Trichodysplasia spinulosa L67.9

Izakovic et al. 1995; Haycox 1999

Rare, apparently virus-induced, preferentially centrofacially localized, folliculotropic, spinular keratinization disorder that may occur in immunosuppressed patients several months after the onset of immunosuppression.

Prior immunosuppressive therapy lasting 8-50 months with various immunosuppressants such as: tacrolimus, ciclosporin, methotrexate, rituximab, fludarabine and vincristine. Polyoma-like virus particles were detectable in the skin (Matthews MR et al 2011). TSPyV (TS-associated polyoma virus) appears to actively replicate in the cells of the inner root sheath of the hair follicle; hyperproliferation of these cells is thought to underlie the clinically observable manifestations of the disease.

Antiviral drugs such as valganciclovir and cidofovir have shown benefit in the treatment of this disease in case reports.

Predominantly centrofacially localized red or red-brown, largely asymptomatic, follicular papules and plaques with a sometimes spiky, spike-like surface structure. Keratosis pilaris-like skin lesions are also observed on the extensor sides of the upper (and lower) extremities, on the lateral thoracic areas, and on the buttocks. Infestation of the capillitium may lead to circumscritic alopecia (analogous to keratosis pilaris syndrome), and infestation of the eyebrows to madarosis.

Dilated infundibula with orthokeratotic horn masses which protrude from the follicle surface like a spike. There is a mild perifollicular lymphocytic infiltrate.

1. Benoit T (2010) Viral-associated trichodysplasia of immunosuppression. Arch Dermatol 146: 871-874
2. Haycox CL et al (1999) Trichodysplasia spinulosa: a newly described folliculocentric viral infection in an immunocompromised host. J Invest Dermatol Symp Proc 4: 268-271
3. Heaphy MR et al (2004) Cyclosporine-induced folliculodystrophy. J Am Acad Dermatol 50: 310-314
4. Kazem S et al (2013) The trichodysplasia spinulosa-associated polyomavirus: virological background and clinical implications. APMIS 121:770-82.
6. Matthews MR et al (2011) Viral-associated trichodysplasia spinulosa: a case with electron microscopic and molecular detection of the trichodysplasia spinulosa-associated human polyomavirus. J Cutan Pathol 38:420-431.
7. Rouanet J et al (2016) Trichodysplasia spinulosa: a polyomavirus infection specifically targeting follicular keratinocytes in immunocompromised patients. Br J Dermatol 174:629-632.
8. Wu JH et al (2016) Molecular insight into the viral biology and clinical features of trichodysplasia spinulosa. Br J Dermatol 174:490-498.