Thursday, 23 January 2014

Laryngo-oculo-cutaneous Syndrome (JEB)

Laryngo-oculo-cutaneous syndrome (LOC), or Shabbir’s syndrome, presents at birth with blistering and erosions that typically favors the head and neck. Nail dystrophy and granulation tissue commonly are observed, while milia and scarring are less common. LOC is predominantly reported in the state of Punjab, India. Oral erosions, dental enamel problems, dental caries, and ocular (eye) erosions and other complications are common. Laryngeal erosions and related complications are a key feature and may lead to airway obstruction and death. Growth retardation and anemia may occur, but problems with the intestines/gastrointestinal tract or genitourinary tract have not been reported. 

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