Trichodysplasia Spinulosa in a 20-Month-Old Girl With a Good Response to Topical Cidofovir 1%

Abstract

Trichodysplasia spinulosa (TS) is a rare entity, characterized by a follicular digitate keratosis predominantly affecting the face and variable degrees of hair loss, most severely facial hair, that occurs in immunosuppressed individuals, and is considered to be a viral infection caused by a human polyomavirus, the "TS-associated polyomavirus." Histologically it is characterized by hair follicles with excessive inner root-sheath differentiation and intraepithelial viral inclusions. Correlation of these findings with clinical features is required for diagnosis. Treatment with antiviral agents appears to be the most effective. We report the occurrence of TS in a 20-month-old girl with multivisceral transplantation due to short-bowel syndrome secondary to intestinal atresia and gastroschisis. The patient was treated with cidofovir 1% cream, with significant improvement and without any adverse effects. We describe the youngest patient, to our knowledge, with TS.

Trichodysplasia Spinulosa in a 7‐Year‐Old Boy Managed Using Physical Extraction of Keratin Spicules

“…TS is characterized by erythematous to flesh-colored, folliculocentric, centro-facial and auricular papules with keratin spicule formation and varying degrees of alopecia. Affected individuals have ranged from 20 months to 70 years of age with no apparent gender predilection 10 .…”

Trichodysplasia spinulosa mimicking lichen nitidus in a renal transplant patient

“…TS typically presents on the central face and ears with multiple skin‐colored, folliculocentric papules and central keratotic spicules. The additional presence of the rash on the trunk, arms, and legs has also been reported . Skin lesions are usually asymptomatic, but are occasionally associated with mild pruritus.…”

“…The development of keratin spicules on the face and ears in the context of immunosuppression, particularly organ transplantation or lymphoreticular malignancy, should raise suspicion for TS. Characteristic histopathologic changes, electron microscopy, or PCR of either a biopsy sample or skin scraping can confirm the diagnosis of TS . Histologically, TS is characterized by dilated hair follicles with proliferation of the inner root sheath cells and the presence of cytoplasmic eosinophilic inclusions consisting of trichohyalin .…”

“…Characteristic histopathologic changes, electron microscopy, or PCR of either a biopsy sample or skin scraping can confirm the diagnosis of TS . Histologically, TS is characterized by dilated hair follicles with proliferation of the inner root sheath cells and the presence of cytoplasmic eosinophilic inclusions consisting of trichohyalin . Electron microscopy can be used to confirm the diagnosis by identifying intranuclear viral inclusions within the hair follicle keratinocytes .…”

“…A reduction in immunosuppressive medications allows immune recovery and facilitates a reduction in viral load. Alternatives that have been reported to be successful include IV cidofovir, topical cidofovir (1%‐3%) cream, oral valganciclovir, and leflunomide . Additionally, physical extraction of keratin spicules has been utilized as a cost‐effective and safe option for temporary improvement in appearance .…”