Hydroa vacciniforme

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2Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Hydroa vacciniforme (HV) is a rare skin condition that causes an unusual and severe sensitivity to sunlight, mainly ultraviolet (UV) light. It most commonly begins in childhood and is characterized by a recurring blistering rash that appears on sun-exposed areas of the skin, such as the face, ears, and backs of the hands. After sun exposure, small fluid-filled blisters (vesicles) develop, which then break open and form crusted sores. As these sores heal, they leave behind permanent pock-like scars that resemble smallpox vaccination marks — which is where the name 'vacciniforme' comes from. The condition typically follows a seasonal pattern, flaring up in spring and summer when sun exposure is greatest, and improving during winter months. In most cases, hydroa vacciniforme is a self-limiting condition that gradually improves or resolves by early adulthood. However, there is a more severe form called hydroa vacciniforme-like lymphoproliferative disorder (HVLPD), which is associated with chronic Epstein-Barr virus (EBV) infection and can progress to a type of lymphoma. This severe form is more commonly seen in certain populations, particularly in Latin America and Asia. Treatment focuses on strict sun protection and avoidance of UV light. There is no cure for hydroa vacciniforme, but symptoms can be managed with photoprotective measures, and in some cases, medications that suppress the immune system or antiviral therapies may be considered, especially for the more severe lymphoproliferative form.

Key symptoms:

Blistering rash on sun-exposed skinSmall fluid-filled blisters on the face, ears, and handsCrusting and scabbing of skin soresPermanent pock-like scars after healingSkin redness and swelling after sun exposureItching or burning sensation on affected skinSeasonal flare-ups in spring and summerFacial swelling, especially around the eyesSkin necrosis (tissue death) in severe casesFever in severe formsEnlarged lymph nodes in severe formsEnlarged liver or spleen in severe formsSensitivity to even brief sun exposure

Clinical phenotype terms (18)— hover any for plain English
Hydroa vacciniformeHP:0032381ScarringHP:0100699Malar rashHP:0025300Papulovesicular eruptionHP:0033700Red eyeHP:0025337Superficial dermal perivascular inflammatory infiltrateHP:0031190Linear C3 deposits along the epidermal basement membrane zoneHP:0031541MalaiseHP:0033834PorphyrinuriaHP:0010473
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Hydroa vacciniforme.

View clinical trials →

No actively recruiting trials found for Hydroa vacciniforme at this time.

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Search ClinicalTrials.gov ↗Join the Hydroa vacciniforme community →

Specialists

2 foundView all specialists →

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Hydroa vacciniforme.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Hydroa vacciniforme

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.Does my child have classic hydroa vacciniforme or the more severe lymphoproliferative form?,Should my child be tested for Epstein-Barr virus infection?,What is the best sun protection strategy for my child's specific situation?,Are there any medications that could help reduce flare-ups or scarring?,How often should we schedule follow-up visits, and what should we monitor at home?,Is there a risk that this condition could progress to lymphoma, and what warning signs should I watch for?,Can you recommend any psychological support resources for my child to cope with the social impact of this condition?

Common questions about Hydroa vacciniforme

What is Hydroa vacciniforme?

Hydroa vacciniforme (HV) is a rare skin condition that causes an unusual and severe sensitivity to sunlight, mainly ultraviolet (UV) light. It most commonly begins in childhood and is characterized by a recurring blistering rash that appears on sun-exposed areas of the skin, such as the face, ears, and backs of the hands. After sun exposure, small fluid-filled blisters (vesicles) develop, which then break open and form crusted sores. As these sores heal, they leave behind permanent pock-like scars that resemble smallpox vaccination marks — which is where the name 'vacciniforme' comes from. Th

How is Hydroa vacciniforme inherited?

Hydroa vacciniforme follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Hydroa vacciniforme typically begin?

Typical onset of Hydroa vacciniforme is childhood. Age of onset can vary across affected individuals.

Which specialists treat Hydroa vacciniforme?

2 specialists and care centers treating Hydroa vacciniforme are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.