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Involutional type of entropion in a child with cutis laxa
  1. RIAD N MA'LUF,
  2. NICOLA G GHAZI
  1. Department of Ophthalmology, American University of Beirut- Medical Center, 113–6044 Beirut, Lebanon
  2. Department of Pathology
  1. MOUEEN N BU GHANIM,
  2. AYMAN N TAWIL
  1. Department of Ophthalmology, American University of Beirut- Medical Center, 113–6044 Beirut, Lebanon
  2. Department of Pathology
  1. RMALUF{at}cyberia.net.lb

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Editor,—The diffuse elastic tissue disease called cutis laxa (CL) is a serious, even lethal systemic illness, involving not only the skin but connective tissues throughout the body.1 The skin hangs in loose folds, producing the appearance of premature ageing. Internal manifestations such as emphysema, ectasia of the aorta, and multiple hernias are usually present.

We report a child with cutis laxa, who presented with an unusual ophthalmic manifestation of the disease.

CASE REPORT

Our patient, who is now a 4 year old boy and the third child to a normal first degree cousin couple, was noted to have redundant skin and a hoarse cry at the age of 3 months. Skin biopsy was consistent with cutis laxa (elastin stain showed focal thickening of the elastic fibres with tapered ends). His 7 month old sister was also diagnosed as having cutis laxa at 3 months of age. Her ophthalmic examination revealed no abnormalities. Otherwise, the family history was negative for such skin problems.

Recently, he presented to our clinic with a 2 month history of a red right eye. Examination revealed an entropion of the right lower lid (Fig 1 …

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