Lipidproteinosis (urbach-wiethe Syndrome).

SIEBENMANN (1908) described lipidproteinosis, and the condition was subsequently reported as a clinical entity by Urbach and Wiethe (1929). During infancy and early childhood a structureless eosinophilic substance develops under the epithelium of the skin and upper respiratory tract. Hoarseness is followed by a skin eruption giving a pale, yellow-brown, pock-marked appearance, especially on the face and scalp, with the extensor surfaces (e.g. the elbows) showing hyperkeratotic lesions. Localized deposits appear as white plaques on the pharyngeal wall and on the tongue which becomes more and more immobilized. Respiratory distress due to laryngeal stenosis in later life may require tracheostomy, but otherwise the prognosis is good. The lid margins usually show discrete nodules resembling solid beads of yellow-brown wax among the lashes. In time these can reach up to 5 mm. in diameter and approach the posterior borders of the lid edges, but the conjunctiva is not involved.