BI09: Rapid and progressive papular facial and truncal rash due to trichodysplasia spinulosa-associated polyomavirus: a diagnosis to consider in immunosuppressed patients

Zahra Moledina, Esther Burden-Teh, Kusum Kulkarni and Shanti Ayob

Nottingham University Hospitals NHS Trust, Nottingham, UK

We would like to share a case of a rare but distinctive rash in an immunosuppressed patient and discuss the limited available evidence on approaches to treatment. A 27-year-old woman presented with a sudden-onset mildly pruritic facial rash, associated with facial swelling, of 3 months’ duration. Since the onset of the rash, she had worsening swelling and enlargement of her nose, which significantly altered her facial appearance. Moreover, she noticed rough skin on palpation of her trunk and limbs. No triggers could be identified. Past medical history included a bilateral lung transplant for cystic fibrosis 18 months prior to rash onset, complicated by mild acute rejection. In addition to this, she had chronic kidney disease stage 3/4, with renal biopsy features suggestive of tacrolimus toxicity. Physical examination revealed a scaly, symmetrical rash predominantly affecting the central face, with milder trunk and limb involvement. A transitional margin of papules was observed behind the ears and the facial skin felt thickened with oedema/infiltration. Scaly papules formed distinctive spicules. Eyebrow and eyelash alopecia were noted. Suggested initial differentials included lichen spinosus, atypical eczema, papular mycosis fungoides, lichen myxoedematosus, graft-versus-host disease and trichodysplasia spinulosa (TS). Histological examination of an incisional biopsy specimen from the thickened skin on her cheek and a punch biopsy of rash on her upper arm revealed numerous hair follicles, plugged by hyperkeratosis, parakeratosis and nuclear debris. The infundibular parts of the hair follicle appeared to be dilated, and viral particles were identified. There was also some perifollicular and perivascular chronic inflammation. Overall, the clinical and histological features correlated with TS. TS is a rare dermatological condition and fewer than 70 cases have been reported in the literature (Curman P, Näsman A, Brauner H. Trichodysplasia spinulosa: a comprehensive review of the disease and its treatment. J Eur Acad Dermatol Venereol 2021; 35: 1067–76). It was first identified in 1995 and the causative agent, TS-associated polyomavirus, was first isolated in 2009 (Curman et al.). It affects immunosuppressed individuals, and the majority of cases are observed in organ transplant recipients. There are no established treatment guidelines and effective treatments reported in case reports include topical cidofovir 3%, reduction in immunosuppression and oral valganciclovir (Curman et al.). Following review at our regional dermatology clinic, a suggested treatment regimen included combination of topical adapalene and topical cidofovir, which was only available in a 1% formulation from our formulary. Subsequently, following liaison with her specialist respiratory and transplant teams, treatment with oral valganciclovir was initiated. However, it was subsequently stopped as it impacted on her already impaired renal function. Therapeutic options for this rare condition are currently limited, but targeted treatments against the polyomavirus may become available in the future.