UPMC Dermatopathology "Case of the Month" Presentations
UPP - Department of Dermatology, Dermatopathology Unit
Kristina Paley, MD ; Drazen Jukic, MD, PhD; Joseph C. English III, MD
JULY 2007 CASE OF THE MONTH
CLINICAL FINDINGS
52 year old white female presented for removal of multiple mucosal and cutaneous lesions. Over the course of her lifetime, the patient has had more than 30 surgical procedures to address her mucocutaneous as well as internal lesions. These procedures included removal of a large benign ovarian tumor at the age of 16, meningioma resection at the age of 37, removal of gastric and colonic polyps, history of goiter and most recently diagnosis of breast cancer for which patient has undergone left mastectomy and right lympectomy. Patient’s father and grandfather both have been diagnosed with the same genetic condition as our patient but have had less prominent manifestations of the syndrome.
Physical exam of the patient was significant for diffuse flesh colored mucocutaneous papules along with prominent cobblestoning of the oral mucosa (Figures 1-4). Pathology showed a verrucous lesion consisting of lobular proliferation of bland cells with peripheral nuclear palisading. Lobules were surrounded by a prominent basement membrane. All histological findings were consistent with a trichilemomma (Figures 5-7).
Patient’s medical and family histories, cutaneous manifestations along with pathology were diagnostic of Cowden syndrome
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