The cutaneous pathology of Cowden's disease: new findings.
Histological, histochemical and immunohistochemical findings were studied in 40 cutaneous biopsies from 7 patients with Cowden's disease. Most facial biopsies showed a spectrum of trichilemmomas and related follicular malformations, including cylindrical trichilemmomas, lobulate trichilemmomas (14 lesions), and a keratinizing type sharing features with inverted follicular keratosis. One facial growth showed trichilemmomal changes without apparent follicular origin. Studies for common papilloma virus structural antigens were negative, apart from a typical common wart in one patient. Immunohistochemical studies in 6 facial trichilemmomas and acral keratoses, using a panel of anti-keratin antibodies, disclosed only abnormal differentiation with lack of large keratins in the lobulate trichilemmomas. Nine biopsies revealed a distinctive type of fibroma characterized by an organized pattern of interwoven fascicles of collagen bundles with a laminated or tortuous appearance, embedded in abundant mucin. A number of fibromas showed striking hyalinization; these may represent a second microscopic hallmark of Cowden's disease in addition to facial trichilemmomas.
Starink
T M
TM
Meijer
C J
CJ
Brownstein
M H
MH
D000956:Antigens, Viral / Q000032:analysis; D001706:Biopsy; D005153:Facial Neoplasms / Q000032:analysis / Q000473:pathology; D005350:Fibroma / Q000032:analysis / Q000473:pathology*; D006222:Hamartoma / Q000473:pathology*; D006801:Humans; D007633:Keratins / Q000032:analysis; D012867:Skin / Q000473:pathology*; D012878:Skin Neoplasms / Q000032:analysis / Q000473:pathology*; D013194:Staining and Labeling; D013577:Syndrome
D000956:Antigens, Viral; D007633:Keratins