History
Patients with trichilemmomas usually give a history of a slow-growing, asymptomatic papule and/or plaque on the face. Such lesions may be solitary or multiple. Patients often desire reassurance that they do not have a skin cancer and/or seek an evaluation for cosmetic removal of the lesions.
If presenting in a nevus sebaceous of Jadassohn, a trichilemmoma may appear as a new growth within the lesion. No single feature allows the clinical diagnosis of a trichilemmoma, but rather this diagnosis is usually rendered histologically. However, if multiple lesions are present on the face, trichilemmomas associated with Cowden syndrome may be suspected. Once the diagnosis of trichilemmoma has been given, reevaluating the patient for clinical features of Cowden syndrome may be prudent.
Physical Examination
Patients with trichilemmomas usually present with either a single papule or multiple, small, flesh-colored papules that are 1-5 mm in diameter on the face or the neck. When these lesions grow in size, small plaques may be found, particularly in the nasolabial fold region. As trichilemmomas slowly enlarge, they often produce a hyperkeratotic surface suggestive of a verruca or a cutaneous horn. Besides the central part of the face, the ears, the forearms, the hands, or within a nevus sebaceous of Jadassohn are other typical sites that are examined for these cutaneous lesions.
Trichilemmoma commonly stems from nevus sebaceous, which usually is found on the face and scalp. However, cases have also been noted on the eyelid and anus. [19, 20]
If a diagnosis of trichilemmoma is rendered, the patient should be completely examined for evidence of Cowden syndrome. (See images below.) Ten key physical features to look for in establishing the diagnosis of Cowden syndrome include the following:
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Adenoid facies with facial trichilemmomas
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Craniomegaly
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High arched palate
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Oral papillomas producing a cobblestone appearance on the lips and the mucosal surfaces
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Goiter or palpable thyroid nodules/tumors
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Gynecomastia in men
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Fibrocystic breast disease and/or palpable breast nodules or tumors in women
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Acral keratoses on the dorsum of the hands and the wrists
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Palmoplantar translucent punctate keratoses
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Sclerotic fibromas on the extremities
In 1983, Salem and Steck [21] proposed diagnostic criteria for Cowden syndrome. See Cowden Disease (Multiple Hamartoma Syndrome) for a more definitive discussion in this area.
Patients with desmoplastic trichilemmomas usually present with lesions less than 1 cm in diameter found predominantly on the face, the neck, the scalp, and, sometimes, on the chest or the vulva. Several reports have described desmoplastic trichilemmomas occurring on the upper eyelid. [22, 23] They may be indurated, with a depressed central region and a raised, annular border. Desmoplastic trichilemmomas, although not associated with Cowden disease, have been described arising from a nevus sebaceous. [3, 24]
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A patient with trichilemmoma papules on the face.
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Low-power histologic view of a trichilemmoma.
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Mid-power histologic view of a trichilemmoma.
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Low-power histologic view of a desmoplastic trichilemmoma.
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High-power histologic view of a desmoplastic trichilemmoma.
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Tumor of the follicular infundibulum.
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Clinical image of the face of a patient with Cowden syndrome.
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Clinical image of the oral mucosa of a patient with Cowden syndrome.
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Clinical image of palmar keratoses in a patient with Cowden syndrome.
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Clinical image of sclerotic fibroma in a patient with Cowden syndrome.
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Clinical image of multiple trichilemmomas in a patient with Cowden syndrome.
