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Buschke-Ollendorff syndrome - differential diagnosis of disseminated connective tissue lesions


European Journal of Dermatology. Volume 11, Number 6, 576-9, November - December 2001, Cas cliniques

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Author(s) : A. ASSMANN, N. MANDT, C. C. GEILEN, U. BLUME-PEYTAVI

Summary : We report a 5-year-old boy presenting with multiple elastic type nevi and osteopoikilosis who was diagnosed as having Buschke-Ollendorff syndrome at an early age. Connective tissue lesions may present as the main symptom of varying clinical entities with different outcomes. Differential diagnosis includes papular elastorrhexis, fibroelastolytic papules of the neck, papular acne scars, and late onset focal dermal elastosis. Rare genodermatoses, i.e. Buschke-Ollendorff syndrome, pseudoxanthoma elasticum, juvenile hyaline fibromatosis and familiar cutaneous collagenoma should be carefully evaluated to provide appropriate genetic counseling and to avoid unnecessary treatment procedures.

Keywords : connective tissue nevi, dermatofibrosis disseminata, osteopoikilosis, Buschke-Ollendorff syndrome.

 

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