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An inherited disorder characterized by dissominated connective tissue nevi and osteopoikilosis.

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Curth Syndrome; Schreus Syndrome; Disseminated Dermatofibrosis with Osteopoikilosis; Dermato-Osteopoikilosis; Dermatofibrosis Lenticularis Disseminata with Osteopoikilosis; Osteopathia Condensans Disseminata.

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Named after Abraham Buschke (1868-1943), a German dermatologist and Helen Ollendorff Curth (born 1899), a German-American dermatologist, who first described the disorder in 1928.

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Approximately 1:20,000 live births.

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Autosomal dominant with incomplete penetrance.

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Unknown.

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Characterized by slightly yellowish papules and nodules distributed symmetrically or asymmetrically on the trunk and extremities with induration of the skin and subcutaneous tissues. These skin changes are also called dermatofibrosis lenticularis disseminata. Radiographs show circumscribed sclerotic areas near the epiphyses and metaphyses of many bones, particularly of the pelvis and extremities.

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Patients can have short stature and often present with hoarseness. Clinical signs concern essentially the skin (dermatofibrosis lenticularis disseminata, palmoplantar hyperkeratosis, connective tissue nevi) and locomotor organs (epiphyseal, diaphyseal or metaphyseal anomalies, osteopoikilosis, osteosclerosis, melorheostosis (cortical thickening of the long bones often associated with pain), stiff joints, muscle fibrosis and contractures). Other features can include otosclerosis, cleft lip palate and insulin-dependent diabetes.

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Evaluate osteomuscular repercussions (clinical, radiographs, laboratory investigations including phosphocalcic metabolism).

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Only a few implications concerning venous/regional anesthesia puncture sites that can be covered by skin lesions. Cautious intraoperative positioning and padding is needed.

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No known pharmacological implications.

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Gunal-Seber-Basaran Syndrome (Dacryocystitis-Osteopoikilosis): Autosomal dominant with pigmented nevi, osteosclerosis, or osteopetrosis and defect of lacrimal system that may be considered in case parasympatholytic drugs are used.

Buschke A, Ollendorff-Curth H: Ein Fall von Dermatofibrosis lenticularis disseminata und Osteopathia condensans disseminata. Dermatol Wochenschrift (Hamburg) 86:257, 1928.
Kawamura A, Ochiai T, Tan-Kinashita M, Suzuki H: Buschke-Ollendorff syndrome: three generations in a Japanese family. Pediatr Dermatol 22:133, 2005.  [PubMed: 15804302]
Nevin NC, Thomas PS, Davis RI, et al: Melorheostosis in a family with autosomal dominant osteopoikilosis. Am J Med Genet 82:409, 1999.  [PubMed: 10069713]

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