that these patients have an increase in one risk factor. Longitudinal studies are indicated to clarify this issue; nevertheless, we recommend periodic skin examinations and the regular use of sunscreens for individuals with Turner syndrome.
Hereditary Mucoepithelial Dysplasia
Authors: Rogers Maureen , Kourt Gina , Cameron Angus
Published: March 2008

Abstract: We report an individual with the rare autosomal recessive condition known as hereditary mucoepithelial dysplasia. The diagnosis was based on clinical features and characteristic ultrastructural changes in oral mucosal biopsy specimens.
Parapsoriasis in Two Children: A Clinical, Immunophenotypic, and Immunogenotypic Study
Authors: Menno Silvano , Piccinno Roberta , Crosti Lucia , Berti Emilio
Published: March 2008

Abstract: Parapsoriasis has been described rarely in childhood; a few reports mention this condition as preceding early mycosis fungoldes. We report a boy age 15 and a girl age 8 years with different clinical features of parapsoriasis who were followed clinically and histologically for about four years. Immunophenotyping of the skin infiltrate and a T cell receptor gene rearrangement analysis were performed on each. The infiltrate was composed mainly of CD4+ and CD45+ lymphocytes in the first patient and of CD8+ and CD45+ lymphocytes in the first patient and of CD8+ and CD45+ in the second. No T cell receptor gene rearrangements were found. The paucity of knowledge about the evolution of this entity in childhood and its relationship to mycosis fungoides makes follow‐up critical. The importance of immunophenotyping and immuno‐genotyping is particularly stressed.
Persistent Erythema Infectiosum‐Like Rash as a Prodrome of Acute Lymphocytic Leukemia
Authors: Sahn Eleanor E. , Bluestein Etta Leah , Oliva Stepan
Published: March 2008

Abstract: Lichen sclerosus et atrophicus (LSA) is uncommonly reported in family members and is seen occasionally in young children. We report genital LSA in two young sisters who were diagnosed a year apart. The English language literature on familial LSA in childhood is reviewed and discussed.
Juvenile Basal Cell Carcinoma
Authors: Price Mark A. , Goldberg Leonard H. , Levy Moise L.
Published: March 2008

Abstract: A 17‐year‐old youth had a solitary basal cell carcinoma of the nose.This malignancy is rare in children and usually occurs withanother disease or syndrome. The de novo form in this patient is especially rare.
Risk of Malignant Transformation of Congenital Melanocytic Nevi in Blacks
Authors: Shpall Steven , Frieden Ilona , Chesney Margaret , Newman Thomas
Published: March 2008

Abstract: The risk of small congenital melanocytic nevi (CMN) developing into melanoma is not known, but Is highly controversial. The frequency of small CMN is, paradoxically, slightly higher in some populations, such as blacks, who are at a lower risk of developing melanoma than whites. An estimate of the risk of malignant transformation of CMN in such a low‐risk population could help In the management of congenital nevi in these patients and might also shed light on the inherent malignant fisk of small CMN. We used a national population‐based cancer registry, the Surveillance, Epidemiology, and End Results program (SEER), and the incidence of CMN in blacks taken from published newborn surveys to calculate a risk of malignant transformation. We calculated a maximum risk using a model based on a worst‐case scenario, assuming that all melanomas on glabrous skin arise in CMN. We also calculated a modified risk based on the known historical association of nevi and melanomas in blacks, and estimates of the histologic association of the two. The cumulative maximum risk of malignant transformation in blacks to age 75 years was 1 in 164. It was strongly age dependent, with the majority occurring in persons over age 45. The estimated maximum risk before age 15 was less than 1 in 10,000 CMN, and in blacks age 15 to 35 less than 1 in 3700 CMN. The modified risk suggests that the worst‐case scenario overestimates the risk by at least a factor of 12, making the actual risk in blacks up to age 75 approximately 1 In 2000. Both the worst‐case scenario model and the modified risk suggest a small to extremely small risk of malignant transformation, particularly before age 45. Although the specific morphology and circumstances of a particular CMN must be considered In decision making, we believe that routine prophylactic removal of small CMN in blacks is probably not warranted.
Sweet Syndrome as the Presenting Manifestation of Chronic Granulomatous Disease in an Infant
Authors: Sedel D. , Huguet P. , Lebbe C. , Donadieu J. , Odièvre M. , Labrune Ph.
Published: March 2008

Abstract: A 2.5‐month‐old infant had Sweet syndrome. Chronic granulomatous disease was subsequently diagnosed by the nitroblue tetrazolium reduction test. To date, this infant is the youngest reported with Sweet syndrome. Moreover, the association of chronic granulomatous disease with this syndrome has not been previously described. The precise relationship between the conditions remains to be determined. Granulocyte function should be evaluated in any infant with Sweet syndrome.
Agminated Lentiginosis: Case Report and Review of the Literature
Authors: Micali Giuseppe , Nasca Maria Rita , Innocenzi Daniele , Lembo Dina
Published: March 2008

Abstract: Agminated lentiglnosis (AL) is characterized by numerous lentfgines confined to a body segment, with a sharp demarcation at the midline. So far, only 13 cases have been reported in the literature. We report a 30‐year‐old woman with a multifocal AL The cutaneous lesions first appeared at 5 years of age and gradually increased in number with time. Clinically, they appeared as numerous brown macules, ranging in size from 1 to 5 mm in diameter, in a peppered distribution over an area extending bilaterally on the neck, chin, and cheeks. On the trunk the lesions were localized to the left shoulder and breast, involving the axilla and upper part of the abdomen down to the umbilicus. Four cafe au lait macules ranging in diameter from 10 to 15 mm were present, with no evidence of neuroflbromas. Histopathology of a macute showed the features of lentigo; in addition, groupings of melanocytes were observed at the dermoepidermal junction. The differential diagnosis of AL includes speckled lentiginous nevus and segmental neurofibromatosis.
Selective Decrease of Eumelanin in Hypopigmented Epidermis of Hypomelanosis of Ito
Authors: Fukai, K. , Ishii, M. , Wakamatsu, K. , Ito, S. , Sakamoto, H. , Fujiwara, H. , Ohno, A. , Hamada, T.
Published: March 2008

Abstract: A Japanese infant had bilateral hypopigmented macules in a whorled or marble‐cakelike configuration on her trunk and extremities. Her irides were bluish gray and her hair was dark brown. She had photophobia and macrocephaly with developmental delay. Analysis of peripheral lymphocytes and dermal fibroblasts disclosed a normal chromosomal pattern. A new finding was that the eumelanin content of the hypopigmented epidermis was decreased. We suggest that at least a portion of patients with this disorder have a defect in eumelanogenesis in hypopigmented skin.
Neonatal Scabies Treated With Permethrin 5% Cream
Authors: Quarterman Mark J. , Lesher Jack L.
Published: March 2008

Abstract: Scabies in neonates and infants has a clinical pattern that may be easily misdiagnosed. The eruption is generalized, including involvement of the head, neck, face, palms, and soles, with an early tendency to pustule formation. Other lesions include papules, vesicles, and burrows, which may be obliterated by secondary lesions. A confusing clinical picture resulted in a delay in diagnosing scabies in a 23‐day‐old infant who was successfully treated with permethrin 5% cream.
Not All Hemangiomas Look Like Strawberries: Uncommon Presentations of the Most Common Tumor of Infancy
Authors: Martinez‐Perez Dolores , Fein Neil A. , Boon Laurence M. , Mulliken John B.
Published: March 2008

Abstract: The typical appearance of cutaneous hemangiomas of infancy is well known. We studied unusual manifestations of this common tumor. We reviewed over 500 hemangiomas in the registry of the Vascular Anomalies Program at Boston Children's Hospital. We found four uncommon morphologic variations: deep hemangiomas with normal overlying skin (n = 12); macular hemangiomas with a port‐wine stainlike appearance (n = 6); bossed hemangiomas with telangiectasia and peripheral patlor (n = 5); and hemangiomas with persistent fast flow (n = 3). Deep and superficial (macular) varieties regressed at a normal rate. Telangiectatic (bossed) hemangiomas, however, invotuted rapidly, usualiy before 1 year of age. Hemangiomas with persistent fast flow required either resection or sclerotherapy for complications in early childhood.
The Frequency of Erythromycin‐Resistant Staphylococcus aureus in Impetiginized Dermatoses
Authors: Misko Monica L. , Terracina Joseph R. , Diven Dayna G.
Published: March 2008

Abstract: A trend toward increasing resistance of Staphylococcus aureus to Standard antibiotic therapy has been reported. Specimens were taken from 98 patients in our outpatient staff clinic who had clinical signs of superficial skin infections. Patients with erythromycin‐resistant 5. aureus were contacted by telephone or seen in clinic. The organism was found in 87% of patients. Twenty‐two (26%) of the 85 cultures that grew 5. aureus were resistant to erythromycin. Treatment failure occurred in one of these patients. We conclude that S. aureus is the most common causative organism in secondary skin infections and impetigo in our patient popuiation. Despite significant erythromycin resistance, there was a low frequency of treatment faiture in this group, Erythromycin may still be a reasonable agent in the treatment of uncomplicated superficial skin infections in our community at this time.
Eosinophilic Panniculitis
Authors: Samlaska Curt P. , de Lorimier Arthur J. , Heldman Leslie S.
Published: March 2008

Abstract: Eosinophilic pannicuiitis is a pooriy defined entity with variabie ciinicai features. We report a case of rapidiy eniarging, asymptomatic subcutaneous scalp nodules in a 6‐year‐old biack boy with atopic dermatitis. The noduies resolved spontaneously over two to three days. Biopsy specimens were remarkable for eosinophilic pannicuiitis without evidence of epidermal change or vasculltis. We beiieve that this is the youngest reported patient with this disorder.
Fixed Drug Eruption on the Tongue of a 4‐Year‐Old Boy
Authors: Dhar Sandipan , Kanwar Amrinder J.
Published: March 2008

Abstract: A 4‐year‐old boy had a rare fixed drug eruption due to amoxicillin and confined to the tongue. Avoidance of the drug resuJted in complete clearance within three months.
Incontinentia Pigmenti: Late Sequelae and Genotypic Diagnosis: A Three‐Generation Study of Four Patients
Authors: Dutheil P. , Vabres P. , Cayla M. C. Hors , Enjolras O.
Published: March 2008

Abstract: Late cutaneous signs of incontinentia pigmenti (IP) are often subtle and misdiagnosed. We focus on these somewhat confusing clinical markers in a family, and on the genotypic diagnosis based on DNA analysis. An infant was born with a typical IP rash. Dermatologic examination of the women In her family revealed that her mother, her maternal aunt, and her grandmother had subtle skin signs reminiscent of IP. The four family members proved to be Informative for DNA markers in the Xq28 region. Familial cases of IP are sometimes missed due to the lack of recognition of some late skin signs that are not always hyperpigmented streaks. Subtle, faint, hypochromic or atrophic lesions in a linear pattern may occur. Thus an accurate diagnosis of the women in a particular family also requires anamnestic data and recognition of extracutaneous anomalies. When a clinical diagnosis has been made, DNA marker analysis allows us to offer a prenatal diagnosis with minimal risk of error In case of further pregnancy. However, early testing of chorionic villus samples does not allow one to predict the severity of the disease in an affected fetus.
Asymmetric Periflexural Exanthem of Childhood and Viral Examinations
Authors: Harangi Ferenc , Várszegi Dalma , Szücs György
Published: March 2008

Abstract: We report on an asymmetric maculopapular exanthem of early childhood. One hundred eighty seven patients (111 girls, 76 boys) were examined over a four‐year period, mainly in winter and spring. The first skin lesions appeared on the trunk or in the large flexures of the limbs. They spread centrifugally, often to the contralateral side, over two to four weeks, and disappeared with slight Itching and moderate desquamation within another two to four weeks. Virologic studies were carried out in 34 cases; parainfluenzavirus 2, parainfluenzavirus 3, and adenoviral infection were diagnosed in two patients each. The chances of a causal relation between the viral infection and the skin symptoms were slight; only an incidental connection is likely to exist between the skin disorders and the viral infection.
Localized Linear Bullous Eruption of Systemic Lupus Erythematosus in a Child
Authors: Roholt Natalie S. , Lapiere Jean Christophe , Wang Jining I. , Bernstein Leonard J. , Woodley David T. , Eramo Lynne R.
Published: March 2008

Abstract: A 9‐year‐old girl newly diagnosed with systemic lupus erythematosus (SLE) developed a localized linear papulovesicular eruption over the right dorsal hand and ulnar forearm. The skin findings were clinically suggestive of herpes zoster, lichen striatus, or lichen planus‐lupus erythematosus overlap. However, histologic, immunofluorescent, Immunoelectron microscopic, and immunobiot studies revealed findings compatible with bullous SLE. Our patient is noteworthy because she is the first one reported with bullous SLE presenting in a localized linear pattern. She is also the second‐youngest reported patient with bullous SLE.
Probable Congenital Melanocytic Nevus of the Oral Mucosa: Case Report
Authors: Allen Carl M. , Pellegrini Arthur
Published: March 2008

Abstract: There are few case reports of congenital melanocytic nevi affecting the oral mucosa. We report the clinical and histologic features of an Intraoral melanocytic lesion that in our opinion meets the criteria for the diagnosis.
Caseating Cutaneous Granulomas in a Child with Common Variable Immunodeficiency
Authors: Torrelo Antonio , Mediero Imelda G , Zambrano Antonio
Published: March 2008

Abstract: Cutaneous, noninfectious, granulomatous lesions have been reported occasionally In different types of Immunodeficiencies, Including common variable immunodeficiency (CVID). We present a child with CVID and cutaneous granulomas with a strikingly prominent caseating necrosis. We think that such granulomatous lesions constitute a distinctive manifestation of Immunodeficiency, and may reflect an altered immune response. Corticosteroids have been of benefit to our patient, as well as in similar cases.
Clinical Pattern of Cutaneous Drug Eruption among Children and Adolescents in
Authors: Sharma Vinod K. , Dhar Sandipan
Published: March 2008

Abstract: Various types of cutaneous drug eruptions and the Incriminating drugs were analyzed tn 50 children and adolescents up to 18 years of age (34 or 65% boys, 16 or 32% girls). Thirteen (26%) patients had a maculopapular rash, 11 (22%) a fixed drug eruption (FDE), 10 erythema multiforme (EM), 6 (12%) toxic epidermal necrolysis (TEN), 5 (10%) Stevens‐Johnson syndrome (SJS), 3 (6%) urticaria, and 2 (4%) erythroderma. The Incubation period for maculopapular rashes, SJS and TEN due to commonly used antibiotics and sulfonamides was short, a few hours to two to three days, reflecting reexposure, and for drugs used sparingly such as antiepileptics and antitubereulosis agents, was approximately one week or more, suggesting a first exposure. Antibiotics were responsible for cutaneous eruptions in 27 patients, followed by antlepileptics In 17, analgin in 4, and metronidazole and albendazole in 1 each. Cotrimoxazole, a combination of sulfamethoxazole and trimethoprim, was the most common antibacterial responsible for eruptions (11 patients), followed by penicillin and its semisynthetlc derivatives (8 patients), sulfonamide alone (3 patients), and other antibiotics (4 patients). Antiepileptics were the most frequently incriminated drugs in EM, TEN, and SJS. The role of systemic corticosteroids in the management of SJS and TEN is controversial. We administered prednisolone or an equivalent corticosteroid 2 mg/kg/day for 7 to 14 days. With this dosage the mortality rate in the combined patients with TEN and SJS was 18.2%. Our limited experience suggests that these drugs might still have a role in the management of SJS and TEN In children and adolescents.
Treatment of Angioblastoma with Recombinant Interferon‐α2
Authors: Park Kyoung Chan , Ahn Phil Soo , Lee Vong Suk , Kim Kyu Han , Cho Kwang Hyun
Published: March 2008

Abstract: interferon‐α2 has been used successfully to treat anglomatous lesions such as Kaposi sarcoma and life‐threatening hemangiomas, possibly by its antlangiogenetic activity. We report the successful use of this agent in a child with an angloblastoma of the right leg.
Congenital Erythrodermic Psoriasis: Case Report and Literature Review
Authors: Salleras Montse , Sanchez‐Regaña Manuel , Umbert Pablo
Published: March 2008

Abstract: A 4‐year‐old girl was seen in our department for erythroderma, palmoplantar hyperkeratosis, and scalp desquamation present since birth. The dermatosis had run an intermittent course, with exacerbations after Infections and spontaneous remissions. A specimen from a skin biopsy performed at 1 year of age showed the characteristic features of psoriasis, findings that were confirmed in our biopsy specimen. Treatment with acltretin controlled the outbreaks. At 7 years of age she has developed, for the first time, plaque type psoriasis. Congenital erythroderma is an unusual form of psoriasis with a wide differential diagnosis.
Kaposi Sarcoma in a Thai Boy with Acquired Immunodeficiency Syndrome
Authors: Pruksachatkunakorn Chulabhorn , Uruwannakul Kittchai , Bhoopat Lertlakana
Published: March 2008

Abstract: Kaposi sarcoma is rare In children with acquired immunodeficiency syndrome (AIDS). We report a 3‐year‐old boy with AIDS and Kaposi sarcoma of the skin and lymph node. This pattent Is the only one with this disease among 278 children with AIDS who have been seen at Chiang Mai University Hospital. He responded well to intravenous vincristine.
Placental Nevus Cells Associated with Giant Congenital Pigmented Nevi
Authors: Antaya Richard J. , Keller Richard A. , Wilkerson James A.
Published: March 2008

Abstract: We present an infant born with a giant congenital pigmented nevus. The placenta revealed nests of nevus cells in the chorionic villi on the fetal side of circulation. These findings are consistent with four reports in the literature to date.
True Tail in a Newborn
Authors: Alashari Mouied , Torakawa Joy
Published: March 2008

Abstract: Human true tall is a rarely reported anomaly that may have a marked psychologic impact on the patient's family and may be associated with other congenital anomalies. A true tail in a newborn girl is reported, and findings from a review of the literature are summarized. The clinical and pathologic differential diagnoses are discussed, as they might affect the management and prognosis of this congenital malformation.
Neurofibromatosis‐Noonan Syndrome
Authors: Buehning Laura , Curry Cynthia J.
Published: March 2008

Abstract: Type I neurolibromatosis (NF‐1) and Noonan syndrome (NS) are two fairly common genetic disorders. Patients with features of both disorders have been described, but considerable variability of phenotypic expression occurs. As a result, the correct nosology of this syndrome is uncertain. We present a patient with full expression of both NF‐1 and NS phenotypes, and discuss the debate regarding the genetics of the combined syndrome.
Upper Extremity Atrophy Associated with a Giant Congenital Melanocytic Nevus
Authors: Skidmore Robert A. , Ivker Rachel A. , Resnick Steven D.
Published: March 2008

Abstract: Persistent limb atrophy in association with a giant congenital melanocytic nevus is described. This association has not been reported previously.
Acute Graft‐versus‐Host Disease with Unusual Cutaneous Intracellular Vacuolation in an Infant with Severe Combined Immunodeficiency
Authors: Farrell Anne , Scerri Lawrence , Stevens Alan , Millard Leslie G.
Published: March 2008

Abstract: We report an infant with severe combined immunodeficiency (SCID) who had cutaneous manifestations of acute graft‐versus‐host disease (GVHD) due to maternofetal lymphocyte engraftment. Histologic and ultrastructural examinations of the skin revealed intracellular vacuoles resembling lipid droplets in the epidermis and dermis, which is not a recognized feature of acute GVHD or SCID.
Linear Porokeratosis Presenting as Erosions in the Newborn Period
Authors: Fisher Carla A. , LeBoit Philip E. , Frieden Ilona J.
Published: March 2008

Abstract: The classic appearance of porokeratosis is characterized by a hyperkeratotic annular rim that expands peripherally, leaving an atrophic center. Linear porokeratosis is a variant with collections of such lesions arranged in a linear fashion, usually corresponding to a dermatome or Blaschko's lines. Ulcerations have rarely been reported in patients with porokeratosis. We report an unusual case of linear porokeratosis at birth, with erosions and Ulcerations of the face and lower extremity, that eluded diagnosis for nearly a year. Porokeratosis should be considered in the differential diagnosis of erosions in the newborn period.
Erythrokeratodermia Variabilis Present at Birth: Case Report and Review of the Literature
Authors: Hendrix John D. , Greer Kenneth E.
Published: March 2008

Abstract: A healthy boy had the distinctive lesions of erythrokeratodermia variabilis (EKV) at birth. Twenty‐eight patients described in the literature had EKV that presented in childhood. Nine of the 28 were said to have had a rash since birth, but none were distinctive of EKV. To our knowledge this is the first well‐documented case describing a child born with the skin manifestations of EKV. We conclude that patients with EKV are infrequently born with a rash, and that only very rarely when the rash is present is it suggestive of the disorder.
Cutaneous Presentation of Juvenile Chronic Myelogenous Leukemia: A Diagnostic and Therapeutic Dilemma
Authors: Sires Ulrike I. , Mallory Susan B. , Hess Jay L. , Keating James P. , Bloomberg Gordon , Dehner Louis P.
Published: March 2008

Abstract: Annular, erythematous, circinate plaques were the first manifestation of juvenile chronic myelogenous leukemia (JCML) in an otherwise healthy 2.5‐year‐old boy who had had these lesions since 6 months of age. The lesions showed an atypical hematopoietic infiltrate on biopsy. Biopsy of a bone marrow specimen and peripheral blood smear were normal six months before leukemic transformation. At 3 years of age the boy developed splenomegaly, thrombocytopenia, and petechiae, and a bone marrow aspirate and cell marker studies were regarded as consistent with, if not diagnostic of, JCML. Four previous cases of cutaneous leukemic infiltrate associated with JCML have been published. Our patient had recurring urticarial‐like plaques for two years before the initial bone marrow finding of JCML. Given the poor prognosis and progressively evolving course of JCML, it may be appropriate to consider therapy before bone marrow changes, based on the presence of the cutaneous eruption with the appropriate findings on skin biopsy and an elevated fetal hemoglobin.
A Clinical Study of Childhood Alopecia Areata in Chandigarh, India
Authors: Sharma Vinod K , Kumar Bhushan , Dawn Goutam
Published: March 2008

Abstract: All new cases of alopecia areata (AA) were studied during the years 1983–1993. Eight hundred forty‐one cases were recorded, including 201 (23.9%) children less than 16 years of age. The female:male ratio was 1.4:1 (117 girls, 84 boys) for childhood AA. Alopecia was severe, (hat is, total, universal, or extensive, in 34(16.9%) children. Onset occurred in 77 (38.3%) children between ages 6 and 10 years, In 67 (33.3%) before 5 years of age, and in 57 (28.4%) between 11 and 16 years. Onset before 5 years of age was more often associated with severe alopecia than onset at ages 11 to 16 years (p < 0.01). Onset before 2 years of age was commonly associated with severe alopecia, seen in 6 (55.5%) of 11 children. Twenty‐five (12.4%) children had one or more family members with AA. Definite evidence of atopy was obtained In 35 (17.5%) children. Association of atopy with severe alopecia was not statistically significant at Initial presentation (16% vs 23.5% for circumscribed and severe alopecia, respectively; p > 0.05). Nail changes were found In 60 (30%) children and were more frequent in severe alopecia (53%) than in circumscribed alopecia (25.2%, p < 0.001). Associated vittllgo was found in seven (3.5%) children, and one child was hypothyroid. Childhood AA in Chandigarh, India, is remarkably similar to that seen in Western countries, except that an association of atopy with younger age at onset and severe alopecia was not confirmed.
Kindler Syndrome: Report of a Case with Ultrastructural Study and Review of the Literature
Authors: Patrizi Annalisa , Pauluzzi Paolo , Neri Iria , Trevisan Giusto , Badiali De Giorgi Lucilla , Pasquinelli Gianandrea
Published: March 2008

Abstract: Kindler syndrome is characterized by a generalized, progressive polkiloderma with cutaneous atrophy, congenital acral skin blistering, and photosensitivity. Since the first description, approximately 70 cases have been reported worldwide, but ultrastructural studies were performed in only five patients. In none of these patients were biopsies done at birth. In our patient ultrastructural studies were performed both of the blister at birth and of the poikilodermatous and atrophic skin at 6 years of age. Some ultrastructural features in the context of a bullous disease of the newborn that resembles epidermolysis bullosa, should alert investigators to the possibility of Kindler syndrome even in absence of the typical clinical signs.
Experience with UVB Phototherapy in Children
Authors: Tay Yong‐Kwang , Morelli Joseph G. , Weston William L.
Published: March 2008

Abstract: Twenty children age 14 months to 12 years with photoresponsive dermatoses were treated with ultraviolet B (UVB) phototherapy over four years. Ten children had psoriasis, five had pityriasis lichenoides, and five had atopic dermatitis. All received short courses (average 34 treatments) of phototherapy with either no maintenance or short maintenance. Treatment was effective and well tolerated in most patients, and no serious side effects were seen. Patients with psoriasis and pityriasis lichenoides cleared completely. No patient with atopic dermatitis cleared completely, but all were moderately improved, with reduction of the extent of eczema and decreased pruritus. It appears that UVB phototherapy is a valuable and safe therapeutic option for selected children who do not respond to other treatments.
Mechanical Properties of Skin in Ehlers‐Danlos Syndrome, Types I, II, and III
Authors: Henry Frédérique , Goffin Véronique , Piérard‐Franchimont Claudine , Piérard Gérald E.
Published: March 2008

Abstract: Mechanical properties of skin were evaluated in vivo in 17 children suffering from Ehlers‐Danlos syndrome (EDS) types I, II, and III. These were compared with normal values from 63 healthy children. Non‐invasive measurements were performed under suction of 500 mbar using a Cutometer equipped with a 4‐mm probe. Prominent increases in skin extensibility and elasticity were the most distinctive and diagnostic features of all three EDS types. Differences in the average values of biome‐chanical variables were present among the three types. Patients with EDS‐I were the most affected, whereas those with EDS‐III had virtually normal skin. However, interindividual differences in the severity of rheo‐logical alterations were found within each type, illustrating a continuum in the variation of mechanical properties of EDS skin rather than yielding step differences among the types.
Malignant Rhabdoid Tumor Presenting as a Hemangioma
Authors: Albregts Anthony E. , Hebert Adelaide A. , Aboul‐Nasr Rashad A. , Raney R. Beverly
Published: March 2008

Abstract: Malignant rhabdoid tumor is a rare and highly aggressive malignancy of unknown etiology. We report a primary cutaneous rhabdoid tumor on the upper back of a newborn. It was initially diagnosed as a hemangioma clinically, and that diagnosis was supported by radiologic studies, including magnetic resonance imaging. However, detailed investigation with light microscopy, immunohistochemistry, and electron microscopy enabled us to make a diagnosis of rhabdoid tumor. This is the seventh reported case of rhabdoid tumor with primary cutaneous involvement.
Congenital Leukemia Cutis Preceding Monoblastic Leukemia by 3 Months
Authors: Monpoux Fabrice , Lacour Jean Philippe , Hatchuel Yves , Hofman Paul , Raynaud Sophie , Sudaka Isabelle , Ortonne Jean Paul , Mariani Roger
Published: March 2008

Abstract: We report an infant born with a cutaneous nodular eruption and neutropenia. Skin biopsy specimens revealed an immature dermal infiltrate suggestive of leukemia cutis, but repeated peripheral blood and bone marrow examinations failed to demonstrate malignant cells. The eruption resolved spontaneously. At the age of 3 months, a second occurrence of maculopapular skin lesions led to discovery of an acute monoblastic leukemia with (9;11)(p21–22;q23) translocation. Congenital acute leukemia is a rare disease associated with skin infiltration in 25% to 30% of patients. Usually the diagnosis is easily made by peripheral blood examination and/or bone marrow aspirate. However, skin involvement may precede acute leukemia by several weeks. Although very rare, this event must be kept in mind.
Chronic Meningococcemia in Childhood: Case Report and Review of the Literature
Authors: Ploysangam Tanusin , Sheth Anita P.
Published: March 2008

Abstract: Chronic meningococcemia is a rare form of meningococcal infection. Most reported cases occur in adults. Only about 10% of reported patients are under 18 years of age. We herein report chronic meningococcemia in a 17‐month‐old immunocompetent child and review the literature. Our patient had a 4‐month history of several episodes of intermittent fever accompanied by widespread erythematous and purpuric, crusted papules and pustules with necrotic centers. Blood cultures were positive for group C Neisseria meningitidis. Histopathology of a skin biopsy specimen revealed leukocytoclastic vasculitis. Because the disease is uncommon and can be confused with other conditions including other infectious processes, vasculitis, and collagen vascular diseases, it is important to have a high degree of suspicion for chronic meningococcemia. This will help avoid a delayed diagnosis and/or inappropriate treatment. The positive blood culture remains the diagnostic sine qua non. Treatment with ceftriaxone was rapidly successful in our patient.
Trichophyton rubrum Infection in a 3‐Dav‐Old Neonate
Authors: Singal Archna , Baruah M. C. , Rawat Sudha , Sharma S. C.
Published: March 2008

Abstract: Trichophyton rubrum infection on the face of a 3‐day‐old neonate is reported because of its rarity.
Trichostasis Spinulosa: A Clinical Simulant of Acne Open Comedones
Authors: Harford Robert R. , Cobb Mark W. , Miller Matthew L.
Published: March 2008

Abstract: Trichostasis spinulosa is a disorder of the pilosebaceous follicles that may simulate acne open comedones. It is a commonly reported cosmetic annoyance in adults but not children. A review of the literature and a case of trichostasis spinulosa in a 13‐year‐old black girl with a history of onset at 18 months of age are presented.
Crouzon Disease with Acanthosis Nigricans and Melanocytic Nevi
Authors: Ginés Elena , Rodriguez‐Piehardo Antonio , Jorquera Enrique , Moreno Jose Carlos , Camacho Francisco
Published: March 2008

A number of craniofacial malformation syndromes are characterized by prominent abnormalities of the skin, hair, nails, and mucous membranes. Crouzon disease has been reported in association with severe acanthosis nigricans in 12 patients. We report a new case of this association in a 16‐year‐old woman with multiple melanocytic nevi. Melanocytic nevi have been already reported in the medical literature in association with several congenital syndromes, although, to our knowledge, not with Crouzon disease.
Phakomatosis Pigmentovascularis: Report of a Case
Authors: Mahroughan M. , Mehregan Amir H. , Mehregan David A.
Published: March 2008

Phakomatosis pigmentovascularis is a rare cutaneous malformation characterized by a combination of melanocytic nevi and nevus flammeus. We report a 16‐year‐old boy with extensive and predominantly unilateral skin involvement combining features of speckled melanocytic nevi over a background of nevus flammeus. A brief review of the literature and classification of this phakomatosis is presented.
Neonatal Acute Hemorrhagic Edema of Childhood: Case Report and Review of the English‐Language Literature
Authors: Cunningham Bari B. , Caro William A. , Eramo Lynne R.
Published: March 2008

Acute hemorrhagic edema of childhood is an unusual form of leukocytoclastic vasculitis previously reported in children age 4 months to 3 years. The etiology remains unknown, however, many authors describe the process as an immune‐mediated vasculitis in response to a variety of antigenic stimuli. We report a case of congenital acute hemorrhagic edema of childhood associated with maternal gastroenteritis six weeks before delivery.