Abstract:
:The disorders of cornification (ichthyoses) comprise acquired and inherited disorders characterized clinically by generalized scaling and histologically by hyperkeratosis. They may arise through defects in the production or maintenance of a normal cornified cell compartment, or both. The stratum corneum is composed of protein-enriched and lipid-depleted corneocytes ("bricks") surrounded by an intercellular domain ("mortar") composed of hydrophobic, lipid-enriched membrane bilayers, and containing desmosomes and a limited array of hydrolytic enzymes. Mechanisms whereby a genetic defect involving either the bricks or the mortar may result in abnormal stratum corneum retention are discussed using ichthyosis vulgaris and recessive X-linked ichthyosis as examples. In addition, epidermal hyperproliferation, which floods the cornified cell compartment with incompletely formed units, results in hyperkeratosis. To date, no primary disorders of epidermal hyperproliferation have been defined. Recent work, however, demonstrates that stratum corneum barrier function regulates epidermal DNA synthesis. For example, in essential fatty acid deficiency, barrier dysfunction is responsible at least in part ror the epidermal hyperproliferation. Defective barrier function due to defective lamellar body secretion may also underlie the phenotypic changes after birth in harlequin ichthyosis; that is, from the massive, constrictive hyperkeratosis of the newborn to an exfoliative erythroderma in survivors. The mechanisms whereby specific defects in cornification result in generalized scaling disease are only beginning to be defined. Yet, even at this early stage, the view of the stratum corneum as a tightly organized structure whose function is highly regulated is emerging. Hence, the disorders of cornification should provide important insights into stratum corneum structure and function.
journal_name
Pediatr Dermatoljournal_title
Pediatric dermatologyauthors
Williams MLdoi
10.1111/j.1525-1470.1992.tb00632.xsubject
Has Abstractpub_date
1992-12-01 00:00:00pages
365-8issue
4eissn
0736-8046issn
1525-1470journal_volume
9pub_type
杂志文章,评审abstract::We report a child with bilateral congenital triangular alopecia. Shaving the scalp and inserting intravenous cannulas into scalp vessels during the neonatal period was misconstrued by the parents as the cause of hair loss, and resulted in medicolegal implications. ...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.1996.tb00708.x
更新日期:1996-09-01 00:00:00
abstract::The term Mycoplasma pneumoniae-induced rash and mucositis (MIRM) was recently proposed to identify the mucocutaneous condition secondary to M. pneumoniae infection that had historically been regarded among the more confusing pathologies of erythema multiforme and Stevens-Johnson syndrome. Based on a number of previous...
journal_title:Pediatric dermatology
pub_type: 杂志文章,评审
doi:10.1111/pde.13140
更新日期:2017-07-01 00:00:00
abstract::Trichothiodystrophy is characterized by sparse, short, sulfur-deficient hair. Numerous symptom complexes have been described in which the hair abnormality represents a constant feature. We report a boy with trichothiodystrophy, ichthyotic skin changes, onychodystrophy, chronic neutropenia, osteosclerosis, hypothyroidi...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.1993.tb00034.x
更新日期:1993-06-01 00:00:00
abstract:BACKGROUND:Fibroblastic connective tissue nevi (FCTN) are benign skin conditions characterized by bland spindle cells infiltrating the reticular dermis and the upper subcutis with preservation of adnexal structures. A subset of FCTN expresses CD34, which may cause difficulties in the differential diagnosis, in particul...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.13571
更新日期:2018-09-01 00:00:00
abstract::The October 2018 report from the United Nations Intergovernmental Panel on Climate Change predicts significant threats to human health secondary to anthropogenic global warming; children have been and will continue to be disproportionately affected by these weather-related changes. Multiple physician groups have ackno...
journal_title:Pediatric dermatology
pub_type: 杂志文章,评审
doi:10.1111/pde.13819
更新日期:2019-07-01 00:00:00
abstract::Acquired ichythosis has been described in adult hematopoietic stem cell transplant recipients. While some authors have suggested the association of acquired ichythosis with cutaneous graft-versus-host disease, the evidence to support this association is rare. We describe a patient who developed enteritis secondary to ...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.2007.00333.x
更新日期:2007-01-01 00:00:00
abstract::Highly active antiretroviral therapy can restore specific immune responses and control of microorganism infections in human immunodeficiency virus-positive patients. This immune recovery may cause an inflammatory reaction to microbial and autoimmune antigens known as immune reconstitution inflammatory syndrome. We des...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.2010.01322.x
更新日期:2010-11-01 00:00:00
abstract::Triethanolamine is used as an emulsifier in many cosmetics and in topical medications, yet the occurrence of contact dermatitis to cosmetics containing triethanolamine is rare in childhood. Our case highlights how young age should not be a deterrent to investigation and demonstrates the importance of patch testing wit...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.12537
更新日期:2015-05-01 00:00:00
abstract::Condylomata acuminata (CA), or anogenital warts, are typically benign lesions caused by human papillomavirus infection. Although they are rare, immunocompromised individuals are at a higher risk of CA undergoing transformation into invasive anal squamous cell carcinoma (SCC). These patients need aggressive evaluation ...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.12617
更新日期:2015-07-01 00:00:00
abstract::This study sought to identify barriers to treatment in children with chronic inflammatory skin disease, particularly those with atopic dermatitis, psoriasis, and acne vulgaris. Caregivers of 101 patients seen in the Children's Specialty Group Division of Dermatology, Children's Hospital of The King's Daughters, Norfol...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.2011.01493.x
更新日期:2011-05-01 00:00:00
abstract::Childhood lichen sclerosus (LS) is a rare and often misdiagnosed inflammatory dermatitis with an unpredictable course. The complications of LS are architectural changes of the vulva; malignant transformation is possible. The objective of our study was to define the background and the long-term course of childhood LS. ...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.12109
更新日期:2013-07-01 00:00:00
abstract::Lichen striatus is a common childhood eruption, but only rare reports link cutaneous manifestations and onychodystrophy. We report a case of lichen striatus and onychodystrophy in a 12-year-old girl, only the eighth such patient in the dermatologic literature. Nail changes may precede the rash of lichen striatus and s...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.1993.tb00399.x
更新日期:1993-12-01 00:00:00
abstract::Bascule syndrome is a recently described benign vasomotor dermatosis characterized by Bier anemic spots, cyanosis, and urticaria-like eruption. We report a case of a 13-year-old girl with cutaneous lesions consistent with Bascule syndrome who had had three exercise-related syncopal episodes. It would be recommended to...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.13425
更新日期:2018-03-01 00:00:00
abstract::Hand-foot-mouth disease (HFMD) is a contagious enteroviral infection occurring primarily in children and characterized by a vesicular palmoplantar eruption and erosive stomatitis. Nail matrix arrest has been associated with a variety of drug exposures and systemic illnesses, including infections, and may result in a v...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1046/j.1525-1470.2000.01702.x
更新日期:2000-01-01 00:00:00
abstract::Tinea capitis is a common infection of childhood. There have been several reports of tinea capitis in newborns. Our patient presented at 19 days of age to the emergency room with a scalp lesion of 5 days duration. The fungal culture grew both Trichophyton rubrum and Trichophyton mentagrophytes. The patient was success...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.1997.tb00245.x
更新日期:1997-05-01 00:00:00
abstract::The human papillomavirus (HPV) infects the squamous epithelium of the skin and produces common warts, plantar warts, and flat warts, which occur commonly on the hands, face, and feet. The objective of this study was to determine the presence of HPV in warts in children in order to associate the virus with the disease....
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.12113
更新日期:2013-11-01 00:00:00
abstract::We describe three teenage siblings with confluent and reticulated papillomatosis, all presenting during a 6-month period. Two of the three patients had confirmed tinea versicolor, with positive potassium hydroxide scrapings, in association with this entity. This is the largest series of siblings with confluent and ret...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.2005.22410.x
更新日期:2005-07-01 00:00:00
abstract::Hoyeraal-Hreidarsson syndrome is a rare telomere biology disorder that is recognized as a severe variant of dyskeratosis congenita. We present a Libyan boy with hematologic and neurologic abnormalities with typical dermatologic manifestations of dyskeratosis congenita. Death usually occurs before the age of 4 years as...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.12693
更新日期:2015-11-01 00:00:00
abstract::The first report of spinous follicular lichen nitidus with perifollicular granulomas was by Madhok and Winkelmann in 1988. Since this report, a few cases of follicular or periappendageal lichen nitidus have been described, in a more localized form or without perifollicular granulomas. We describe a 5-year-old girl wit...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.2012.01789.x
更新日期:2013-05-01 00:00:00
abstract:INTRODUCTION:Extravasation injuries are a common and challenging problem in hospitalized newborns. Accidental infusion leakage into the surrounding tissues in immature infants may frequently result in skin necrosis, with significant risk of functional and cosmetic impairment. MATERIAL AND METHODS:In the present study ...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.12664
更新日期:2015-11-01 00:00:00
abstract::A research task force of the Society for Pediatric Dermatology (SPD) was established to investigate the barriers to expanding research in the field of pediatric dermatology. A survey was designed to address constraints limiting research activities among members of the SPD. A nine-question survey was distributed to SPD...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.2010.01162.x
更新日期:2010-07-01 00:00:00
abstract::Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon lesion with remarkably consistent histopathologic features that arises primarily in the pediatric population. We describe a MNTI arising in the anterior maxilla of a 6-month-old boy. ...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.2011.01566.x
更新日期:2012-09-01 00:00:00
abstract::Hereditary progressive mucinous histiocytosis is a rare, benign, skin-limited form of non-Langerhans cell histiocytosis. We report on a 5-year-old boy who presented in infancy with self-resolving dermal nodules but later developed persistent and progressive erythematous papules on the face and scalp. Histologic evalua...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.12694
更新日期:2015-11-01 00:00:00
abstract:BACKGROUND:Phosphodiesterase-4 (PDE4) is a promising target in atopic dermatitis (AD) treatment. The pharmacokinetics (PK), safety, and efficacy of crisaborole topical ointment, 2% (formerly AN2728) (Anacor Pharmaceuticals, Palo Alto, CA), a boron-based benzoxaborole PDE4 inhibitor, were evaluated in children with mild...
journal_title:Pediatric dermatology
pub_type: 杂志文章,多中心研究
doi:10.1111/pde.12872
更新日期:2016-07-01 00:00:00
abstract::Diaper dermatitis, an acute inflammation of the skin in the diaper area, is the most common dermatologic disorder of infancy. This placebo-controlled, randomized, double-blind, parallel-group trial compared the efficacy and safety of miconazole nitrate 0.25% in a zinc oxide/petrolatum base with that of the ointment ba...
journal_title:Pediatric dermatology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1046/j.1525-1470.2001.018002149.x
更新日期:2001-03-01 00:00:00
abstract::A 6-month-old baby had erythema, fissuring of the skin, and craquelé due to prolonged use of a liquid soap mistakenly believed to be a moisturizer. We think these kinds of skin disorders will become increasingly common due to such misunderstandings. The proliferation of cosmetic products for baby skin care, the increa...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.1993.tb00378.x
更新日期:1993-09-01 00:00:00
abstract::Ataxia telangiectasia (AT) is a rare autosomal recessive neurodegenerative disorder caused by a mutation in the ATM gene. An impaired immune response due to the gene mutation leads to an increased risk of infection and malignancy. We present a rare case of dermatofibrosarcoma protuberans arising in a patient with AT. ...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.13779
更新日期:2019-05-01 00:00:00
abstract::Menkes's syndrome (trichopoliodystrophy) is an x-linked, recessive genodermatosis characterized by hair defects, severe retardation, convulsions, progressive neurologic deterioration, and early death. Recent studies in copper metabolism suggest that Menkes's syndrome may be a storage disease in which copper is irrever...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/j.1525-1470.1984.tb01135.x
更新日期:1984-04-01 00:00:00
abstract:BACKGROUND/OBJECTIVES:Previous studies have demonstrated an increased prevalence of inflammatory bowel disease (IBD) in adults with hidradenitis suppurativa (HS). Whether the same association exists in pediatric patients is unknown. Fecal calprotectin (FC) is used to screen and monitor disease activity in IBD. There ar...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.14417
更新日期:2020-10-25 00:00:00
abstract::The most common cause of death in blue rubber bleb nevus syndrome is gastrointestinal bleeding. Here we present a case of central nervous system bleeding that resulted in death. ...
journal_title:Pediatric dermatology
pub_type: 杂志文章
doi:10.1111/pde.12718
更新日期:2016-01-01 00:00:00