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Clinical and Experimental Dermatology

Facial spicules and pink papules in a renal transplant recipient F. R. Ali,1 A. Aslam,1 L. Motta2 and J. T. Lear1 1 Dermatology Centre and 2Department of Cellular Pathology, University of Manchester, Manchester Academic Health Science Centre, Salford Royal NHS Foundation Trust, Manchester, UK

doi: 10.1111/ced.12615

Clinical findings A 42-year-old woman presented to our dermatology transplant clinic with a pruritic, red facial rash. Six months previously, she had received a cadaveric renal transplant for end-stage renal disease caused by underlying Alport-type basement membrane nephropathy. Medications at the time of presentation included mycophenolate mofetil (720 mg twice daily), tacrolimus (2 g twice daily), lercarnidipine, atorvastatin and aspirin. Physical examination revealed protuberant keratotic spicules, superimposed upon pink papules, which were distributed symmetrically on both cheeks, the forehead and the nose (Fig. 1).

Histopathological findings Biopsies of the skin on the patient’s nose and face were taken. In both specimens, there was marked cystic dilatation of hair follicles and hyperplasia of the inner root sheath cells. Within the hair follicular epithelium, there were abundant intracytoplasmic eosinophilic globules, in keeping with viral inclusions. Immunohistochemistry for SV40 (polyomavirus) demonstrated weak to moderate nuclear staining of epithelial cells, particularly in the dilated hair follicles (Fig. 2).

Figure 1 Protuberant spicules studded upon pink papules,

distributed symmetrically on both cheeks, the forehead and the nose.

What is your diagnosis?

Correspondence: Dr John T. Lear, Dermatology Centre, University of Manchester, Manchester Academic Health Science Centre, Salford Royal NHS Foundation Trust, Stott Lane, Manchester M6 8HD, UK E-mail: [email protected] Conflict of interest: JTL has accepted honoraria for speaking at meetings by Leo, Galderma, Almirall, Astellas, Roche and GSK. The other authors declare that they have no conflicts of interest. Accepted for publication 30 September 2014

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Figure 2 (a) Prominent cystic dilatation of hair follicles with intracytoplasmic eosinophilic globules within the hair follicular epithelium

(haematoxylin and eosin, original magnification 9 100); (b) cells positive for SV40 (original magnification 9 40).

Diagnosis Trichodysplasia spinulosa (TS).

Discussion TS, also known as pilomatrix dysplasia, ciclosporininduced folliculodystrophy or virus-associated trichodysplasia, is a rare viral infection that presents with folliculocentric friable spiny spicules and erythematous papules.1,2 Reported cases have pronounced involvement of the central face, and the majority have involvement of extrafacial sites, including the extremities and the eyebrows. Other less frequently affected sites include the trunk, ears, eyelashes and scalp. A review of all published cases of TS from 1995 to 2011 suggested a median age of onset of 27 years (range 5–70 years) and equal sex preponderance.3 The cases reported to date have all been in immunosuppressed individuals, predominantly in solid-organ transplant recipients and in patients with haematological malignancies.2,3 Additionally, one patient with systemic lupus erythematosus also developed TS after taking various immunosuppressive medications (including corticosteroids, rituximab, mycophenolate mofetil and azathioprine).4 The aetiological agent of TS has recently been identified as trichodysplasia spinulosa-associated polyomavirus.5 The polyomaviridae are double-stranded DNA viruses, present in mammals and birds. Several polyomaviruses have been described in humans, of

ª 2015 British Association of Dermatologists

which the two best-known are JC polyomavirus (causing progressive multifocal leucoencephalopathy in patients with acquired immunodeficiency syndrome) and BK polyomavirus (causing nephropathy in renal transplant recipients). Subclinical infection is believed to be present in large sections of the population, with infection becoming clinically apparent, owing to unchecked viral replication, when the patient is immunocompromised. Histological features of TS include markedly dilated hair follicles, hyperkeratotic infundibula and copious inner root sheath cells (giving rise to the keratotic spicules). Additionally, intracytoplasmic eosinophilic globules (representative of viral inclusions) are present within the hair follicular epithelium. While there are some reports of self-resolution, symptoms are ameliorated with reduction in the dose of immunosuppressive drugs. Other reported treatments comprise topical agents (including retinoids, imiquimod, corticosteroids, tacrolimus), antiviral preparations (e.g. cidofovir, aciclovir) and systemic therapies (e.g. valganciclovir, antibiotics, minocycline). In consultation with the renal team, our patient’s immunosuppressive dose has been reduced and topical imiquimod has been commenced. The patient has demonstrated a good therapeutic response to date. Dermatologists and dermatopathologists should be vigilant for this rare entity that may complicate immunosuppressive therapy or haematological malignancies, and recognize that modulation of immunosuppression may facilitate resolution of TS.

Clinical and Experimental Dermatology (2015) 40, pp816–818

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Learning points

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TS is a rare viral infection affecting immunocompromised individuals. • TS is caused by trichodysplasia spinulosa-associated polyomavirus. • TS commonly presents with central facial erythematous papules studded with keratotic spicules. • Treatment of TS most commonly comprises modulation of immunosuppression and antiviral agents.

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in an immunocompromised host. J Investig Dermatol Symp Proc 1999; 4: 268–71. Jawa P, Jain K, Allen R et al. Trichodysplasia spinulosa. Kidney Int 2014; 85: 715. Fischer MK, Kao GF, Nguyen HP et al. Specific detection of trichodysplasia spinulosa-associated polyomavirus DNA in skin and renal allograft tissues in a patient with trichodysplasia spinulosa. Arch Dermatol 2012; 148: 726– 33. Moktefi A, Laude H, Brudy Gulphe L et al. Trichodysplasia spinulosa associated with lupus. Am J Dermatopathol 2014; 36: e70–4. van der Meijden E, Janssens RW, Lauber C et al. Discovery of a new human polyomavirus associated with trichodysplasia spinulosa in an immunocompromized patient. PLoS Pathog 2010; 6: e1001024.

References 1 Haycox CL, Kim S, Fleckman P et al. Trichodysplasia spinulosa: a newly described folliculocentric viral infection

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Clinical and Experimental Dermatology (2015) 40, pp816–818

ª 2015 British Association of Dermatologists

Facial spicules and pink papules in a renal transplant recipient.

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