BOS; Buschke-Ollendorf syndrome; Dermatofibrosis lenticularis disseminata with osteopoicilie; Dermatoosteopoicilosis; Disseminated dermatofibrosis with osteopoicilosis; OMIM 166700
HistoryThis section has been translated automatically.
Stieda, 1905; Albers-Schönberg, 1916; Buschke and Ollendorff-Curth, 1928
DefinitionThis section has been translated automatically.
Autosomal dominant inherited combination of multiple connective tissue nevi with osteopoicilie.
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EtiopathogenesisThis section has been translated automatically.
Loss-of-function mutation in the LEMD3 gene mapped on gene locus 12q14.
ManifestationThis section has been translated automatically.
Mostly adult, first skin changes possible in childhood.
LocalizationThis section has been translated automatically.
Extremities; distal trunk parts; hyperdense bone foci near the epiphysis.
Clinical featuresThis section has been translated automatically.
Sclerotic areas in the bone without pathological significance, accompanied by anomalies in the anatomically associated soft tissues such as dermatosclerosis, muscle atrophy, lymphedema, as well as multiple, 0.2-1.0 cm large, firm, symmetrically or asymmetrically distributed, skin-coloured nodules and nodules.
HistologyThis section has been translated automatically.
Corial compression of connective tissue with accumulation of broad elastic fibres.
Differential diagnosisThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Albers-Schonberg HE (1916) A rare, previously unknown structural anomaly of the skeleton. Progress Roentgenstr 23: 174-177
- Buschke A, Ollendorf-Curth H (1928) A case of Dermatofibrosis lenticularis disseminata and Osteopathia condensans disseminata. Dermatol Wochenschr 86: 257-260
- Danielson L, Midtgaard K, Christensen HE (1969) Osteopoicilosis associated with dermatofibrosis lenticularis disseminata. Arch Dermatol 100: 465
- Drouin CA et al (2002) The association of Buschke-Ollendorf syndrome and nail-patella syndrome. J Am Acad Dermatol 46: 621-625
- Kawamura A et al (2005) Buschke-Ollendorff syndrome: three generations in a Japanese family. Pediatric dermatol 22: 133-137
- Stieda A (1905) On circumscribed bone densification in the area of the substantia spongiosa in x-ray images. Beitr Klin Chir 45: 700-703
Incoming links (10)Connective tissue nevus; Connective tissue nevus lumbosacral; Dermatofibrosis lenticularis disseminata with osteopoicilie; Elastic nevus; Elastin gene; Juvenile elastoma; Malformation lymphatic; Melorheostosis; Ollendorf-curth, helen; Pseudoxanthoma elasticum;
Outgoing links (5)Albers-schönberg, heinrich ernst; Connective tissue nevus; Lymphedema, primary; Pseudoxanthoma elasticum; Xanthoma tuberosum;
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