Image taken from Clinical Guide to Oral Diseases.
A 62-year-old woman was referred by her family doctor for evaluation of several red spots on her lips, mouth, and the skin of her fingers.
The red spots had been present since childhood, but had become greater on the surface of her face over the last five years causing cosmetic problems and patient’s concern.
Her medical history revealed a chronic iron deficiency anemia which still remained despite the fact that the patient was in the post-menopause phase and had been treated occasionally with iron tablets. No other serious medical problems were recorded except for a few episodes of nose and gut bleeding which had caused her to ask for medical advice. She was a non-smoker and non-drinker.
The examination revealed numerous red vascular papules, variable in size, ranging from pin head-like lesions to small red plaques at the vermilion border of her lips, and on the tongue and buccal mucosae. A few asteroid-like red lesions, were also seen on the skin of her fingers and inside her nose which were responsible for her episodes of epistaxis.
Possible cause of her red spots: Rendu-Osler-Weber syndrome.
Rendu-Osler-Weber syndrome or hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant condition that affects blood vessels through out the body (telangiectasia; arteriovenous malformations) with a tendency for bleeding. This vascular dysplasia is commonly seen in oral, nasopharynx, lung, liver, spleen, gastrointestinal and urinary tracts, conjunctiva and the skin of arms and fingers.
Iron deficiency anemia is a very common complication induced by a series of episodes of blood loss through the nose (epistaxis) and gastrointestinal tract (melena stools) from telangiectic lesions.
Pulmonary hemorrhage is mainly found in patients older than 40 years old and with multiple visceral involvements, causing breathing problems, portal hypertension and liver cirrhosis.
Ischemic stroke is a rare yet serious complication in patients with HHT, and requires special care.