Hydroa Vacciniforme is a very rare chronic, scarring, photo induced disorder initially seen in childhood and resolves during puberty. However few cases may persist for life. The exact cause of this disorder is unknown, but it is thought to be induced by UVB radiation. Milder form of the disease is commonly seen in females, while, severe form is seen is common in males.
Symptoms of Hydroa Vacciniforme
Hydroa Vacciniforme eruption is common during spring and summer and is characterized by stinging or burning sensation, followed by appearance of symmetrically scattered and tender vesicles or papulo-vesicles lesion within few hours of sun exposure. The lesion may then crust and heal with permanent hypopigmented scar within in weeks, which can be very disfiguring. Most common affected sites are face, outer aspect of arms and dorsum of the hands. It is rarely seen on other areas of the body.
In most cases, the disease resolves spontaneously by puberty. In few cases, the disease may continue to mid-age and may persist for life.
Lab Testing
Your doctor may perform blood, urine and stool tests to exclude other possible disease like, cutaneous porphyria and cutaneous lupus. Other viral studies may also be performed to exclude viral infection such as herpes.
Other possible condition to consider
- Herpes simplex
- Polymorphic light eruption
- Xeroderma pigmentosum
- Erythropoietic protoporphyria
- Subacute cutaneous lupus
Treatment of Hydroa Vacciniforme
The mainstay of treatment is to avoid ultraviolet radiation. This can be achieved by wearing appropriate clothing and the use of broad-spectrum sunscreen of at-least SPF 30. Other therapy with antimalarials has also been tried with mixed results. For patients with chronic cases, prophylactic phototherapy with narrowband UVB or PUVA, may be helpful [1,2] but it may further exacerbate the condition, so caution should be taken. When nothing works oral steroids and immunosuppressive therapy may also be tried, but the results are not satisfactory in most cases. Prophylactic therapy with dietary fish oil [3] may be beneficial in some patients; however poor tolerance limits its use.
References:
[1] Hann SK, Im S, Park Y-K, Lee S. Hydroa vacciniforme with unusually severe
scar formation: diagnosis by repetitive UVA phototesting. J Am Acad Dermatol
1991; 25: 401–3.
[2] Halasz CLG, Leach EE, Walther RR, Poh-Fitzpatrick MB. Hydroa vacciniforme
induction of lesions with ultraviolet A. J Am Acad Dermatol 1983; 8: 171.
[3] Rhodes LE, White SI. Dietary fi sh oil as a photoprotective agent in hydroa vacciniforme. Br J Dermatol 1998; 138: 173–8.
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