Laryngo-onycho-cutaneous syndrome: an inherited epithelial defect.

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

“…Involvement of the trachea is also documented in EB 128 and laryngoonycho-cutaneous (LOC) syndrome. [150][151][152] Relatively mild stridor may be managed with nebulized epinephrine or dexamethasone. However, with more significant laryngeal involvement, it may be necessary to perform a tracheostomy to maintain the airway.…”

Visual Rehabilitation in Laryngo-Onycho-Cutaneous (LOC) Syndrome

“…However, patients who survive the early neonatal period often survive to adulthood and symptoms of hoarseness and ulceration slowly improve with age. As granulation tissue accumulates in the larynx, a permanent tracheostomy often becomes necessary to ensure a clear respiratory passageway [1][2][3]5]. Clinical criteria for diagnosis of LOC syndrome can be found in Table 1 [3].…”

Aggressive periodontitis in a young Pakistani female with laryngo-onycho-cutaneous syndrome

“…LOC syndrome usually starts within two-weeks after birth and the affected children present with hoarseness in voice, dystrophic changes in nails, chronic bleeding, crusted lesions of facial skin and corneal scarring leading to blindness (1,2). The ulcers do not respond to medications including antibiotics, anti-tuberculosis drugs, dapsone or steroids.…”

“…Laryngo-onycho-cutaneous (LOC) syndrome is a homozygous recessive condition characterized by proliferation of dermal and mucosal granulation tissue and progressive scarring of the conjunctiva and cornea (1). LOC syndrome has been associated with mutation in the gene encoding laminin alpha-3a on chromosome 18q11.2 (2).…”

“…Respiratory tract complications of LOC syndrome are associated with erosions and subsequent formation of granulation tissue causing airway obstruction that may lead to premature death (3). Orofacial manifestations of LOC syndrome include enamel hypoplasia, dental caries, gingival ulcerations, mucosa-covered nodules of the hard palate, and erosion of the lower lip and nares (1)(2)(3).…”