A child with laryngo-onychocutaneous syndrome partially responsive to treatment with thalidomide.
Laryngo-onychocutaneous syndrome (LOCS) is a condition characterized by erosive or ulcerative skin lesions associated with excessive granulation tissue, at sites of trauma such as the digits, elbows and knees. Similar lesions can occur within the conjunctival mucosa, leading to corneal scarring and blindness. The main complications, however, occur in the respiratory tract, where a similar process of erosions and subsequent formation of granulation tissue causes airway obstruction which may lead to premature death. LOCS is now believed to be a nonblistering variant of junctional epidermolysis bullosa and to date there are no efficacious treatments available. We report a 16-year-old girl with LOCS who failed to respond to methylprednisolone and cyclophosphamide, but had a partial response to oral thalidomide with marked decrease in granulation tissue and tracheal secretions. Interruption of treatment resulted in prompt resurgence of the granulation tissue which was again controlled by reintroduction of thalidomide. We propose that in the absence of effective therapies for LOCS, a trial of thalidomide in these patients should be considered.
Strauss
R M
RM
Department of Dermatology, St James's University Hospital, Leeds, UK.
Bäte
J
J
Nischal
K K
KK
Clayton
T
T
Gooi
J
J
Darling
J C
JC
Newton-Bishop
J A
JA
D000293:Adolescent; D003229:Conjunctival Diseases / Q000188:drug therapy* / Q000209:etiology; D004820:Epidermolysis Bullosa / Q000188:drug therapy*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007818:Laryngeal Diseases / Q000188:drug therapy* / Q000209:etiology; D009260:Nail Diseases / Q000188:drug therapy* / Q000209:etiology; D013577:Syndrome; D013792:Thalidomide / Q000627:therapeutic use*; D014456:Ulcer / Q000188:drug therapy / Q000209:etiology
D007166:Immunosuppressive Agents; D013792:Thalidomide