Connective tissue nevus and osteopoikilosis in the hand: the Buschke-Ollendorff syndrome

The Buschke-Ollendorff syndrome is a rare disorder of uncertain etiology characterized by osteopoikilosis and connective tissue nevi. An unusual presentation of this syndrome is described that involves both the cutaneous and skeletal manifestations affecting the hand. The bones of the hand and carpus are a common site for osteopoikilosis. Connective tissue nevi of the hand may require excision for diagnosis or mechanical impingement. Special elastin stains must be done on these cutaneous lesions to confirm the diagnosis. Routine hematoxylin-eosin stains may fail to show any pathologic change in mild cases.