A Unique Case of Trichorrhexis Nodosa—"Bamboo Hairs"

Netherton, E. W.

doi:10.1001/archderm.1958.01560100059009

Cited by 254 publications

(129 citation statements)

References 2 publications

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“…52,61,62,215,216 However, one should consider that a reduced or absent SG suggestive for IV can also be seen in acquired ichthyosis, NS, Refsum syndrome, TTDs, or Conradi-Hünermann-Happle syndrome. Hair mounts can demonstrate bamboo hairs (trichorrhexis invaginata) in NS 123 ; although not invariably present, bamboo hairs are pathognomonic of this disorder. Parakeratosis and hypergranulosis is regarded a histopathological clue to loricrin keratoderma.…”

Section: Use Of Ultrastructural Analysesmentioning

confidence: 99%

Revised nomenclature and classification of inherited ichthyoses: Results of the First Ichthyosis Consensus Conference in Sorèze 2009

Oji

Tadini

Akiyama

et al. 2010

Journal of the American Academy of Dermatology

660

704

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Section: Use Of Ultrastructural Analysesmentioning

confidence: 99%

Revised nomenclature and classification of inherited ichthyoses: Results of the First Ichthyosis Consensus Conference in Sorèze 2009

Oji

Tadini

Akiyama

et al. 2010

Journal of the American Academy of Dermatology

660

704

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“…Spink5 is expressed in the differentiated layers of stratified epithelial tissue, and mutations in this gene have been shown to be the cause of autosomal-recessive Netherton syndrome (Netherton, 1958). Patients with this condition suffer from frequent bacterial infections.…”

Section: Expression Of Epithelial-specific Genes During Cervical Softmentioning

confidence: 99%

Cervical remodeling during pregnancy and parturition: molecular characterization of the softening phase in mice

Read¹,

Word²,

Ruscheinsky³

et al. 2007

Reproduction

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217

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Cervical remodeling during pregnancy and parturition is a single progressive process that can be loosely divided into four overlapping phases termed softening, ripening, dilation/labor, and post partum repair. Elucidating the molecular mechanisms that facilitate all phases of cervical remodeling is critical for an understanding of parturition and for identifying processes that are misregulated in preterm labor, a significant cause of perinatal morbidity. In the present study, biomechanical measurements indicate that softening was initiated between gestation days 10 and 12 of mouse pregnancy, and in contrast to cervical ripening on day 18, the softened cervix maintains tissue strength. Although preceded by increased collagen solubility, cervical softening is not characterized by significant increases in cell proliferation, tissue hydration or changes in the distribution of inflammatory cells. Gene expression studies reveal a potentially important role of cervical epithelia during softening and ripening in maintenance of an immunomucosal barrier that protects the stromal compartment during matrix remodeling. Expression of two genes involved in repair and protection of the epithelial permeability barrier in the gut (trefoil factor 1) and skin (serine protease inhibitor Kazal type 5) were increased during softening and/or ripening. Another gene whose function remains to be elucidated, purkinje cell protein 4, declines in expression as remodeling progressed. Collectively, these results indicate that cervical softening during pregnancy is a unique phase of the tissue remodeling process characterized by increased collagen solubility, maintenance of tissue strength, and upregulation of genes involved in mucosal protection.

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“…NS involves both cutaneous and immunological abnormalities, including congenital ichthyosiform erythroderma, pro nounced peeling of the skin with detachment of the stratum corneum (SC) from the under lying epidermis, severe atopy with elevated serum IgE, and a characteristic hair shaft defect (Comel, 1949;Netherton, 1958;Judge et al, 1994;Hausser and Anton Lamprecht, 1996). Collec tively, these detriments represent a serious chal lenge to neonate health and promote frequent complications, including hypernatremic dehy dration, failure to thrive and recurrent bacterial infections that can be life threatening.…”

mentioning

confidence: 99%

Transgenic kallikrein 5 mice reproduce major cutaneous and systemic hallmarks of Netherton syndrome

Furio¹,

Veer²,

Jaillet³

et al. 2014

J Cell Biol

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A Unique Case of Trichorrhexis Nodosa—"Bamboo Hairs"

Cited by 254 publications

References 2 publications

Revised nomenclature and classification of inherited ichthyoses: Results of the First Ichthyosis Consensus Conference in Sorèze 2009

Revised nomenclature and classification of inherited ichthyoses: Results of the First Ichthyosis Consensus Conference in Sorèze 2009

Cervical remodeling during pregnancy and parturition: molecular characterization of the softening phase in mice

Transgenic kallikrein 5 mice reproduce major cutaneous and systemic hallmarks of Netherton syndrome

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