Desmoplastic Trichoepithelioma Symptoms, Causes, Diagnosis, Treatment

Desmoplastic Trichoepithelioma Symptoms, Causes, Diagnosis, Treatment

Trichoepithelioma is a benign, cutaneous neoplasm that develops from the hair follicle. It is divided into three types: singular trichoepithelioma, multiple trichoepithelioma, and desmoplastic trichoepithelioma, which is thought to be an unusual cutaneous adnexal tumor. 

Hartzell first reported desmoplastic trichoepithelioma in 1904, labeling it a benign cystic epithelioma. Desmoplastic trichoepithelioma is also known as adenoides cysticum epithelioma or morphea-like epithelioma. It is a rare type of trichoepithelioma. Desmoplastic trichoepithelioma develops primarily in sun-exposed areas and resembles basal cell carcinoma tumors.

Desmoplastic trichoepithelioma develops from hair follicle cells. It is called a benign tumor of the adnexa or hair follicle. Middle-aged women are most commonly affected, however, desmoplastic trichoepithelioma can occur at any age or in any gender. 

Desmoplastic Trichoepithelioma Symptoms, Causes, Diagnosis, Treatment

Desmoplastic Trichoepithelioma Symptoms

Desmoplastic trichoepithelioma usually looks like a firm, ring-shaped, skin-colored to red, the plaque with a depression in the middle. The upper cheek is the most common location for this growth. Desmoplastic trichoepithelioma can be stable or grow up to 1 cm in diameter over time. Most of DTEs, like other benign skin tumors, grow slowly at first and subsequently stabilize, requiring no treatment. 

The females are mostly affected by this disease and the condition most frequently manifests on the face or the cheeks. The other symptoms or signs such as; lack of ulceration, superficial invasion, non-neoplastic character, and histopathological appearance make this tumor rare. DTE, like other forms of skin cancer, progresses relatively slowly during its early growth phase, after which it transforms into a stable lesion.

The lesion lacks pain, itching, ulceration, subsequent lymph node enlargement, and other signs. Nodular basal cell carcinoma and desmoplastic trichoepithelioma are found in suspected family members. 

Desmoplastic Trichoepithelioma Causes

Trichoepithelioma is mostly hereditary. Desmoplastic trichoepithelioma is common among children and adults. It has a bimodal age distribution. There are normally no symptoms associated with the lesion, although it may grow in shape and size over time.

Trichoepithelioma is caused by the same gene that causes basal cell carcinoma. Since trichoepithelioma is passed down in an autosomal dominant way, both men and women have the same chance of getting it. However, men tend to show fewer symptoms and have a lower risk of getting it, so most patients are women.

Basal cell carcinoma and desmoplastic trichoepithelioma share the same genetic tendency, which can lead to many precancerous lesions. Desmoplastic trichoepithelioma is a tumor that can be related to basal cell carcinoma or cylindroma. This association denotes an autosomal dominant pattern of disease inheritance. Cylindroma and desmoplastic trichoepithelioma share CYLD mutations. 

Desmoplastic Trichoepithelioma Diagnosis

Full-thickness skin biopsy is used to detect desmoplastic trichoepithelioma. The diagnosis is difficult, even when examined by an expert, especially when the tumor looks like other malignant tumors. A preliminary diagnosis is determined based on the patient’s medical history and physical appearance. Histopathologic examination of a lesion following biopsy is required for a definitive diagnosis.

Desmoplastic Trichoepithelioma resembles certain other malignancies clinically and histopathologically, such as microcystic adnexal carcinoma, conventional trichoepithelioma, syringoma, and morphea Basal Cell Carcinoma. Histological evidence, in combination with clinical symptoms, is useful in determining the diagnosis of certain of these lesions.

In most cases, DTE is identified during normal skin cancer screenings and may have been there for years without showing any signs of change or improvement. Skin cancer screening as part of routine physical examinations, follow-up, biopsy, and ongoing monitoring are all critical for proper treatment.

Desmoplastic Trichoepithelioma Treatment

This type of desmoplastic trichoepithelioma has no malignant potential and does not need to be removed. However, because basal cell carcinoma can seem quite similar, it is preferable to monitor the lesions if the diagnosis is erroneous.

Local surgical excision is the preferred treatment for DTE and is regarded as first-line therapy for most benign tumors. Even though this technique can lead to complete remission with very little recurrence, post-surgical complications, such as scarring and hypopigmentation, are still the main problem. This is especially true for the face, which is a very sensitive area from a cosmetic point of view.

Mohs micrographic surgery is also one of the treatment options just like surgical excision, but it is relatively costly. Closed monitoring is the only method that is necessary for the cost-effective management of DTE in classical cases that have definitively benign results.

Mohs surgery is advantageous in situations with unusual clinical and histological characteristics and when the preservation of healthy surrounding tissue is crucial, such as when the tumor arises in a cosmetically sensitive location like the face. Other than this, Curettage and electrodesiccation are also used for treating this asymptomatic disease.

Leave a Reply