INTRODUCTION: In the late spring of 2017, a 15 years old male heart transplant patient was seen in the Dermatology outpatient department of Thanjavur Medical College and Hospital because of spots and spines in the face. One and a half year prior to presentation, a year after transplantation and start of immune suppressive treatment, his skin condition had started to develop with desquamation of the eyebrows, gradually followed by the development of follicular, skin-colored, indurated papules on the eyebrows, nose, ears, malar-region, and forehead. Subsequent symptoms were a loss of eyebrow hairs and part of the eyelashes. From the enlarged follicular orifices, small hyperkeratotic white-yellowish spicules started to protrude on the eyebrows, nose, and ears. Comparable solitary hyperkeratotic papules and spicules also developed on the legs. Over a period of one year, as the skin of his ears, eyebrows, and nose had thickened, his overall facial appearance had changed dramatically.
What is your diagnosis? Describe the pathology behind this? And what are the investigation and management?
A case of trichodysplasia spinulosa.
- It is a very rare case of immunosuppressives.
- Trichodysplasia spinulosa (also known by many other names, including viral-associated trichodysplasia spinulosa, viral-associated trichodysplasia, pilomatrix dysplasia and ciclosporin-induced folliculodystrophy) is a rare cutaneous condition that has been described almost exclusively in immunocompromised patients, usually organ transplant recipients, on regimens of immunosuppressive drugs.
- TS has been described as an emerging infectious disease.
- In the above picture the nose of a patient diagnosed with trichodysplasia spinulosa. The left panel shows characteristic findings of facial papules, protrusive keratotic spicules, and thickened skin.
- Fresh colored to erythematous papules
- protrusive “spicules” or spines made of keratin
- alopecia of affected skin, typically the eyebrows
- Immunocompromised individual
- Hematolymphoid malignancy
- Organ transplant recipient
- That trichodysplasia spinulosa is caused by a polyomavirus called trichodysplasia spinulosa polyomavirus (TSPyV) or Human polyomavirus 8.
- The hyperproliferation of keratinocyte inner root sheath cells in which large aggregates of viral particles can be found suggests that TSPyV actively replicates in these cells.
- This is thought to underlie the clinical manifestations of TS. There is limited evidence implicating the large tumor antigen as responsible for inducing cellular proliferation through pathways involving phosphorylated retinoblastoma protein (pRB).
- Thickening of skin called acanthosis,
- Hair follicle shows enlarged and dysmorphic appearance,
- Characteristics of eosinophilic protein granules.
- Antibodies against major capsid protein VP1, the major component of the viral capsid, can be used to confirm the presence of viral particles in cell nuclei.
- Electron microscopy can also be used to detect viral particles.
- Quantification of viral load can be performed using quantitative PCR, as affected skin demonstrates much higher viral loads compared to the unaffected skin or to asymptomatic individuals who test positive for viral DNA.
- Most of the cases spontaneously cured.
- But valganciclovir and acyclovir helpful to treat this condition.