Laryngo-Onycho-Cutaneous (LOC) Syndrome

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.

Autosomal recessive.

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.