Trichodysplasia spinulosa

Author: Dr Catriona Glen, Dermatology Registrar, Royal Infirmary of Edinburgh, Scotland; Chief Editor: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, June 2016.

 

What is trichodysplasia spinulosa?

Trichodysplasia spinulosa is rare condition first described in 1995. It typically presents with flesh coloured papules and keratin spines over the nose and forehead.

Who gets trichodysplasia spinulosa?

Trichodysplasia spinulosa most often affects immunocompromised patients, such as people who have had an organ transplant. It is reported in patients of all age groups and sex equally.

What causes trichodysplasia spinulosa?

The definitive cause of trichodysplasia spinulosa is unknown. However, research has implicated a virus called Trichodysplasia spinulosa-associated polyomavirus (TSPyV) as the most likely causative agent.  Patients with trichodysplasia spinulosa are more likely to have a high viral load of TSPyV than the general population.  

What are the clinical features of trichodysplasia spinulosa?

Trichodysplasia spinulosa presents as an eruption of flesh coloured follicular papules each with a central keratin spine. These are usually located over the nose and forehead, but can more rarely affect other areas of the body including the ears and trunk.   

Trichodysplasia spinulosa can result in hair loss (localised alopecia). This most commonly affects the eyebrows but can also affect eyelashes (madarosis) and scalp.

How is trichodysplasia spinulosa diagnosed?

Trichodysplasia spinulosa can be diagnosed clinically or by performing a skin biopsy.

Typical histopathological features include large and distended hair follicles, which are often plugged with keratin and other debris.

What is the treatment for trichodysplasia spinulosa?

As trichodysplasia spinulosa is rare, there is limited data on treatment. Success has been reported from:

What is the outcome for trichodysplasia spinulosa?

Little is known about the long-term outcome for patients with trichodysplasia spinulosa.

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