Trichodysplasia Spinulosa in a Kidney Transplant Recipient

Abstract

A kidney transplant recipient in her 60s (who was taking mycophenolate mofetil and belatacept) presented with a 1-year history of pruritic pink follicular papules with central white spicules on her face, ears, and extremities associated with eyebrow and eyelash alopecia (Figure). Skin biopsy results demonstrated distended hair follicles occluded by hyperkeratotic and parakeratotic debris. At the suprabulbar level, the outer root sheath had central plugging with enlarged intracytoplasmic trichohyaline granules diagnostic of trichodysplasia spinulosa (TS). Polymerase chain reaction confirmed the presence of TS polyoma virus.Treatment with compounded topical 1% cidofovir cream, applied twice daily, produced significant clinical improvement within 2 months of treatment that was maintained at 6-month and 1-year follow-up visits. Trichodysplasia spinulosa is a rare folliculocentric infection seen almost exclusively in patients with immunosuppression, including transplant recipients and patients with a hematologic malignancy. 1 In 2010, the TS human polyoma virus was identified by electron microscopy, sequenced, and named. 2 To our knowledge, only 30 cases of TS have been reported in association with iatrogenic immunosuppression.Trichodysplasia spinulosa is characterized by numerous erythematous to skin-colored follicular papules with central white spicules distributed predominantly over the central face, often resulting in loss of the eyebrows and eyelashes. While the TS human polyoma virus has been identified in saliva, blood, and nasal swabs, the virus appears to primarily affect the skin, with unknown longterm consequences. 2 Trichodysplasia spinulosa can be diagnosed clinically. However, because the differential diagnosis is broad, a skin biopsy provides diagnostic confirmation. The differential diagnosis may include lichen spinulosus, multiple minute digitate hyperkeratosis, spiculate demodicosis, lichen planopilaris, keratosis pilaris, trichostasis spinulosa and the follicular spicules of multiple myeloma.Treatments for TS include 1% to 3% topical cidofovir, oral valganciclovir, and a reduction of immunosuppression when clinically appropriate. 3 Given the risk of transplant rejection when reducing immunosuppression, as well as potential adverse effects of valganciclovir, including creatinine elevations and bone marrow suppression, topical cidofovir was chosen as the initial option. More recently, leflunomide has also been described as an oral treatment. 4 This patient highlights a rare condition that was effectively and safely treated with topical therapy.

Trichodysplasia spinulosa: a presentation of polyomavirus infection in immunosuppressed patients

“…Although initially described in association with cyclosporine use in solid organ transplant patients [7,8], TS was subsequently reported in association with other immunosuppressants [9,10] and hematological malignancies [11,12]. Trichodysplasia spinulosa has also been reported in one patient with no history of immunosuppression that was treated with vismodegib for basal cell carcinomas arising from Gorlin syndrome [13].…”

“…On dermatoscopy, TS can be distinguished from other hyperkeratotic disorders by the presence of long, bright white spicules that protrude peripherally from follicular openings, compared to the dark, confined keratin plugs seen in other hyperkeratotic conditions [20]. The differential diagnosis of TS includes folliculitis, keratosis pilaris, ulerythema, lichen spinulosus, lichen planopilaris, multiple minute digitate hyperkeratosis, spiculate demodicosis, alopecia mucinosa, trichostasis spinulosa, follicular graft-versus-host disease, and follicular spicules of multiple myeloma [5,9].…”

Widespread Hyperkeratotic Papules in a Transplant Recipient

“…Two other cases utilized spicule PCR analysis for confirmation of TSPyV. 11,12 This technique may represent a viable option for diagnostic confirmation in pediatric cases.…”