Overview
Hydroa vacciniforme-like lymphoma (HVLL) is a very rare and serious type of lymphoma (cancer of the lymph system) that mainly affects the skin. It is closely related to a milder skin condition called hydroa vacciniforme, but HVLL is more aggressive and involves cancerous growth of certain white blood cells called T cells or natural killer (NK) cells. The disease is strongly linked to chronic infection with the Epstein-Barr virus (EBV), the same virus that causes mononucleosis. HVLL typically begins in childhood or adolescence and is most commonly seen in people of Asian, Latin American, or Indigenous descent. The main symptoms include severe blistering skin lesions that appear on sun-exposed areas such as the face, ears, and hands. These blisters can become deep, leave pitted scars (similar to smallpox vaccination scars, hence the name 'vacciniforme'), and may also appear on areas not exposed to the sun. Patients often experience fever, swelling of the liver and spleen, swollen lymph nodes, and general feelings of being unwell. The disease can wax and wane over months to years but tends to progress over time. Treatment is challenging and may include chemotherapy, immunotherapy, and in some cases, bone marrow (stem cell) transplantation. Because this lymphoma is so rare, there is no single standard treatment protocol, and outcomes vary widely. Early diagnosis and referral to specialized cancer centers are important for the best possible care.
Also known as:
Key symptoms:
Severe blistering skin rash, especially on the face, ears, and handsDeep skin ulcers that leave pitted or pockmark-like scarsSkin lesions triggered or worsened by sun exposureSkin lesions appearing on areas not exposed to the sunSwelling of the face, especially around the eyes and cheeksFever that comes and goesSwollen lymph nodesEnlarged liverEnlarged spleenFatigue and general feeling of being unwellWeight lossSensitivity to sunlight (photosensitivity)Tissue death (necrosis) in skin lesionsNight sweats
Sporadic
Usually appears on its own, not inherited from a parent
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
1 availableValchlor
VALCHLOR is indicated for the topical treatment of Stage IA and IB mycosis fungoides-type cutaneous T-cell lymphoma in patients who have received prior skin-directed therapy
Clinical Trials
View all trials with filters →No actively recruiting trials found for Hydroa vacciniforme-like lymphoma at this time.
New trials open frequently. Follow this disease to get notified.
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 Hydroa vacciniforme-like lymphoma.
Community
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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.
Questions for your doctor
Bring these to your next appointment
- Q1.What stage is the lymphoma, and has it spread beyond the skin?,What treatment plan do you recommend, and what are the expected benefits and risks?,Is bone marrow transplantation an option for my case, and when should we consider it?,Are there any clinical trials available for this type of lymphoma?,What sun protection measures should I follow, and how strictly?,How will treatment affect my daily life, school, or work?,What signs or symptoms should prompt me to seek emergency care?
Common questions about Hydroa vacciniforme-like lymphoma
What is Hydroa vacciniforme-like lymphoma?
Hydroa vacciniforme-like lymphoma (HVLL) is a very rare and serious type of lymphoma (cancer of the lymph system) that mainly affects the skin. It is closely related to a milder skin condition called hydroa vacciniforme, but HVLL is more aggressive and involves cancerous growth of certain white blood cells called T cells or natural killer (NK) cells. The disease is strongly linked to chronic infection with the Epstein-Barr virus (EBV), the same virus that causes mononucleosis. HVLL typically begins in childhood or adolescence and is most commonly seen in people of Asian, Latin American, or Ind
How is Hydroa vacciniforme-like lymphoma inherited?
Hydroa vacciniforme-like lymphoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Hydroa vacciniforme-like lymphoma typically begin?
Typical onset of Hydroa vacciniforme-like lymphoma is childhood. Age of onset can vary across affected individuals.
Which specialists treat Hydroa vacciniforme-like lymphoma?
14 specialists and care centers treating Hydroa vacciniforme-like lymphoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.