Overview
Virus-associated trichodysplasia spinulosa (VATS), also known as trichodysplasia spinulosa or pilomatrix dysplasia, is a rare skin disease caused by infection with the trichodysplasia spinulosa-associated polyomavirus (TSPyV). It occurs almost exclusively in immunosuppressed individuals, particularly organ transplant recipients on immunosuppressive therapy, though it has also been reported in patients with hematologic malignancies or those receiving other forms of immunosuppression. The condition primarily affects the skin, especially the face, and is characterized by the development of follicular papules and keratin spicules (spine-like projections) on the nose, eyebrows, ears, and other facial areas. Patients may also experience alopecia of the eyebrows and eyelashes, facial thickening or leonine facies, and widespread spiny papules that can extend to the trunk and extremities. The underlying mechanism involves TSPyV infection of the inner root sheath cells of hair follicles, leading to abnormal proliferation and distorted hair follicle differentiation. Histopathologically, the disease shows enlarged, dysplastic inner root sheath keratinocytes with characteristic eosinophilic intranuclear inclusion bodies. Diagnosis is confirmed through skin biopsy and detection of TSPyV DNA by polymerase chain reaction (PCR). Treatment primarily involves reduction of immunosuppression when feasible, which can lead to resolution of the lesions. Topical cidofovir, an antiviral agent, has been used with reported success in several cases. Other treatments that have been tried include oral valganciclovir and topical retinoids. Spontaneous resolution may occur if immune function is restored. Given the rarity of the condition, there are no standardized treatment guidelines, and management is largely based on case reports and small case series.
Also known as:
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
10 eventsShanghai Pulmonary Hospital, Shanghai, China — NA
University Hospital, Strasbourg, France — PHASE3
Chinese University of Hong Kong — NA
Bursa City Hospital — NA
Georgios Kotsovolis — NA
Beijing Tiantan Hospital — NA
Beijing Tiantan Hospital — NA
National Taiwan University Hospital — NA
The First Affiliated Hospital of Guangzhou Medical University — NA
The First Affiliated Hospital of Guangzhou Medical University — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Virus-associated trichodysplasia spinulosa.
20 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Virus-associated trichodysplasia spinulosa.
Community
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Start the conversation →Latest news about Virus-associated trichodysplasia spinulosa
Disease timeline:
New recruiting trial: SPSIP Block vs SAP Block for Post-VATS Pain
A new clinical trial is recruiting patients for Virus-associated trichodysplasia spinulosa
New recruiting trial: The Efficacy and Safety of Liposomal Bupivacaine for Rhomboid Intercostal Nerve Block in Relieving Postoperative Pain After Video-assisted Thoracoscopic Surgery
A new clinical trial is recruiting patients for Virus-associated trichodysplasia spinulosa
New recruiting trial: Intrapleural Nebulization of Bupivacain to Reduce Postoperative Pain
A new clinical trial is recruiting patients for Virus-associated trichodysplasia spinulosa
New recruiting trial: Post-operative Urinary Retention (POUR) Following Thoracic Surgery
A new clinical trial is recruiting patients for Virus-associated trichodysplasia spinulosa
New recruiting trial: ESP vs SPSIP Block in VATS Analgesia
A new clinical trial is recruiting patients for Virus-associated trichodysplasia spinulosa
New recruiting trial: Assessing the Impact of Additional Serratus Anterior Block Within 24-Hour on Postoperative Pain After Rhomboid Intercostal Block in VATS Patients
A new clinical trial is recruiting patients for Virus-associated trichodysplasia spinulosa
New recruiting trial: Comparison of SPSIP Block and SAP Block in Non-Intubated Uniportal VATS.
A new clinical trial is recruiting patients for Virus-associated trichodysplasia spinulosa
New recruiting trial: Robotic Versus Video-assisted Lobectomy/Segmentectomy for Lung Surgery
A new clinical trial is recruiting patients for Virus-associated trichodysplasia spinulosa
New recruiting trial: Opioid-Free Combined Anesthesia With Spontaneous Breathing for VATS
A new clinical trial is recruiting patients for Virus-associated trichodysplasia spinulosa
New recruiting trial: Combined Zephyr Valve System With Inter-lobar Fissure Completion for Lung Volume Reduction in Emphysema
A new clinical trial is recruiting patients for Virus-associated trichodysplasia spinulosa
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Common questions about Virus-associated trichodysplasia spinulosa
What is Virus-associated trichodysplasia spinulosa?
Virus-associated trichodysplasia spinulosa (VATS), also known as trichodysplasia spinulosa or pilomatrix dysplasia, is a rare skin disease caused by infection with the trichodysplasia spinulosa-associated polyomavirus (TSPyV). It occurs almost exclusively in immunosuppressed individuals, particularly organ transplant recipients on immunosuppressive therapy, though it has also been reported in patients with hematologic malignancies or those receiving other forms of immunosuppression. The condition primarily affects the skin, especially the face, and is characterized by the development of follic
Are there clinical trials for Virus-associated trichodysplasia spinulosa?
Yes — 20 recruiting clinical trials are currently listed for Virus-associated trichodysplasia spinulosa on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Virus-associated trichodysplasia spinulosa?
20 specialists and care centers treating Virus-associated trichodysplasia spinulosa are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.