Trichodysplasia spinulosa: a comprehensive review of the disease and its treatment

Abstract

Trichodysplasia spinulosa (TS) is a rare dermatological disease caused by TS-associated polyomavirus (TSPyV) in immunosuppressed patients. The seroprevalence of TSPyV in immunocompetent adults is high and the number of immunosuppressed patients developing TS remains low, suggesting that TS is underdiagnosed and/or that additional unknown factors are needed in order to develop TS. There is no well-established treatment for TS, and to date a majority of reported cases have consequently received ineffective therapies, likely due to the unavailability of reviews and recommendations of treatments for TS. The few treatments reported in case reports to be effective include topical cidofovir 3%, reduction of immunosuppression and oral valganciclovir. In this comprehensive review, we present all published cases to date, together with a summary of all treatments for TS categorized by overall clinical efficacy, thus addressing this rare disease and what appears to be its clinically efficacious treatment.

Dermatoscopy case of the month: Trichodysplasia spinulosa

“… 1 , 2 Histologically, TS is characterized by dilated hair follicles with enlarged, eosinophilic trichohyalin granules and dystrophy of the inner root sheath epithelium with apoptotic follicular keratinocytes. 1 , 2 Timely diagnosis of TS is crucial as it may progress to leonine facies if left untreated. 1 , 2 Dermatoscopic evaluation can aid in rendering a timely diagnosis.…”

“… 1 , 2 Timely diagnosis of TS is crucial as it may progress to leonine facies if left untreated. 1 , 2 Dermatoscopic evaluation can aid in rendering a timely diagnosis. The most specific dermatoscopic clue for distinguishing TS from other hyperkeratotic follicular disorders is the presence of bright white spicules that protrude peripherally from follicular openings.…”

“…The most specific dermatoscopic clue for distinguishing TS from other hyperkeratotic follicular disorders is the presence of bright white spicules that protrude peripherally from follicular openings. 1 , 3 In contrast to the dark, confined keratin plugging seen in other hyperkeratotic disorders, it is both the stark whiteness of the spicules and their considerable length that are characteristic of TS. 1 , 3 TS in patients with skin of color may also present with perifollicular hyperpigmentation with a central white or pink circle.…”

“… 1 , 3 In contrast to the dark, confined keratin plugging seen in other hyperkeratotic disorders, it is both the stark whiteness of the spicules and their considerable length that are characteristic of TS. 1 , 3 TS in patients with skin of color may also present with perifollicular hyperpigmentation with a central white or pink circle. Treatment options include reduction of immunosuppression, topical cidofovir, or systemic valganciclovir, among other regimens.…”

“… 1 , 2 Histologically, TS is characterized by dilated hair follicles with enlarged, eosinophilic trichohyalin granules and dystrophy of the inner root sheath epithelium with apoptotic follicular keratinocytes. 1 , 2 …”