Slideshow
-
Slide
An 82-year-old man presents for examination and removal of a growth on his nose. The patient reports that the asymptomatic lesion has been present for approximately 1 year. The patient was previously treated for a basal cell carcinoma of his scalp and actinic keratoses of his hands. He has no history of other malignancies. Physical examination reveals a 0.4-cm firm, erythematous papule on the tip of his nose.
Submit your diagnosis to see full explanation.
The patient is diagnosed with desmoplastic tricholemmoma. Desmoplasia refers to fibrosis. When accompanying cutaneous tumors, desmoplasia may be associated with both benign and malignant neoplasms.1 Desmosplastic trichilemmomas, however, are benign lesions that arise from the infundibulum of pilosebaceous hair follicles and are comprised of glycogen-rich clear cells embedded within a fibrous stroma.2 They are similar clinically to the nondesmoplastic form and their most common locations include the cheeks, nose, and forehead.3 Lesions presenting elsewhere are rare.4,5
In this case, desmoplastic tricholemmoma was found in association with an epidermoid cyst. Most trichilemmomas appear as solitary lesions. Multiple lesions should raise suspicion of Cowden syndrome, which is an autosomal dominant genodermatosis with incomplete penetrance that is associated with underlying systemic malignancies including thyroid, colon, and breast cancer.6 Most cases of desmoplastic tricholemmoma have a mutation of PTEN, a suppressor gene, resulting in increased cell proliferation. Trichilemmomas are readily removed by shave excision that allows for histopathologic confirmation.
Stephen Schleicher, MD, is director of the DermDox Dermatology Centers, associate professor of medicine at Geisinger Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.
References
- Abbas O, Mahalingam M. Desmoplasia: not always a bad thing. Histopathology. 2011;58(5):643-659. doi:10.1111/j.1365-2559.2010.03617.x
- Tellechea O, Reis JP, Baptista AP. Desmoplastic trichilemmoma. Am J Dermatopathol. 1992;14(2):107-114. doi:10.1097/00000372-199204000-00004
- Goldman L, Richfield DF. Trichilemmoma clinical lesions. Arch Dermatol. 1977;113(1):107-108. doi:10.1001/archderm.1977.01640010109019
- Spiegel JH, Khodai N. Trichilemmoma of the nose. Am J Otolaryngol. 2006;27(6):430-432. doi:10.1016/j.amjoto.2006.01.014
- Sano DT, Yang JJ, Tebcherani AJ, Bazzo LA. A rare clinical presentation of desmoplastic trichilemmoma mimicking Invasive carcinoma. An Bras Dermatol. 2014;89(5):796-798. doi:10.1590/abd1806-4841.20143095
- Lopes S, Vide J, Moreira E, Azevedo F. Cowden syndrome: clinical case and a brief review. Dermatol Online J. 2017;23(8):13030/qt0023k3x0
QUESTION
