ESPN 51th Annual Meeting

ESPN 2018


 
Trichodysplasia Spinulosa in a kidney transplant recipient with concurrent JC, BK, and TSPyV viruria and decoy or degenerated cells.
Licia Peruzzi 1 Silvia Albertini 2 Marisa Gariglio 2 Cinzia Borgogna 2 Paola Savoia 3 Elisa Zavattaro 3 Renzo Boldorini 4 Roberta Camilla 1 Francesca Mattozzi 1 Roberto Bonaudo 1 Bruno Gianoglio 1

1- NEPHROLOGY DIALYSIS TRANSPLANT, R MARGHERITA CHILDRENS HOSPITAL TURIN ITALY
2- Virology Unit, Department of Translational Medicine, Novara Medical School, Italy
3- Dermatology Unit, Department of Health Sciences, Novara Medical School, Italy
4- Pathology Unit, Department of Health Sciences, Novara Medical School, Italy
 
Introduction:

Trichodysplasia spinulosa (TS) is a rare disfiguring skin disease of immunocompromized patients: papules and spines on the face, alopecia of eyebrows and lashes, thickening of affected skin layers. We present the first reported Italian case of TS in a 8 year-old kidney transplant recipient (second transplant at 5, induction with thymoglobulins and steroids, maintenance with steroids mycophenolate and tacrolimus, well functioning graft) for congenital nephrotic syndrome.

Material and methods:

He developed typical TS in September 2017 after steroid pulses for VII cranial nerve palsy during pertussis.

Results:

The first lesion  was on his face and gradually extended to neck, back and extremities. Histopathological examination showed keratotic masses expanding from the hair follicles, with widened infundibuli and inner follicular layers containing increased cellularity of nucleated eosinophilic inner root sheath cells with trichohyalin granules. PCR analysis of DNA from keratotic spines revealed high viral load values for TSPyV (6x106 copies/cell); only 2 copies/cells for MCPyV and none for HPyV6 and 7. HPV21 and 92 genotypes from the beta genus were found in the DNA  from skin swabs. Analysis of serum samples collected at diagnosis revealed JC DNA (approximately103 copies/mL). PCR analysis for TSPyV was not performed. Blood samples in March 2018 confirmed the positivity for JC DNA (500 copies/mL) and resulted also positive for BK DNA (600 copies/mL); TSPyV was always undetectable. Urine samples in February 2018 displayed high viral loads for TSPyV (4x105 copies/ml), BK (2x108 copies/ml) and JC (1x109 copies/mL). A huge number of decoy cells with enlarged ground-glass nuclei were observed alongside many other cells with degenerative changes in the cytoplasm and large hyaline granules very much resembling those observed in TS skin biopsy. All these cells were stained with antibodies against SV40 large T antigen. Electron microscopy is underway. He was treated without significant success with topical retinoids, systemic antibiotics for superinfection, valgancyclovir and is now under topical cidofovir. Because of JC viremia and transient increase in serum creatinine, immunosuppression was lowered; JC viremia progressively reduced,  renal function was good without proteinuria.

Conclusions:

 TSPyV infection is usually limited to the skin but might diffuse also to kidney graft. The graft biopsy will be performed soon.