Laryngo-Onycho-Cutaneous Syndrome Managed With Thalidomide.

Abstract

Laryngo-onycho-cutaneous syndrome (LOCS), a subtype of junctional epidermolysis bullosa, results from mutations affecting laminin-332 subunits which alter epidermal-dermal adhesion. LOCS phenotype includes cutaneous, ocular, and laryngeal granuloma formation and scarring. Management relies on multidisciplinary symptomatic care, and successful disease-modifying therapies are infrequently documented. Here, we describe a 7-year-old female with characteristic manifestations of LOCS who has had a significant response to oral thalidomide.