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Buschke-Ollendorff syndrome in a 6-year-old patient: clinical and histopathological aspects of a rare disease.
Acta Dermatovenerol Alp Pannonica Adriat
March 2020
Department of Clinical and Molecular Sciences, Dermatological clinic, Polytechnic University of the Marche Region, Ancona, Italy.
Buschke-Ollendorff syndrome (BOS) is a rare genetic hereditary genodermatosis characterized by benign skeletal and cutaneous lesions. Skeletal alterations known as osteopoikilosis (OPK) or "spotted bone disease" are asymptomatic areas of sclerosing dysplasia. Two skin lesion patterns have been described because they may be of either elastic tissue (juvenile elastoma) or collagenous composition (dermatofibrosis lenticularis disseminata).
View Article and Find Full Text PDFThe Buschke-Ollendorff syndrome: a case report of simultaneous osteo-cutaneous malformations in the hand.
BMC Res Notes
June 2016
Department of Hand Plastic and Reconstructive Surgery, Cantonal Hospital, St. Gallen, Switzerland.
Background: We describe a male with functionally impairing radial deviation of the thumb who presented to us at 24 years of age. Two sclerotic skin lesions had been excised 7 years before because of consecutive skin contracture. Latest radiological examination showed a spotted pattern consistent with osteopoikilosis.
View Article and Find Full Text PDFBuschke-Ollendorff syndrome presenting as a painful nodule.
JAAD Case Rep
March 2015
Dermatology Service, Tripler Army Medical Center, Honolulu, Hawaii.
Buschke-Ollendorff syndrome: a novel case series and systematic review.
Br J Dermatol
April 2016
Pediatrics Section of Dermatology, Hospital for Sick Children, Toronto, ON, Canada.
Buschke-Ollendorff syndrome (BOS) is a rare, often benign, autosomal skin disorder. BOS commonly presents with nontender connective tissue naevi and sclerotic bony lesions (osteopoikilosis [OPK]). Herein, we summarize the presenting features of BOS and potential associations by conducting a systematic review of the literature and summarizing a cohort seen at the Hospital for Sick Children (HSC), Toronto, Canada.
View Article and Find Full Text PDFPapular elastorrhexis. report of five cases.
Dermatology
December 2002
Department of Dermatology, University of Basel, Switzerland.
Papular elastorrhexis is a rare disorder that occurs predominantly during adolescence. We report 5 patients with asymptomatic white, nonfollicular, firm papules scattered over the trunk and extremities. Histologically, the papules demonstrate focal areas of collagen homogenization with decreased and fragmented elastic fibers.
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