pediatrics
Cutis Laxa
Symptoms and Signs of Cutis Laxa
Inhereditary forms,dermal laxity may be present at birth or develop later; it occurs wherever the skin is normally loose and hanging in folds, most obviously on the face. Affected children have mournful or Churchillian facies and a hooked nose. The benign autosomal recessive form also causes intellectual disability and joint laxity. Gastrointestinal tract hernias and diverticula are common. If the disorder is severe, progressive pulmonary emphysema may precipitate cor pulmonale. Bronchiectasis, heart failure, and aortic aneurysms can also occur. In theacquired forms,presentation differs according to age of onset. There are two types. Type 1 typically manifests in adulthood and often also involves internal organs. Type 2 (postinflammatory elastolysis and cutis laxa [Marshall syndrome]) manifests in early childhood and only rarely involves internal organs. It is often preceded by an inflammatory phase. 