Severe progressive multisystem disorder involving the
skin (dermal granula and ulcerations) and larynx (vocal cord granuloma). It is
often lethal during childhood.
Laryngeal and Ocular Granulation in Indian Children;
Unknown; fewer than 50 cases reported in the literature.
Caused by an inherited defect affecting the lamina
lucida of the skin basal membrane layer.
Demonstration of dermal and submucosal granulation with
vocal cord involvement.
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.
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.
Permanent tracheotomies may be required,
which may lead to granulation formation and obstruction distally in the
Determined by degree of airway
Hodges UM, Lloyd-Thomas A: Anaesthesia for airway obstruction in
laryngo-onycho-cutaneous syndrome. Anaesthesia
Phillips RJ, Atherton DJ, Gibbs ML, et al: Laryngo-onycho-cutaneous
syndrome: An inherited epithelial defect. Arch Dis Child