The trichilemmoma is a benign solid tumor originating from external sheath cells of pilosebaceous follicles, and the desmoplastic trichilemmoma is a rare benign histological variant.
What is desmoplastic trichilemmoma?
Desmoplastic trichilemmoma (DT) is a rare benign histologic variant of trichilemmoma, a benign growth of the pilosebaceous follicle’s outer sheath. 1. Headington and French first described trichilemmoma in 1962.
Is trichilemmoma cancer?
Trichilemmomas are benign tumors arising from the outer cells of the hair follicle.
How common are trichilemmoma?
Approximately 40 cases per 100,000 consecutive skin biopsies may be found every year in any given dermatopathologic laboratory. Unlike isolated trichilemmomas, multiple trichilemmomas associated with Cowden disease are very rare. The international frequency is unknown.
What does Cowden syndrome look like?
Skin: The characteristic skin findings of patients with PHTS/Cowden syndrome are papillomatous papules (benign, skin-colored, raised bumps), trichilemmomas (benign tumors coming from the outer cells of the hair follicle, most frequently on the head), and lipomas (benign fatty growths).
What is a Trichoepithelioma?
Trichoepithelioma is a rare benign skin lesion that originates from hair follicles. Trichoepitheliomas are mostly seen in the scalp, nose, forehead, and upper lip. These skin lesions originate from benign proliferation of epithelial-mesenchymal origin cells.
Should Desmoplastic Trichoepithelioma be removed?
True desmoplastic trichoepithelioma is a benign tumour with no malignant potential, so there is no need to remove them.
Should trichoepithelioma be removed?
All in all, as a benign tumor, trichoepithelioma can be managed safely with surgical removal. Alternatives include dermal abrasion and laser surgery, curettage although these options may be associated with an increased rate of recurrence.
What is Cowden’s syndrome?
Cowden syndrome is a genetic disorder characterized by multiple noncancerous, tumor-like growths called hamartomas and an increased risk of developing certain cancers. Almost everyone with Cowden syndrome develops hamartomas.
What causes Brooke-Spiegler syndrome?
Brooke-Spiegler syndrome, familial cylindromatosis and multiple familial trichoepitheliomas are due to germline mutations in the cylindromatosis (CYLD) gene on chromosome 16q12. These disorders have an autosomal dominant inheritance, meaning half of an affected individual’s children will also have the condition.
What causes desmoplastic trichilemmoma?
The underlying cause of trichilemmoma is unknown but because of its similarity to a viral wart/verruca, it has been postulated that it may come from a viral infection such as the human papillomavirus (HPV). To date there is no evidence for this. Desmoplastic trichoepithelioma is a harmless facial skin lesion.
Are all facial papules in CS patients trichilemmomas?
Even in CS patients with the characteristic presentation of multiple facial trichilemmomas, not all of the facial papules may be trichilemmomas. Studies have found that CS patients also present with multiple facial papillomatous papules or viral warts coexisting with multiple facial trichilemmomas.
Does DNA from epidermodysplasia verruciformis (EV) appear in trichilemmomas?
Studies have shown the presence of DNA from epidermodysplasia verruciformis (EV)-associated HPVs (supergroup B) in trichilemmomas, but not for other types of HPV.
How is trichilemmoma differentiated from other skin tumours?
The histology of trichilemmoma will differentiate it from other skin tumours that have similar clinical presentations, such as trichoepithelioma, trichofolliculoma and basal cell carcinoma.
