Eccrine Syringofibroadenomatosis With Eyelid Abnormalities

Chen et al. (1998) described a father and his 2 sons with multiple eccrine syringofibroadenomas in association with eyelid abnormalities and progressive corneal scarring. The 39-year-old father had multiple erythematous papules and plaques on the feet, toes, and scrotum. The scrotal lesions began in early childhood. The children, aged 2 and 6 years, had similar erythematous plaques on the scrotum. None had signs of ectodermal dysplasia, and their hair, teeth, and palmar sweating were normal. The father had had multiple eyelid reconstructions to correct upper lid entropion and lower lid ectropion. He had a right corneal transplant that failed. Both sons showed marked cicatricial ectropion of the lower lids and a mild entropion of the upper lids with an extra roll of skin above the lid margins. There was absence of the lashes in the lower lids. Chen et al. (1998) proposed that this family had a newly recognized genetic syndrome consisting of eccrine syringofibroadenomatosis and ocular disease manifested by lid abnormalities, lack of meibomian glands, and progressive corneal scarring.