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Dermatology > PHOTO-RELATED DISEASES
Hydroa Vacciniforme
Article Last Updated: Feb 28, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Quynh L Sebastian, MD, Clinical Instructor, Division of Dermatology, UCLA David Geffen School of Medicine
Quynh L Sebastian is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, and American Society for Laser Medicine and Surgery
Coauthor(s):
Raul DelRosario, MD, Surgical Pathology and Dermatopathology, South Coast Medical
Editors: Maureen B Poh-Fitzpatrick, MD, Professor Emerita of Dermatology and Special Lecturer, Columbia University; Professor of Medicine (Dermatology), University of Tennessee; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
Bazin hydroa vacciniforme, HV, photodermatosis
Background
Hydroa vacciniforme (HV) is a rare, chronic photodermatosis of unknown origin occurring in childhood. Recurrent vesicles on sun-exposed skin that heal with vacciniform or varioliform scarring characterize HV. The histopathologic features are distinctive and demonstrate intraepidermal reticular degeneration and cellular necrosis. Most cases remit spontaneously by late adolescence.
Pathophysiology
The etiology of HV is not known. HV may be a distinct entity distinguished by scarring or may occur within the spectrum of polymorphous light eruption. Skin lesions occur on sun-exposed skin, such as the face, ears, and hands, and they may be accompanied by a mild keratoconjunctivitis, photophobia, or constitutional symptoms.
Frequency
United States
The frequency of HV in the United States is unknown.
International
The frequency of HV varies according to country. In Scotland, for example, the reported prevalence is 0.34 cases per 100,000 people.
Mortality/Morbidity
No mortality is associated with typical HV.
Sex
Females have a higher incidence of HV than males and report earlier onset. Males who are affected have a longer course of disease than females.
Age
HV predominately affects children aged 3-15 years. Cases of HV in infants and elderly persons have also been described.
History
- Mild burning, itching or stinging in exposed sites beginning 30 minutes to 2 hours after sun exposure (common)
- Vesicles heal with varioliform scarring.
- The initial onset of lesions occurs in spring with recurrences in summer months.
- Constitutional symptoms (uncommon)
- Photophobia (seldom)
Physical
Skin and mucous membranes are the primary sites affected by HV.
- Skin
- Tense, edematous papules progress to clear, then, cloudy discrete vesicles.
- Lesions become umbilicated, necrotic papules on an erythematous base.
- Papules heal with hypopigmented depressed scars.
- Eye
- Mild keratoconjunctivitis
- Corneal clouding and stellate keratotic precipitates in the cornea, indicating an inflammatory keratitis (one report)
- Other symptoms
- Photo-onycholysis
- Limited partial absorption of bone and cartilage in severe HV
- Earlobe mutilation and flexion contracture of a digit (reported in 1 patient)
Causes
The development of lesions and their distribution suggest a causal relationship between HV and ultraviolet (UV) exposure, although the pathogenetic mechanism remains unknown. Ultraviolet A (UV-A) radiation is most often implicated. Two reports of HV in siblings have been documented, suggesting a genetic component to HV. Some cases have occurred in the setting of hematopoietic malignancy.
Actinic Prurigo
Chickenpox
Epidermolysis Bullosa
Epidermolysis Bullosa Acquisita
Erythropoietic Protoporphyria
Hartnup Disease
Herpes Simplex
Polymorphous Light Eruption
Porphyria Cutanea Tarda
Pseudoporphyria
Urticaria, Solar
Other Problems to be Considered
Hydroa aestivale
Lymphoproliferative disorders
Lab Studies
- CBC count to investigate constitutional symptoms
- Viral culture of blister fluid to rule out herpes virus infection
- Erythrocyte sedimentation rate and antinuclear antibody, anti-Ro, and anti-La antibody tests to rule out bullous lupus erythematosus
- Plasma or serum porphyrin assay to exclude cutaneous porphyrias
- DNA repair studies to rule out xeroderma pigmentosum
Imaging Studies
- Imaging studies are not routinely obtained as part of the workup of HV.
Other Tests
- Repetitive, broad-spectrum, UV-A phototesting
- Repetitive, broad-spectrum, low-dose UV-A irradiation can elicit lesions clinically and histologically identical to those produced by natural UV exposure, although results are inconsistent.
- The minimal erythemal dose of ultraviolet B (UV-B) and UV-A in these patients is usually normal.
Histologic Findings
Early lesions reveal an epidermal, multilocular vesicle with reticular degeneration. A dense, perivascular, lymphohistiocytic infiltrate with vessel hemorrhage and thrombosis may occur in the dermis. Late lesions show epidermal and dermal necrosis with a surrounding, chronic, inflammatory infiltrate. Direct immunofluorescence study results are usually normal, except for rare reports (2 patients) of C3 deposits at the dermoepidermal junction and in the small dermal vessels.
Medical Care
In addition to medication and certain restrictions on activity, management may include a prophylactic "hardening" course of phototherapy.
- Low-dose, narrow-band UV-B (TL-01) phototherapy, either daily or 3 times a week in the spring, may confer relative photoprotection.
- Psoralen photochemotherapy (PUVA) administered prophylactically in older patients may be effective in desensitization.
Consultations
- Consult a dermatologist for evaluation and management of HV.
- Consult an ophthalmologist for evaluation and management of associated eye findings.
Diet
Generally, no diet restrictions or requirements are indicated in the management of HV.
Activity
Strict sun avoidance, appropriate clothing, and frequent application of high sun protection factor (SPF) sunscreens with UV-A blocking agents are advised.
To date, no oral therapy reliably prevents the appearance of HV lesions. Oral antimalarials and beta-carotene are most commonly used and are occasionally useful, especially when combined with a strict sun avoidance program. Other therapies that have been used with varying success include thalidomide, azathioprine, cyclosporine, and fish oil supplementation.
Drug Category: Antimalarials
These agents suppress cutaneous lesions associated with many photodermatoses.
| Drug Name | Hydroxychloroquine (Plaquenil) |
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| Description | Can be used for suppression of lesions; mechanism of action in HV is unknown. |
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| Adult Dose | Active disease: 200-400 mg PO qd
Maintenance: 100-200 mg PO qd |
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| Pediatric Dose | 6 mg/kg/d PO for 4-6 wk, followed by 5 mg/kg/d; not to exceed 400 mg/d |
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| Contraindications | Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones |
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| Interactions | Serum levels increase with cimetidine; magnesium trisilicate may decrease absorption |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | Caution in hepatic disease, alcoholism, and G-6-PD deficiency; coadministration with other hepatotoxic drugs; children are especially sensitive to 4-aminoquinolone compounds in antimalarials; fatalities reported following accidental ingestion; perform periodic (6 mo) ophthalmologic examinations; test periodically for muscle weakness |
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| Drug Name | Chloroquine (Aralen) |
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| Description | Anti-inflammatory activity by suppressing lymphocyte transformation and may have photoprotective effect. Can be used for suppression of lesions; mechanism of action in HV is unknown. |
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| Adult Dose | 500 mg (300 mg base) PO qd |
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| Pediatric Dose | 3.5 mg base/kg/d PO |
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| Contraindications | Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones |
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| Interactions | Cimetidine may increase serum levels (possibly other 4-aminoquinolones); magnesium trisilicate may decrease absorption of 4-aminoquinolones |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | Caution in hepatic disease, G-6-PD deficiency, psoriasis, and porphyria; not recommended for long-term use in children; perform periodic ophthalmologic examinations; test for muscle weakness; retinopathy, tinnitus, nerve deafness, skin eruption, headache, anorexia, nausea, vomiting, and diarrhea may occur |
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Drug Category: Carotenoids
These agents are yellow-orange pigments that may have photoprotective properties in selected photodermatoses.
| Drug Name | Beta-carotene |
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| Description | Mechanism of action not completely elucidated but may relate to ability of carotenoids to quench photoexcited molecular species. Reduces severity of photosensitivity reactions in some patients with photodermatoses, especially erythropoietic protoporphyria (EPP). |
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| Adult Dose | 30-300 mg PO qd in divided doses |
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| Pediatric Dose | 30-150 mg PO qd in divided doses |
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| Contraindications | Documented hypersensitivity |
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| Interactions | Coadministration with vitamin A may result in additive toxic effects |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
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| Precautions | Caution in patients with renal or hepatic impairment; may increase risk for lung cancer in heavy smokers; may cause orange stools and cause diarrhea or loose stools at onset of therapy that tend to resolve with continued use |
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Drug Category: Psoralens
After intercalating with DNA and irradiation by UVA, it leads to formation of DNA psoralen adducts that cross-link DNA. This affects gene expression by inhibiting DNA replication, mitosis, and cell division. 5-methoxypsoralen (5-MOP, Bergapten, Psoraderm 5) has also been used, although it is not currently available in the United States.
| Drug Name | Methoxsalen (8-MOP, Oxsoralen) |
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| Description | Inhibits mitosis by binding covalently to pyrimidine bases in DNA when photoactivated by UV-A. |
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| Adult Dose | 0.57 mg/kg PO 1.5-2 h before exposure to UV light, at least 48 h apart |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; squamous cell cancer; cataract; light sensitive diseases, such as lupus or porphyria; ingestion of photosensitizing drugs; hepatitic disease; arsenic therapy |
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| Interactions | Toxicity increases with phenothiazines, griseofulvin, nalidixic acid, tetracyclines, thiazides, and sulfanilamides |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | Severe burns may occur from sunlight or UV-A if dose or treatment frequency exceeded; use only if response to other forms of therapy is inadequate; long-term use may increase risk of skin cancer |
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Further Inpatient Care
- HV is managed on an outpatient basis.
Further Outpatient Care
- Patients with extracutaneous findings associated with HV should be referred to the appropriate specialist.
In/Out Patient Meds
Deterrence/Prevention
- Avoiding the sun, frequently applying high SPF sunscreens with UV-A blocking agents, and wearing protective clothing may prevent episodes of HV.
Complications
- Complications are rare in HV. The most common severe sequela is the varioliform scarring.
Prognosis
- The prognosis is excellent; HV remits by adolescence.
Patient Education
- Patients are advised regarding strict sun avoidance, frequent application of high SPF sunscreens with UV-A blocking agents, and protective clothing.
Medical/Legal Pitfalls
- Failure to educate the patient regarding sun avoidance measures is poor management. The physician should also monitor for adverse effects and laboratory abnormalities while the patient is on oral therapy, particularly antimalarials.
Special Concerns
- Rigid sun avoidance may be socially devastating for the pediatric patient with HV. The physician should work closely with the parents and management team in supporting and educating the child regarding this disease.
| Media file 1:
Characteristic vesicular lesions occur on sun-exposed skin and heal with varioliform scarring. |
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Media type: Photo
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Hydroa Vacciniforme excerpt Article Last Updated: Feb 28, 2006
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