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Severe progressive multisystem disorder involving the skin (dermal granula and ulcerations) and larynx (vocal cord granuloma). It is often lethal during childhood.

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Laryngeal and Ocular Granulation in Indian Children; LOGIC Syndrome.

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Unknown; fewer than 50 cases reported in the literature.

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Autosomal recessive.

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Caused by an inherited defect affecting the lamina lucida of the skin basal membrane layer.

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Demonstration of dermal and submucosal granulation with vocal cord involvement.

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Onset within 2 weeks of birth; death common in childhood. Hoarse, weak cry, vocal cord granulation, dystrophic changes in the nails, with recurrent loss of toenails and fingernails, dermal granuloma, skin ulceration, conjunctival scarring, amelogenesis imperfecta.

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Significant risk of airway obstruction because of the presence of a laryngeal web and large supraglottic and glottic nodules; epiglottic and supraglottic edema may be present with ulceration and contact bleeding, together with swollen and poorly defined cords.

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Permanent tracheotomies may be required, which may lead to granulation formation and obstruction distally in the trachea.

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Determined by degree of airway obstruction.

Hodges UM, Lloyd-Thomas A: Anaesthesia for airway obstruction in laryngo-onycho-cutaneous syndrome. Anaesthesia 48:503, 1993.  [PubMed: 8322991]
Phillips RJ, Atherton DJ, Gibbs ML, et al: Laryngo-onycho-cutaneous syndrome: An inherited epithelial defect. Arch Dis Child 70:319, 1994.  [PubMed: 8185366]

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