Abstract:
:Three siblings with infantile-onset biotin-responsive multiple carboxylase deficiency are described. Recognition of the characteristic dermatologic manifestations, alopecia and periorificial dermatitis, should result in early diagnosis and institution of potentially lifesaving therapy with biotin. Other metabolic disorders may present a similar clinical picture. Immunologic dysfunction and/or aberration in lipid or branched chain amino acid metabolism may be the common pathophysiologic link in some or all of these disorders.
journal_name
J Am Acad Dermatoljournal_title
Journal of the American Academy of Dermatologyauthors
Williams ML,Packman S,Cowan MJdoi
10.1016/s0190-9622(83)70113-1subject
Has Abstractpub_date
1983-07-01 00:00:00pages
97-103issue
1eissn
0190-9622issn
1097-6787pii
S0190-9622(83)70113-1journal_volume
9pub_type
杂志文章abstract::The analysis of population-specific human leukocyte antigen (HLA) haplotypes has provided evidence that susceptibility to psoriasis is linked to the class I and II major histo-compatibility complex on human chromosome 6. In addition, these studies show that psoriasis consists of two distinct disease subtypes (type I a...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:
更新日期:1997-08-01 00:00:00
abstract::Diabetic dermopathy (DD) is the most common cutaneous manifestation of diabetes mellitus. DD refers to atrophic, hyperpigmented macules characteristically located on the shins of patients with diabetes. They have an unfavorable association with the 3 most common microangiopathic complications of diabetes mellitus: neu...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/j.jaad.2007.11.013
更新日期:2008-03-01 00:00:00
abstract::The neutrophil function and plasma leukotactic activity of a patient with Sweet's syndrome and cystonodular acne were evaluated during a 2 1/2-year period. These studies demonstrated that chemotaxis was frequently slightly increased, especially during an exacerbation of Sweet's syndrome, but showed some decrease durin...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(85)70130-2
更新日期:1985-06-01 00:00:00
abstract:BACKGROUND:Griseofulvin has been used in the treatment of toenail onychomycosis with limited success. Evidence suggests that terbinafine may be more effective. OBJECTIVE:In a double-blind, parallel-group study we compared 250 mg/day terbinafine for 16 weeks with 500 mg/day griseofulvin for 52 weeks (or for shorter per...
journal_title:Journal of the American Academy of Dermatology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1016/0190-9622(95)91454-4
更新日期:1995-05-01 00:00:00
abstract::A patient with symptomatic urticaria pigmentosa who responded to nifedipine therapy is reported. Relief from cold-induced urtication and flushing was obtained with 10 mg taken orally three times daily. Calcium influx is an early step in the degranulation of mast cells. We hypothesize that the beneficial effect of nife...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(84)70233-7
更新日期:1984-10-01 00:00:00
abstract::In this third and last part of our review of cutaneous vascular proliferations we include malignant vascular neoplasms and a group of heterogeneous cutaneous neoplasms characterized by a significant vascular component. We also review some disorders that, in our opinion, have been erroneously considered as vascular neo...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/s0190-9622(98)70237-3
更新日期:1998-02-01 00:00:00
abstract::The clinical and histologic features of 26 cases of erythema induratum are reviewed. The characteristic cutaneous lesions on the legs of middle-aged women were violaceous, indurated nodules, 1 to 2 cm in diameter, which were painful, occasionally ulcerated, and could heal with scarring. The diagnosis was confirmed in ...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(89)70248-6
更新日期:1989-10-01 00:00:00
abstract::Blistering of light-exposed skin, pink-stained fluorescing diapers, and fluorescing peripheral erythrocytes led to diagnosis of congenital porphyria in an infant born to consanguineous parents. Although massive coproporphyrinuria and coproporphyrinemia initially suggested a coproporphyrinogen oxidase deficiency disord...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(96)90081-x
更新日期:1996-05-01 00:00:00
abstract:BACKGROUND:Conventional therapy of severe bullous pemphigoid (BP) relies on the use of high-dose systemic corticosteroids with or without adjuvants, notably immunosuppressive agents. This approach can result in debilitating and potentially fatal side effects, hence the need to explore alternative therapies. Intravenous...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,meta分析
doi:10.1067/mjd.2000.112288
更新日期:2001-01-01 00:00:00
abstract:BACKGROUND:It is unclear whether patients with atopic dermatitis (AD) have an altered prevalence or risk for contact sensitization. Increased exposure to chemicals in topical products together with impaired skin barrier function suggest a higher risk, whereas the immune profile suggests a lower risk. OBJECTIVE:To perf...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,meta分析,评审
doi:10.1016/j.jaad.2017.02.001
更新日期:2017-07-01 00:00:00
abstract::Oral juvenile xanthogranuloma is a rare condition, with only 10 histologically proven cases previously described. A histologically unusual variant is reported here, diagnosis of which was aided by the use of immunohistochemical stains. Previously reported cases are reviewed, and their characteristics are tabulated and...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/0190-9622(95)91438-2
更新日期:1995-08-01 00:00:00
abstract::Dacarbazine (DTIC-Dome) is a widely used chemotherapeutic agent employed in the therapy of malignant melanoma. In this communication, we present two patients who developed photosensitivity skin reactions immediately after its administration. Physicians using dacarbazine should be aware of this side effect and should i...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(81)70053-7
更新日期:1981-05-01 00:00:00
abstract::Atopic dermatitis is a chronic inflammatory skin disease that is frequently associated with respiratory allergies. Atopic dermatitis develops as a result of a complex interrelationship of environmental, immunologic, genetic, and pharmacologic factors. Efforts to understand the relative contributions of these factors h...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1067/mjd.2001.109815
更新日期:2001-01-01 00:00:00
abstract:BACKGROUND:Condyloma acuminatum are common lesions caused by human papillomavirus (HPV). HPV is associated with many human cancers, and a vaccine now prevents infection with high-risk HPV. However, eradication of established disease is difficult, indicating that these lesions are capable of local immunosuppression. OB...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2008.08.011
更新日期:2008-12-01 00:00:00
abstract:BACKGROUND:Patients with epidermal nevi strongly demand cosmetic improvement. Laser treatment appears appealing and is frequently used in clinical practice. Nevertheless, large series with long-term follow-up are missing, preventing definitive conclusions about its real benefit. OBJECTIVE:To evaluate the long-term eff...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2019.06.013
更新日期:2020-12-01 00:00:00
abstract:BACKGROUND:Cutaneous calcification is an acquired disorder whereby insoluble, amorphous calcium salts deposit in the skin. Classically, cutaneous calcification is categorized as metastatic, dystrophic, idiopathic, or iatrogenic. OBJECTIVE:The purpose of this study was to further elucidate the underlying pathogenic mec...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/j.jaad.2010.01.046
更新日期:2011-02-01 00:00:00
abstract::Minimal erythema doses (MEDs) of individuals with different skin types were compared. Each skin type was shown to be statistically different from each other skin type in terms of sunburn sensitivity. Individuals of each skin type who primarily stayed indoors were compared with those who attempted to be outdoors. In th...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(81)70106-3
更新日期:1981-10-01 00:00:00
abstract:BACKGROUND:The negative pigment network (NPN) is seen as a negative of the pigmented network and it is purported to be a melanoma-specific structure. OBJECTIVES:We sought to assess the frequency, sensitivity, specificity, and odds ratios (ORs) of NPN between melanoma cases and a group of control lesions. METHODS:Digi...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2012.08.012
更新日期:2013-04-01 00:00:00
abstract::A diabetic renal transplant recipient developed fever, generalized rash, and genital ulcers which showed typical histologic changes of cytomegalovirus (CMV) infection in the dermal vascular endothelium. This infection was confirmed by rising serologic titer of specific antibody, positive viral cultures, and typical nu...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(83)70212-4
更新日期:1983-12-01 00:00:00
abstract::A woman with pemphigus vulgaris was treated by plasma exchange. Four separate exchanges of approximately 4,000 ml each were performed over a 2-week period. The patient's pemphigus antibody titer decreased from 1/320 before the first exchange to 1/20 after the final exchange, and no new lesions developed for 3 weeks fo...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(81)70033-1
更新日期:1981-03-01 00:00:00
abstract:BACKGROUND:Tinea corporis gladiatorum has been an infrequently reported condition in the dermatological literature. Two previous reports have investigated wrestlers during recognized epidemics of tinea corporis gladiatorum, but no study has examined wrestlers to determine the point prevalence of tinea corporis gladiato...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1067/mjd.2000.109284
更新日期:2000-12-01 00:00:00
abstract:BACKGROUND:Lack of agreement on how to accurately capture disease outcomes in localized scleroderma (LS) has hindered the development of efficacious treatment protocols. The LS Cutaneous Assessment Tool (LoSCAT), consisting of the modified LS Skin Severity Index (mLoSSI) and the LS Damage Index, has potential for use i...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2013.02.007
更新日期:2013-08-01 00:00:00
abstract:BACKGROUND:Highly variable results of topical diphenylcyclopropenone (DPCP) in the treatment of alopecia areata have been reported so far. OBJECTIVE:The purposes of our study were to evaluate the efficacy and tolerability of DPCP in the treatment of chronic, extensive alopecia areata and to assess the long-term overal...
journal_title:Journal of the American Academy of Dermatology
pub_type: 临床试验,杂志文章
doi:10.1067/mjd.2001.109309
更新日期:2001-01-01 00:00:00
abstract:BACKGROUND:There is growing evidence that Mohs micrographic surgery (MMS) is the treatment of choice for dermatofibrosarcoma protuberans (DFSP). OBJECTIVE:This study retrospectively compared the recurrence rates of DFSP after MMS with those after wide surgical excision; results at the Mayo Clinic and in the world lite...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:
更新日期:1996-07-01 00:00:00
abstract:BACKGROUND:Electronic claims and medical record databases are increasingly used for observational studies of psoriasis. The purpose of this study was to assess the validity of psoriasis diagnostic codes in an electronic database. METHODS:This study was performed in a population-based setting in Olmsted County, Minneso...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2008.08.034
更新日期:2008-12-01 00:00:00
abstract:BACKGROUND:Lentigo maligna (LM) often displays extensive subclinical spread. Mohs micrographic surgery (MMS) has been proposed to help delineate the true histologic margin; however, visualizing atypical melanocytes on frozen section is challenging and often requires confirmatory permanent paraffin sections. OBJECTIVE:...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1067/mjd.2002.119197
更新日期:2002-01-01 00:00:00
abstract:BACKGROUND:Patients with cutaneous lupus erythematosus (LE) and a history of disease- related photoaggravation, confirmed by phototesting, may not respond to photoprotection and/or medical intervention. Ultraviolet B-hardening therapy may improve tolerance for environmental ultraviolet radiation (UVR) in photosensitive...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2005.10.046
更新日期:2006-03-01 00:00:00
abstract::In a pilot study the therapeutic effect and side effect profile of low-dose interferon alfa-2b in combination with a retinoid for the treatment of cutaneous T cell lymphoma were evaluated. Seven patients (four women, three men) with histologically confirmed cutaneous T cell lymphoma were included. Four patients had re...
journal_title:Journal of the American Academy of Dermatology
pub_type: 临床试验,杂志文章
doi:10.1016/0190-9622(91)70036-2
更新日期:1991-02-01 00:00:00
abstract::The Chimila, a Colombian Amerindian group that lives at a low altitude, have a high incidence of actinic prurigo. We present the human lymphocyte antigen (HLA) findings of 43 Chimilas with the disease and of 62 control subjects and show an association of actinic prurigo with the Cw4 antigen. ...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/0190-9622(90)70150-g
更新日期:1990-06-01 00:00:00
abstract::A 5-month-old boy with hypomelanosis of Ito was studied. Lesions presented as bizarre, hypopigmented whorls and streaks. Histologic studies showed that the hypopigmented areas contained normal melanocytes with decreased content of intracellular melanin. A review of the literature revealed the disease to be a discrete ...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(81)70023-9
更新日期:1981-02-01 00:00:00