A clinicopathologic study was made of 35 patients with primary eccrine adenocarcinoma diagnosed in the past 20 years from among 450,000 consecutive skin biopsy specimens. Histologically the following four distinct variants were identified: eccrine porocarcinoma (18 cases), syringoid eccrine carcinoma (12 cases), mucinous eccrine carcinoma (three cases), and clear cell eccrine carcinoma (two cases). Overall, eccrine adenocarcinomas are destructive lesions with a tendency to local recurrence. The syringoid histologic variant appears to be well differentiated and may have a benign clinical course; the lesion remained localized to the skin in our 12 cases. Regional lymphatic and distant metastasis, however, occurred in two patients with eccrine porocarcinoma.

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During the past 2 years we have studied eighty-nine biopsy specimens from nineteen patients with Cowden's syndrome. Among fifty-three facial lesions, twenty-nine were trichilemmomas. Twenty-three of the others were consistent with trichilemmoma, showed a non-specific benign verrucous acanthoma, or were not diagnostic; one was a blue naevus. All fourteen oral mucosal biopsy specimens were benign fibromas. Nineteen of twenty-two biopsy specimens from the hands and feet showed the pattern of benign keratosis. Multiple trichilemmomas were found in all patients with Cowden's syndrome, but at times several biopsy specimens were required before a diagnostic picture was uncovered. All patients with multiple facial trichilemmomas were found to have Cowden's syndrome. The combination of multiple facial trichilemmomas, oral fibromas, and benign acral keratoses enables one to diagnose Cowden's syndrome at a stage before serious internal complications develop.

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