++
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;
LOGIC Syndrome.
++
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
trachea.
++
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]