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

Assmann, A.; Mandt, N.; Geilen, C.C.; Blume-Peytavi, U.

European Journal of Dermatology Ejd 11(6): 576-579

2001


ISSN/ISBN: 1167-1122
PMID: 11701413
Document Number: 527070
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.

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