Abstract：
BACKGROUND:The focal facial dermal dysplasias (FFDD) are a genetically heterogeneous group of disorders characterized by congenital bilateral scarlike facial lesions, with or without associated facial anomalies. The cases have been reported under various names; thus the nosology is confusing and unclear. OBJECTIVE:Our purposes were to report our kindred, clearly delineate the various types of FFDD reported, and propose a new simplified classification. METHODS:The clinical and histologic changes were examined and genealogy determined for our kindred. The medical literature was reviewed and the reported cases reexamined and categorized according to their clinical features and inheritance patterns. RESULTS:We determined that there are three distinct varieties of FFDD: type I, autosomal dominant FFDD; type II, autosomal recessive FFDD; and type III, FFDD with other facial features. CONCLUSION:We propose a new classification and provide evidence for three distinct varieties of FFDD: type I, autosomal dominant FFDD; type II, autosomal recessive FFDD; and type III, FFDD with other facial features (Setleis syndrome). Our kindred represents type II.
journal_name
J Am Acad Dermatoljournal_title
Journal of the American Academy of Dermatologyauthors
Kowalski DC,Fenske NAdoi
10.1016/0190-9622(92)70225-5subject
Has Abstractpub_date
1992-10-01 00:00:00pages
575-82issue
4eissn
0190-9622issn
1097-6787pii
0190-9622(92)70225-5journal_volume
27pub_type
杂志文章,评审abstract：BACKGROUND:Cutaneous basal cell carcinomas (BCCs) and squamous cell carcinomas (SCCs) have poorer outcomes if treated when large. OBJECTIVE:We sought to estimate the growth rate of BCCs and SCCs and examine the relationship of personal, pathway, and cancer factors with cancer size (diameter). METHODS:We surveyed pati...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/j.jaad.2013.11.009
更新日期：2014-03-01 00:00:00
abstract：:Keratoacanthomas infrequently are treated by radiotherapy. However, keratoacanthomas that are recurrent after surgical excision or whose resection would result in cosmetic deformity may benefit from radiotherapy. Between January 1970 and June 1988, 29 such keratoacanthomas in 18 patients were irradiated. Doses ranged ...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/0190-9622(90)70247-f
更新日期：1990-09-01 00:00:00
abstract：BACKGROUND:Embryofetal developmental toxicity associated with oral administration of vitamin A analogs has led to concern about risks from topical application. OBJECTIVE:This study was conducted to evaluate the potential developmental toxicity of tretinoin emollient cream when applied to the skin of pregnant New Zeala...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/s0190-9622(97)70062-8
更新日期：1997-03-01 00:00:00
abstract：BACKGROUND:Increasing incidence and mortality rates from cutaneous melanoma are a major public health concern. As part of a national effort to enhance early detection of melanoma/skin cancer, the American Academy of Dermatology (AAD) has sponsored an annual education and early detection program that couples provision o...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/s0190-9622(96)90274-1
更新日期：1996-06-01 00:00:00
abstract：:Keratosis lichenoides chronica is a rare dermatosis of unknown etiology with a wide variety of cutaneous manifestations. We present a 47-year-old male with a history of progressive and recalcitrant hyperkeratotic and warty plaques, mimicking verrucous secondary syphilis both clinically and microscopically. We review t...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1067/s0190-9622(03)00894-6
更新日期：2003-09-01 00:00:00
abstract：BACKGROUND:Melanoma in African Americans is rare, and the diagnosis is often delayed, leading to advanced presentation and poor prognosis. OBJECTIVE:The purpose of this retrospective study is to determine whether African American patients diagnosed with melanoma at the Washington Hospital Center were initially seen wi...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/s0190-9622(03)02091-7
更新日期：2004-01-01 00:00:00
abstract：BACKGROUND:The current standard tool for diagnosing allergic contact dermatitis (ACD) in the United States is the T.R.U.E. test panels, which consist of 23 allergens. Previous studies have raised concern regarding the adequacy of these panels in fully assessing patients with possible ACD. OBJECTIVE:We sought to examin...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1067/mjd.2003.489
更新日期：2003-07-01 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：BACKGROUND:Hyperlipidemia has received little attention as a side effect of cyclosporine therapy for severe psoriasis. OBJECTIVE:We report changes in fasting serum lipids in patients treated with low-dose oral cyclosporine for psoriasis and discuss their significance. METHODS:Twenty-two patients with severe, recalcit...
journal_title：Journal of the American Academy of Dermatology
pub_type： 临床试验,杂志文章,多中心研究
doi：10.1016/0190-9622(92)70213-y
更新日期：1992-09-01 00:00:00
abstract：:Palliative care has been shown to improve quality of life, symptom, and caregiver burden for a range of life-limiting diseases. Palliative care use among patients with severe dermatologic disease remain relatively unexplored, but the limited available data suggest significant unmet care needs and low rates of palliati...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章,评审
doi：10.1016/j.jaad.2020.08.029
更新日期：2020-08-12 00:00:00
abstract：:The case presented here involves a 32-year-old homosexual man with human immunodeficiency virus (HIV) seropositivity and unusual manifestations of secondary syphilis. The patient presented with syphilitic keratoderma and chorioretinitis, and his appearance superficially resembled that of a patient with Reiter's syndro...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/s0190-9622(88)70062-6
更新日期：1988-02-01 00:00:00
abstract：BACKGROUND:A narrow-band UVB lamp (Philips TL 01) emitting a peak of approximately 311 nm was developed to improve the phototherapy for psoriasis. Only a few studies have been performed with promising results. OBJECTIVE:The therapeutic efficacy of the Philips TL 01 lamp in a new 100 W version was compared with convent...
journal_title：Journal of the American Academy of Dermatology
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1016/0190-9622(93)70032-o
更新日期：1993-02-01 00:00:00
abstract：:A 4-year-old girl with failure to thrive and intense pruritus associated with Alagille syndrome is described. The clinical features of this autosomal dominant disorder are reviewed. This case highlights the importance of recognizing the severe pruritus as a manifestation of the disease. ...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/j.jaad.2007.03.029
更新日期：2008-02-01 00:00:00
abstract：BACKGROUND:To prevent DNA damage caused by the ultraviolet (UV) radiation of sunlight, sunscreens are commonly used to protect human skin. Current analysis of sunscreens' effectiveness is done through complicated procedures, including human exposure. OBJECTIVE:We sought to design a simple system using thymidine-thymid...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/j.jaad.2010.05.038
更新日期：2011-08-01 00:00:00
abstract：BACKGROUND:Scleredema adultorum (SA) is a rare connective tissue disorder for which no treatment has proven to be effective. OBJECTIVE:Our purpose was to determine the effect of bath-PUVA therapy on SA. METHODS:Three patients were treated. Clinical evaluation of skin induration and thickness as well as ultrasonograph...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/s0190-9622(98)70244-0
更新日期：1998-02-01 00:00:00
abstract：:Pathologic T-cell activation is implicated in psoriasis progression. CD80, a costimulatory molecule involved in T-cell activation, likely plays a key role. IDEC-114, an IgG(1) anti-CD80 antibody, was evaluated for safety, pharmacokinetics, and preliminary clinical activity in this open-label, single-dose, dose-escalat...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1067/mjd.2002.124698
更新日期：2002-11-01 00:00:00
abstract：:Granuloma annulare (GA) represents a cutaneous reaction pattern of unknown cause with a variety of previously described potential disease associations and triggers. This review attempts to synthesize the available data regarding potential etiopathogenesis, reviews the available data on potential GA disease association...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章,评审
doi：10.1016/j.jaad.2015.03.055
更新日期：2016-09-01 00:00:00
abstract：:Intravascular lymphomatosis (IVL) is a rare type of lymphoma with a poor prognosis. Its distinctive clinical and histopathologic features are generated by the proliferation of neoplastic mononuclear cells within blood vessels. We describe a patient with IVL of the skin as a manifestation of a recurrent diffuse large B...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1067/mjd.2003.112
更新日期：2003-02-01 00:00:00
abstract：BACKGROUND:The impact of dermatology consultation on the care of children with oncologic conditions is unknown. OBJECTIVE:To review outpatient dermatology visits and the resulting impact on diagnosis and management of pediatric oncology patients. METHOD:Retrospective review of pediatric oncology patients with outpati...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/j.jaad.2017.06.005
更新日期：2017-11-01 00:00:00
abstract：:Extramedullary hematopoiesis (EMH) is a relatively rare, but well-documented, manifestation of chronic myeloproliferative disorders. Microscopically, foci of EMH consist of erythroid and myeloid precursors intermixed with megakaryocytes. It typically occurs in the spleen and liver, but very occasionally manifests as c...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/j.jaad.2007.10.489
更新日期：2008-04-01 00:00:00
abstract：BACKGROUND:There are limited data on the effectiveness of skin cancer prevention education and early detection programs at beaches. OBJECTIVES:We evaluate 4 strategies for addressing skin cancer prevention in beach settings. METHODS:This prospective study at 4 beaches included 4 intervention conditions: (1) education...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章,随机对照试验
doi：10.1016/j.jaad.2010.01.040
更新日期：2011-02-01 00:00:00
abstract：:POEMS syndrome is an unusual plasma cell dyscrasia with multisystemic manifestations featuring polyneuropathy, organomegaly, endocrinopathy, M protein and constant skin involvement. We have seen a 58-year-old white man presenting with generalized eruptive angiomas as a rare skin manifestation of this syndrome. We revi...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/s0190-9622(85)70123-5
更新日期：1985-05-01 00:00:00
abstract：BACKGROUND:Retrospective data have suggested the effectiveness of intravenous immunoglobulins (IVIG) for solar urticaria (SU). OBJECTIVE:We sought to prospectively assess the efficacy of IVIG for SU. METHODS:We conducted a multicentric phase II study to test the efficacy of a single course of IVIG (2 g/kg) in patient...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jaad.2014.07.023
更新日期：2014-11-01 00:00:00
abstract：:The clinical, electron microscopic, and freeze-fracture features of the skin of a harlequin fetus are described. Ultrastructural findings included large, concentric lamellar bodies, focal absence of intercellular stratum corneum lipid, and an increase in the size of desmosomes and the number of tight junctions. Althou...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/s0190-9622(89)70289-9
更新日期：1989-11-01 00:00:00
abstract：:A 60-year-old man with postpolycythemic myeloid metaplasia developed acute neutrophilic dermatosis (Sweet's syndrome), which responded to systemic steroid therapy. Six months later he developed acute myelogenous blast crisis refractory to leukemic induction therapy. ...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/s0190-9622(87)70061-9
更新日期：1987-02-01 00:00:00
abstract：:Mutations in the cathepsin C gene have recently been detected in Papillon-Lefèvre syndrome (PLS). Until now, 5 cases with the late-onset variation of this disease have been reported in the literature. The genetic background of this type of PLS is still unknown. We describe a 46-year-old woman with late-onset transgred...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章,评审
doi：10.1016/s0190-9622(03)01558-5
更新日期：2003-11-01 00:00:00
abstract：:Reflex sympathetic dystrophy (RSD) is a poorly understood neurovascular disorder characterized by pain, altered sensation, motor disturbance, soft tissue changes, vasomotor changes, and autonomic changes that occurs after trauma to an extremity. Unilateral leukonychia, Beau's lines, nailfold swelling, and nail clubbin...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/j.jaad.2007.02.028
更新日期：2008-02-01 00:00:00
abstract：:Significant progress has been made in skin replacement options in the past several decades. Although initially various materials have been used mainly for burn coverage, their application to dermatologic practice has increased significantly. We review the research, progress, and other aspects of wound coverage with sy...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章,评审
doi：10.1016/0190-9622(90)70279-q
更新日期：1990-10-01 00:00:00
abstract：:Skin cancer is less prevalent in people of color than in the white population. However, when skin cancer occurs in non-whites, it often presents at a more advanced stage, and thus the prognosis is worse compared with white patients. The increased morbidity and mortality associated with skin cancer in patients of color...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章,评审
doi：10.1016/j.jaad.2013.11.038
更新日期：2014-04-01 00:00:00
abstract：:Isolated plantar cerebriform collagenomas are a relatively rare type of connective tissue nevus. They have been suggested to be pathognomonic of Proteus syndrome. However, their presence is now considered to be a major criterion of Proteus syndrome, but the diagnosis of Proteus syndrome also requires the presence of o...
journal_title：Journal of the American Academy of Dermatology
pub_type： 杂志文章
doi：10.1016/j.jaad.2007.03.016
更新日期：2008-03-01 00:00:00
