<\/span><\/h2>\nBuschke-Ollendorff syndrome refers to a rare, hereditary<\/span> disorder affected connective tissues. Also known as disseminated lenticularis dermatofibrosis, dermatoosteopoikilosis and family<\/span> cutaneous<\/span> collagenome<\/p>\n<\/span>Who gets Buschke-Ollendorff syndrome?<\/span><\/h2>\nBuschke-Ollendorff syndrome occurs in 1 in 20,000 live births worldwide. There seems to be no race, sexual or geographic predilection<\/span>. Lesions are present at birth.<\/p>\n\n
\n
\n
\n<\/div><\/div><\/div><\/div>\n
<\/span>What causes Buschke-Ollendorff syndrome?<\/span><\/h2>\nBuschke-Ollendorff syndrome is a genetic<\/span> disease. The underlying cause of Buschke-Ollendorff syndrome is mutation<\/span> of the LEMD3<\/em> gene<\/span>, which provides instructions for the manufacture of proteins that support connective tissues.<\/p>\n\n- The inheritance is in a autosomal<\/span> recessive pattern (a defective gene must be inherited from both parents).<\/li>\n
- May result from abnormal regulation of the the extracellular matrix<\/span>, resulting in an accumulation of elastin<\/span> at dermal<\/span> fur cape<\/li>\n<\/ul>\n
<\/span>What are the clinical features of Buschke-Ollendorff syndrome?<\/span><\/h2>\nBuschke-Ollendorff syndrome is characterized by a small connective tissue<\/span> naevi<\/span>.<\/p>\n\n- The torso and extremities are more frequently affected.<\/li>\n
- The nevi are elastomas<\/span> (made of elastin fibers), collagenomas<\/span> (Made of collagen<\/span> fibers) or fibroids<\/span> (made of fibrous tissue).<\/li>\n
- They are slightly raised, yellowish papules<\/span>, nodules<\/span> or plates<\/span>.<\/li>\n
- These lesions are painless and do not itch.<\/li>\n<\/ul>\n
The non-cutaneous but significant features of Buschke-Ollendorff syndrome include:<\/p>\n
\n- Osteopoikilosis (areas of increased bone density in the long bones of the extremities)<\/li>\n
- Nasolacrimal duct obstruction (tear)<\/li>\n
- Amblyopia (lazy eye) or strabismus (strabismus)<\/li>\n
- \nBenign<\/span> lymphoid<\/span> hyperplasia<\/span> (growth of lymphatic tissue)<\/li>\n
- Short stature<\/li>\n
- Mellitus diabetes<\/li>\n
- \nAortic<\/span> stenosis<\/span> (restricted heart valve)<\/li>\n
- Hearing impairment \/ deafness.<\/li>\n<\/ul>\n\n
<\/span>Connective tissue nevi<\/span><\/h5>\n<\/section>\n\n\n
\n
\n<\/div>\n
Connective tissue nevi in Buschke Ollendorf syndrome<\/p>\n
\n
\n<\/div>\n
Connective tissue nevi<\/p>\n
\n
\n<\/div>\n
Connective tissue nevi<\/p>\n<\/p><\/div>\n<\/section>\n
<\/div>\n
<\/span>How is Buschke-Ollendorff syndrome treated?<\/span><\/h2>\nTreatment for Buschke-Ollendorff syndrome may include:<\/p>\n
\n- Surgical excision<\/span> of connective tissue nevi for aesthetic reasons<\/li>\n
- Surgical treatment of deafness, if indicated.<\/li>\n
- Surgical treatment or other treatment of associated disorders, such as diabetes mellitus or aortic stenosis.<\/li>\n<\/ul>\n
\n
\n
\n
\n<\/div><\/div><\/div><\/div>\n
<\/span>What is the result of Buschke-Ollendorff syndrome?<\/span><\/h2>\nThe outcome of Buschke-Ollendorff syndrome depends on the associated disorders.<\/p>\n
\n- A leading cause of death related to Buschke-Ollendorff syndrome is the development of areas of bone density in bone cancers such as osteosarcoma, chondrosarcoma, or giant cell<\/span> tumors <\/li>\n
- When there are other disorders such as aortic stenosis or diabetes mellitus, the forecast<\/span> can be serious<\/li>\n<\/ul>\n<\/section>","protected":false},"excerpt":{"rendered":"
\u00bfQu\u00e9 es Buschke-Ollendorff? s\u00edndrome? El s\u00edndrome de Buschke-Ollendorff se refiere a un raro, hereditario trastorno afect\u00f3 los tejidos conectivos. Tambi\u00e9n se conoce como dermatofibrosis lenticularis diseminada, dermatoosteopoiquilosis y familiar cut\u00e1neo…<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[204],"tags":[],"class_list":["post-7579","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/7579","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/comments?post=7579"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/7579\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=7579"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=7579"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=7579"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}