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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.

 


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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.

 

References

  • Rouanet J, Aubin F, Gaboriaud P, Berthon P, Feltkamp MC, Bessenay L, Touzé A,  Nicol JT, Franck F, D'Incan M. Trichodysplasia spinulosa: a polyomavirus infection specifically targeting follicular keratinocytes in immunocompromised patients. Br J Dermatol. 2016 Mar;174(3):629–32. doi: 10.1111/bjd.14346. Epub 2016 Jan 28. PubMed PMID: 26663358.
  • Wu JH, Nguyen HP, Rady PL, Tyring SK. Molecular insight into the viral biology and clinical features of trichodysplasia spinulosa. Br J Dermatol. 2016 Mar;174(3):490–8. doi: 10.1111/bjd.14239. Epub 2015 Dec 14. Review. PubMed PMID:  26479880. 
  • Kazem S, van der Meijden E, Feltkamp MC. The trichodysplasia spinulosa-associated polyomavirus: virological background and clinical implications. APMIS. 2013 Aug;121(8):770–82. doi: 10.1111/apm.12092. Epub 2013 Apr 18. Review. PubMed PMID: 23593936. 
  • Matthews MR, Wang RC, Reddick RL, Saldivar VA, Browning JC. Viral-associated trichodysplasia spinulosa: a case with electron microscopic and molecular detection of the trichodysplasia spinulosa-associated human polyomavirus. J Cutan Pathol. 2011 May;38(5):420–31. doi: 10.1111/j.1600-0560.2010.01664.x. Epub 2011 Jan 19. Review. PubMed PMID: 21251037; PubMed Central PMCID: PMC3756806.

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