Papular elastorrhexis: a distinct variant of connective tissue nevi or an incomplete form of Buschke-Ollendorff syndrome?

Abstract

Papular elastorrhexis is a rare entity, possibly a form of multiple elastic tissue naevi. The cutaneous lesions in this disorder are characterized by multiple white papules usually occurring on the trunk. These tend to be nonfollicular and scattered evenly over the affected area. Histopathologically, there is a decrease of elastic fibres, that may also appear thin and fragmented. Most reported cases are sporadic but familial occurrence has been described and some authors believe that papular elastorrhexis may represent a mild form of Buschke-Ollendorff syndrome. We report an 18-year-old woman whose clinical and histopathological features were compatible with papular elastorrhexis. There was no evidence of skeletal changes or relevant family history.