Diagnosis: Schopf-Schulz-Passarge syndrome.
MICROSCOPIC FINDINGS AND CLINICAL COURSE:
The biopsy specimen revealed large cystic spaces within the dermis. Two cell layers of the cystic wall were evident: outer cuboidal cells and inner columnar cells with decapitation secretion. These findings were consistent with multiple apocrine hidrocystomas. A diagnosis of Schopf-Schulz-Passarge syndrome was made. For skin cancer prophylaxis and possible treatment of both hyperkeratosis and apocrine hidrocystomas, acitretin therapy (25 mg/d) was initiated. There was minimal improvement of the hyperkertosis, and the acitretin therapy was discontinued.
DISCUSSION
In 1971, Schopf et al1 described the syndrome of multiple eyelid apocrine hidrocystomas, palmoplantar hyperkeratosis, hypodontia, and hypotrichosis. Since then, 20 cases (13 reports) have been reported in the literature.1-8 Multiple apocrine hidrocystomas of the eyelids have been described and can occur unrelated to this syndrome.
Schopf-Schulz-Passarge syndrome, which is characterized as a rare variant of ectodermal dysplasia,4 is commonly diagnosed in the second half of life.2 There are more than 150 recognized forms . . . [Full Text of this Article]
