Mucocutaneous pigmented spots and oral myxomas: the oral manifestations of the complex of myxomas, spotty pigmentation, and endocrine overactivity.
The complex of myxomas, spotty pigmentation, and endocrine overactivity is a recently recognized syndrome, transmitted as an autosomal dominant trait. The most serious component of the disorder is cardiac myxoma, which has caused the death of one fourth of the affected patients and serious disability in an equal number. It is, therefore, important to recognize patients at risk from the syndrome and, in particular, to test them for cardiac myxoma. Fortunately, in many patients the myxoma complex has a clearly visible marker: mucocutaneous pigmentation. Among 58 patients with the syndrome, spotty facial pigmentation was present in 36 (62%), and 29 (50%) of these also had pigmented spots on their lips. This type and distribution of pigmentation should be a clue to the possible presence of the complex of myxomas, spotty pigmentation, and endocrine overactivity, and patients thus affected should be referred for further investigation. Oral cavity myxoma(s) occurred in four patients with the syndrome.
D000328:Adult; D005153:Facial Neoplasms / Q000235:genetics* / Q000473:pathology; D005260:Female; D006338:Heart Neoplasms / Q000235:genetics / Q000473:pathology; D006801:Humans; D007223:Infant; D008297:Male; D009061:Mouth Mucosa / Q000473:pathology; D009062:Mouth Neoplasms / Q000235:genetics* / Q000473:pathology; D009377:Multiple Endocrine Neoplasia / Q000235:genetics* / Q000473:pathology; D009232:Myxoma / Q000235:genetics* / Q000473:pathology; D010859:Pigmentation Disorders / Q000235:genetics* / Q000473:pathology; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology; D013577:Syndrome