Trichodysplasia spinulosa

A rare skin condition associated with immunosuppression

Trichodysplasia spinulosa is a rare skin condition that primarily affects individuals who are immunocompromised. It is characterized by the development of follicular papules and spiny keratotic projections, primarily on the face. This condition is associated with the Trichodysplasia spinulosa polyomavirus (TSPyV), a member of the Polyomavirus family.

Presentation

Trichodysplasia spinulosa typically presents in immunosuppressed patients, such as those who have undergone organ transplantation or are receiving chemotherapy. The condition is marked by the appearance of papules and spicules on the face, particularly around the nose, ears, and eyebrows. These lesions are often described as "spiny" or "keratotic" and can be cosmetically disfiguring.

Pathophysiology

The underlying cause of trichodysplasia spinulosa is infection with the Trichodysplasia spinulosa polyomavirus. This virus is thought to infect the hair follicles, leading to abnormal keratinization and the formation of the characteristic spiny lesions. The exact mechanism by which TSPyV causes these changes is not fully understood, but it is believed to involve viral replication within the follicular epithelium.

Diagnosis

Diagnosis of trichodysplasia spinulosa is primarily clinical, based on the characteristic appearance of the lesions in an immunocompromised patient. Skin biopsy can be performed to confirm the diagnosis, revealing dilated hair follicles filled with keratin and viral particles. Electron microscopy and PCR testing can be used to detect the presence of TSPyV DNA in the lesions.

Treatment

There is no specific antiviral treatment for trichodysplasia spinulosa. Management typically involves reducing the level of immunosuppression if possible, which may lead to improvement in the lesions. Topical treatments, such as retinoids or cidofovir, have been used with varying success. In some cases, the lesions may resolve spontaneously if the patient's immune function improves.

Prognosis

The prognosis for trichodysplasia spinulosa is generally good, especially if the level of immunosuppression can be reduced. The condition is primarily a cosmetic concern, and there is no evidence that it leads to more serious health problems. However, the lesions can be persistent and may recur if the patient's immune status deteriorates.

Related pages

• Immunosuppression
• Polyomavirus
• Skin lesion
• Organ transplantation