An inherited disorder characterized by dissominated connective
tissue nevi and osteopoikilosis.
Curth Syndrome; Schreus Syndrome; Disseminated
Dermatofibrosis with Osteopoikilosis; Dermato-Osteopoikilosis;
Dermatofibrosis Lenticularis Disseminata with Osteopoikilosis; Osteopathia
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.
Approximately 1:20,000 live births.
Autosomal dominant with incomplete penetrance.
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.
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
Evaluate osteomuscular repercussions
(clinical, radiographs, laboratory investigations including phosphocalcic
Only a few implications concerning venous/regional anesthesia
puncture sites that can be covered by skin lesions. Cautious
intraoperative positioning and padding is needed.
No known pharmacological
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
Nevin NC, Thomas PS, Davis RI, et al: Melorheostosis in a family with
autosomal dominant osteopoikilosis. Am J Med Genet