• Disease
  • Harlequin
  • Harlequin Ichthyosis
  • Philip H Fleckman

    Prominent publications by Philip H Fleckman

    KOL Index score: 18936

    BACKGROUND: Onychomycosis is a relatively common condition affecting toenails more than fingernails. It is caused predominantly by dermatophytes. Onychomycosis can cause pain and discomfort and has the potential to be a source of morbidity.

    OBJECTIVE: We evaluated the efficacy and safety of ciclopirox nail lacquer solution 8% used to treat onychomycosis of the toe in the United States and in centers worldwide.

    METHODS: Two identically designed, double-blind, vehicle controlled, parallel ...

    Also Ranks for: Ciclopirox Nail Lacquer |  treatment onychomycosis |  united states |  48 weeks |  topical solution
    KOL Index score: 13123

    BACKGROUND: Many chronic diseases, such as non-healing wounds are characterized by prolonged inflammation and respond poorly to conventional treatment. Bacterial biofilms are a major impediment to wound healing. Persistent infection of the skin allows the formation of complex bacterial communities termed biofilm. Bacteria living in biofilms are phenotypically distinct from their planktonic counterparts and are orders of magnitude more resistant to antibiotics, host immune response, and ...

    Also Ranks for: Aureus Biofilm |  gene expression |  cytokine production |  chronic wounds |  bcm pcm
    KOL Index score: 10774

    Flaky tail (gene symbol ft) is an autosomal recessive mutation in mice that results in a dry, flaky skin, and annular tail and paw constrictions in the neonatal period. Previous studies demonstrated that the ft mutation maps to the central region of mouse chromosome 3, in the vicinity of the epidermal differentiation complex, a gene locus that includes many nonkeratin genes expressed in epidermis. In this study we report a detailed characterization of the flaky tail mouse. Affected ...

    Also Ranks for: Flaky Tail |  animal model |  ichthyosis vulgaris |  profilaggrin filaggrin |  skin disease
    KOL Index score: 10645

    Filaggrin is an intermediate filament-associated protein which functions to aggregate keratin intermediate filaments in the stratum corneum of mammalian epidermis. It is synthesized as a large precursor protein, profilaggrin, that consists of multiple filaggrin units and is localized in keratohyalin granules. In this report, we describe the characterization of cosmid genomic clones containing the human profilaggrin gene coding for 11 complete filaggrin repeats of 324 amino acids each. At ...

    Also Ranks for: Calcium Binding |  amino terminus |  profilaggrin filaggrin |  cultured cloning |  sequence homology
    KOL Index score: 10630

    BACKGROUND: Autosomal recessive congenital ichthyosis (ARCI) is a rare hereditary disorder of cornification. Mutations in the transglutaminase-1 (TGM1) gene, which encodes for the epidermal enzyme transglutaminase-1 (TGase-1), are one of the causes of ARCI.

    METHODS: The TGM1 mutation spectrum was characterised and genotype-phenotype correlations investigated in 104 patients with ARCI ascertained through the National Registry for Ichthyosis and Related Disorders in the USA. Methods: ...

    Also Ranks for: Patients Arci |  recessive congenital |  tgm1 mutations |  collodion membrane |  ichthyosiform erythroderma
    KOL Index score: 9938

    Filaggrin is an intermediate filament (IF)-associated protein that aggregates keratin IFs in vitro and is thought to perform a similar function during the terminal differentiation of epidermal keratinocytes. To further explore the role of filaggrin in the cytoskeletal rearrangement that accompanies epidermal differentiation, we generated keratinocyte cell lines that express human filaggrin using a tetracycline-inducible promoter system. Filaggrin expression resulted in reduced ...

    Also Ranks for: Cell Adhesion |  human filaggrin |  epidermal keratinocytes |  intermediate filament |  terminal differentiation
    KOL Index score: 9852

    BACKGROUND: Inherited ichthyoses belong to a large, clinically and etiologically heterogeneous group of mendelian disorders of cornification, typically involving the entire integument. Over the recent years, much progress has been made defining their molecular causes. However, there is no internationally accepted classification and terminology.

    OBJECTIVE: We sought to establish a consensus for the nomenclature and classification of inherited ichthyoses.

    METHODS: The classification ...

    Also Ranks for: Inherited Ichthyoses |  ichthyosis consensus |  umbrella term |  mendelian disorders |  entire integument
    KOL Index score: 9546

    Skin biopsies and scale samples from nine infants and one fetus affected with harlequin ichthyosis (HI) were obtained from eight families. Epidermal differentiation was examined by morphologic and biochemical criteria and cell culture studies. Two striking abnormalities were identified; first, keratin and filaggrin expression were abnormal and varied between cases, and, second, in all cases lamellar granules were absent or abnormal, and intercellular lamellae within the stratum corneum ...

    Also Ranks for: Lamellar Granules |  harlequin ichthyosis |  inborn error |  protein expression |  stratum corneum
    KOL Index score: 9451

    Two monoclonal antibodies (AKH1 and AKH2) were elicited with partially purified human filaggrin and characterized by immunohistochemistry on normal and abnormal skin biopsies, immunoblotting techniques, and antigen purification. Both antibodies react strongly with the granular cell layer consistent with the distribution of keratohyalin and show a more diffuse reaction with the stratum corneum in normal skin biopsies. Reaction in cultured human keratinocytes is limited to ...

    Also Ranks for: Monoclonal Antibodies |  filaggrin proteins |  ichthyosis vulgaris |  stratum corneum |  human epidermal
    KOL Index score: 9153

    Chronic wounds are a major clinical problem that lead to considerable morbidity and mortality. We hypothesized that an important factor in the failure of chronic wounds to heal was the presence of microbial biofilm resistant to antibiotics and protected from host defenses. A major difficulty in studying chronic wounds is the absence of suitable animal models. The goal of this study was to create a reproducible chronic wound model in diabetic mice by the application of bacterial biofilm. ...

    Also Ranks for: Chronic Wounds |  delayed wound healing |  pseudomonas aeruginosa |  biofilm challenge |  diabetic mice
    KOL Index score: 8831

    BACKGROUND: Onychomycosis is a widespread refractory disease deleteriously affecting quality of life via social stigma and disrupting daily activities. Many physicians perceive onychomycosis as a cosmetic rather than a medical problem.

    OBJECTIVE: Our purpose was to develop a questionnaire-based instrument to quantify the impact of onychomycosis on patients' quality of life.

    METHODS: The questionnaire was developed and validated in a multinational cross-sectional study. Completed ...

    Also Ranks for: Quality Life |  impact onychomycosis |  daily activities |  social stigma |  medical problem
    KOL Index score: 8582

    Atopic disease, including atopic dermatitis (eczema), allergy and asthma, has increased in frequency in recent decades1 and now affects ∼20% of the population in the developed world. Twin and family studies have shown that predisposition to atopic disease is highly heritable2. Although most genetic studies have focused on immunological mechanisms, a primary epithelial barrier defect has been anticipated3. Filaggrin is a key protein that facilitates terminal differentiation of the ...

    Also Ranks for: Atopic Dermatitis |  function variants |  major predisposing factor |  protein filaggrin |  genetic studies
    KOL Index score: 8571

    Bacterial biofilm has been shown to play a role in delaying wound healing of chronic wounds, a major medical problem that results in significant health care burden. A reproducible animal model could be very valuable for studying the mechanism and management of chronic wounds. Our previous work showed that Pseudomonas aeruginosa (PAO1) biofilm challenge on wounds in diabetic (db/db) mice significantly delayed wound healing. In this wound time course study, we further characterize the ...

    Also Ranks for: Wound Healing |  pseudomonas aeruginosa |  mouse model |  bacterial biofilm |  db mice
    KOL Index score: 8524

    BACKGROUND: Onychomycosis, a fungal infection of the nail bed, is responsible for up to 50% of nail disorders. Although several surveys have been conducted in different parts of the world, there have been no multicenter epidemiologic surveys of onychomycosis in North America.

    OBJECTIVE: A 12-center study was undertaken to (1) determine the frequency of onychomycosis, (2) identify organisms recovered from the nails, and (3) determine the antifungal susceptibility of isolates.

    METHODS: A ...

    Also Ranks for: Antifungal Susceptibility |  fungal isolates |  north america |  onychomycosis study |  canada child child
    KOL Index score: 8356

    Bacteria colonizing chronic wounds are believed to exist as polymicrobial, biofilm communities; however, there are few studies demonstrating the role of biofilms in chronic wound pathogenesis. This study establishes a novel method for studying the effect of biofilms on the cell types involved in wound healing. Cocultures of Staphylococcus aureus biofilms and human keratinocytes (HK) were created by initially growing S. aureus biofilms on tissue culture inserts then transferring the ...

    Also Ranks for: Aureus Biofilms |  human keratinocytes |  bcm pcm |  cell viability |  chronic wounds


    Philip H Fleckman: Influence Statistics

    Sample of concepts for which Philip H Fleckman is among the top experts in the world.
    Concept World rank
    biopsy dermatologist #1
    khg light microscopy #1
    neutrophil‐mediated damage #1
    ichthyosis keratinocytes #1
    methodology discuss #1
    gene profilaggrin #1
    inherited scaling #1
    components nail #1
    topical treatment regimen #1
    program nail #1
    filaggrin repeats individuals #1
    prpaffected individuals #1
    calpain epidermis #1
    injury gingival cells #1
    normal cultures 20 #1
    autosomaldominant prp prp #1
    painstaking effort patients #1
    true fungal infection #1
    cytoskeleton desmosomal #1
    filaggrinexpressing lines #1
    solubilized basement membrane #1
    profillagrin #1
    ivaffected keratinocytes #1
    cell lines filaggrin #1
    doublestable cell lines #1
    coequal expression #1
    detachment gingival #1
    biopsy humans onychomycosis #1
    culture staphylococci #1
    familial pityriasis mutations #1
    keratinocytes cultured subjects #1
    anucleate cornified layers #1
    peptides profilaggrin #1
    activity harlequin cultures #1
    nail plate matrix #1
    layer calpain #1
    flg epidermis #1
    keratinocytes lamellar infant #1
    mature human filaggrin #1

    Key People For Harlequin Ichthyosis

    Top KOLs in the world
    Philip H Fleckman
    harlequin ichthyosis chronic wounds granular layer
    Masashi Akiyama
    harlequin ichthyosis ichthyosiform erythroderma atopic dermatitis
    Beverly A Dale
    harlequin ichthyosis stratum corneum epithelial cells
    Hiroshi Shimizu
    bullous pemphigoid epidermolysis bullosa prenatal diagnosis
    Virginia P Sybert
    turner syndrome prenatal diagnosis epidermolysis bullosa
    Edel A O’Toole
    type vii collagen pachyonychia congenita harlequin ichthyosis

    Philip H Fleckman:Expert Impact

    Concepts for whichPhilip H Fleckmanhas direct influence:Harlequin ichthyosis,  Granular layer,  Cultured keratinocytes,  Ichthyosis vulgaris,  Chronic wounds,  Streptococcus pyogenes,  Wound healing,  Inherited ichthyoses.

    Philip H Fleckman:KOL impact

    Concepts related to the work of other authors for whichfor which Philip H Fleckman has influence:Atopic dermatitis,  Wound healing,  Skin barrier,  Stratum corneum,  Food allergy,  Harlequin ichthyosis,  Staphylococcus aureus.



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    Department of Medicine, Division of Dermatology, University of Washington, Seattle | Department of Medicine, Division of Dermatology, University of Washington, Seattle, WA, USA | Department of Medicine, University of Washington, Seattle WA, United St