Birt-Hogg-Dubé Syndrome is an autosomal dominant condition characterized by a triad of fibrofolliculomas, trichodiscomas, and acrochordons. Since the first description in 1977, many conditions have been described in association with its clinical triad. Recent epidemiological studies have shown a significant association between the occurrence of lesions in the fibrofolliculoma/trichodiscoma category with renal neoplasms and pneumothoracies. The BHD protein folliculin had recently been identified. The histological findings of the clinical lesions are distinctive. We report a patient with a history of melanoma who presented for routine surveillance. Facial lesions in the fibrofolliculoma/trichodiscoma category were identified. Diagnostic work-up revealed concomitant multinodular goiter, pulmonary cyst, and renal mass. The patient later developed pneumothorax. Clinical manifestations, histological findings, associations, management, and a review of the Birt-Hogg-Dubé Syndrome are discussed.

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Primary cutaneous adenoid cystic carcinoma is a rare skin neoplasm with a high potential for recurrence after local excision. We present a case of a 45-year-old white female with recurrent primary cutaneous adenoid cystic carcinoma of the scalp. The tumor was resected with Mohs surgery in two stages and defect repaired with split-thickness skin graft. There has been no recurrence of the tumor in the 12-month follow-up period.

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