Eccrine syringofibroadenomatosis associated with hidrotic ectodermal dysplasia

Eccrine syringofibroadenoma (ES) is generally regarded as a solitary benign adnexal neoplasm of the skin. Recently, the epithelial and stromal changes of this lesion have been recognized as being a reaction pattern in certain settings. We describe two individuals in whom this process was a manifestation of hidrotic ectodermal dysplasia. In both cases, the diagnosis had been missed for many years and the process had been thought to be a manifestation of an inflammatory dermatosis of the palms and soles. In both instances, other family members were found to be affected. Thus, ES may be manifested clinically as erythematous erosive plaques, especially of the palms and soles. It is important that clinicians be familiar with this disorder and be aware that it may exhibit unusual cutaneous manifestations.