There is no consensus regarding the optimal treatment of LOC, and management is often based on single case reports or anecdotal experience. Treatment is difficult and often conservative with wound care and symptom control, such as with antibiotics and corticosteroids. Procedures such as tracheostomy, gastrostomy, and suprapubic catheterization can be utilized to treat strictures associated with the trachea, gastrointestinal tract, and urethra, respectively [19]. The main complication in patients with LOC syndrome is airway obstruction secondary to respiratory secretions as well as laryngeal and tracheal granulation tissue. Various treatments such as methylprednisolone and cyclophosphamide have not been associated with a successful outcome [21]. However, thalidomide markedly reduced granulation tissue and tracheal secretions in a single patient with LOC syndrome. Interruption of treatment resulted in recurrence of the granulation tissue, but reintroduction of thalidomide led to control [21]. Another therapeutic option for laryngeal stenosis is the short contact application of topical mitomycin C (2 mg/ml) applied on two occasions a month apart; in the treated patient, the airway remained patent and free of granulation tissue for a year after intervention [22]. Laser may also be useful therapy in the management of laryngeal granulation tissue [5, 20].

The treatment of ocular involvement is challenging and requires meticulous removal of granulation tissue from the conjunctivae, cornea, and nearby forniceal area and amniotic membrane transplantation to reduce ocular scarring [23]. The amniotic membrane is thought to provide laminin-332 to the healing ocular mucosa. The final consideration is iron supplementation if anemia is present.

LOC syndrome is a rare autosomal recessive subtype of JEB-other with a handful of reported cases in the literature. It is caused by several mutations affecting the N-terminus of the LAMA3 gene, whereas loss-of-function mutations affecting the same LAMA3 gene result in Herlitz JEB, emphasizing the clear genotype-phenotype correlation in this subtype of JEB.