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

We report a 5-year-old boy presenting with multiple elastic type nevi and o steopoikilosis who was diagnosed as having Buschke-Ollendorff syndrome at a n early age. Connective tissue lesions may present as the main symptom of v arying clinical entities with different outcomes. Differential diagnosis in cludes 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 evaluat ed to provide appropriate genetic counseling and to avoid unnecessary treat ment procedures.