A child with laryngo-onychocutaneous syndrome partially responsive to treatment with thalidomide.

[junctional epidermolysis bullosa]

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 .

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Diseases presenting "where a similar process of erosions and subsequent formation of granulation tissue causes airway obstruction which may lead to premature death" symptom

junctional epidermolysis bullosa

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