Clinical case

Annales de dermatologie et de v?n?r?ologie 2001; 128: 247-249
? Masson, Paris, 2001

Hydroa vacciniforme: dietary fish oil (Maxepa?) as an effective treatment

A.-B.?Modeste?⁽¹⁾, N.?Cordel?⁽¹⁾, X.?Balguerie?⁽¹⁾, D.?Leroy?⁽²⁾, Ph.?Lauret?⁽¹⁾, P.?Joly?<sup>(1)

(1)</sup>Clinique Dermatologique, Unit? INSERM U519, H?pital Charles Nicolle, CHU de Rouen.
⁽²⁾Service de Dermatologie, H?pital Cl?menceau, CHU de Caen.

Tir?s ? part : P.?JOLY Clinique Dermatologique, H?pital Charles Nicolle, 1, rue de Germont, 76031 Rouen Cedex. E-mail: Pascal.Joly@chu-rouen.fr

SUMMARY

Background

Hydroa vaccinniforme is a highly uncommon photodermatosis acquired in childhood. The clinical course is dominated by the risk of varioliform scars. Numerous treatments have been proposed with variable efficacy. One recent open study suggested dietary fish oil could be useful. We report a case of hydroa vacciniforme treated successfully with dietary fish oil (Maxepa?).

Case report

A 15-year-old girl consulted in May 1988 for vesiculobullous lesions typical of hydroa vacciniforme in photo-exposed areas. Anti-malaria drugs and photoprotection had been used for several years without success. Maxepa? was introduced in June 1998 and was followed by regression of the lesions within a few weeks despite the summer season. The treatment was interrupted at the patient's request due to fetid breath. Reintroduction of Maxepa? in April 1999 after an episode of recurrent lesions, again led to total resolution of the lesions within three weeks.

Discussion

Hydroa vacciniforme is an exceptional photodermatosis of uncertain etiology. It may possibly be related to an abnormal sensitivity to ultraviolet A. Fish oil rich in 3-omega polyunsaturated fatty acids would reduce the local inflammation triggered by sun exposure. Recent studies have demonstrated that dietary fish oil can increase the level of 3-omega polyunsaturated fatty acids in the epidermis and reduce the level of prostaglandins in the skin. Our case would appear to confirm the contribution of dietary fish oil to treatment despite the poor tolerance due to fetid breath.

R?SUM?

Hydroa vacciniforme?: traitement par huiles de poisson (Maxepa?).

A.-B.?Modeste, N.?Cordel, X.?Balguerie, D.?Leroy, Ph.?Lauret, P.?Joly

Introduction

L'hydroa vacciniforme est une photodermatose rare et acquise de l'enfance dont l'?volution est domin?e par le risque de survenue de cicatrices varioliformes. Les traitements sont multiples et d'efficacit? inconstante. Une ?tude ouverte r?cente a sugg?r? l'int?r?t des huiles de poisson. Nous rapportons une observation d'hydroa vacciniforme trait?e avec succ?s par les huiles de poisson?: Maxepa?.

Observation

Une jeune fille de 15 ans consultait en mai 1998 pour des l?sions v?siculobulleuses d'hydroa vacciniforme des zones photoexpos?es. Un traitement par antipalud?ens de synth?se et photoprotection externe avait ?t? instaur? depuis plusieurs ann?es sans efficacit?. Le Maxepa? ?tait introduit en juin 1998 avec une r?gression des l?sions en quelques semaines malgr? la p?riode estivale. Le traitement ?tait interrompu ? la demande de la malade en raison d'une haleine f?tide. Une r?introduction du Maxepa? en avril 1999, apr?s une r?cidive des l?sions, entra?nait de nouveau la disparition compl?te des l?sions en trois semaines.

Discussion

L'hydroa vacciniforme est une photodermatose rare d'?tiopathog?nie incertaine, possiblement li?e ? une sensibilit? anormale aux rayons ultraviolets A. Les huiles de poisson riches en acides gras polyinsatur?s 3 om?ga diminueraient les ph?nom?nes inflammatoires locaux d?clench?s par l'exposition solaire. Des ?tudes r?centes ont montr? qu'une suppl?mentation en huiles de poisson augmentait le taux d'acides gras polyinsatur?s 3 om?ga au sein de l'?piderme et diminuait le taux de prostaglandines dans la peau. Notre observation semble confirmer l'int?r?t de ce traitement, malgr? une mauvaise tol?rance li?e ? une haleine f?tide.

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Hydroa vacciniforme, described by Bazin in 1862 is a rare acquired photodermatosis that starts in childhood and spontaneously heals during adolescence [1]. It is characterized by vesiculobullous and crusted lesions of the sun exposed areas, which heal with varioliform scars and recur each summer until puberty. The risk of scarring motivates initiation of a treatment to improve patients' quality of life. The treatments proposed are inconsistently effective. A British study recently prompted interest in fish oil (Maxepa?) for the treatment of hydroa vacciniforme [2]. In this paper we report our observations of a patient suffering from hydroa vacciniforme who was successfully treated with Maxepa?.

Case report

A fifteen year-old girl presented in May 1998 because of vesiculobullous, crusted, and necrotic lesions associated with varioliform scars on the forehead, cheeks, ears, nasal bridge, and dorsal hands fig.?1. The lesions had evolved over seven years in flares lasting from March to September. A diagnosis of hydroa vacciniforme had been made several years earlier. The interview revealed no family history of photodermatosis or administration of photosensitizing or phototoxic substances. Other than the skin lesions the clinical examination was unremarkable. Normal porphyrin levels in the blood, urine, and stool excluded a diagnosis of erythropoietic protoporphyria. The absence of antinuclear antibodies also eliminated the diagnosis of bullous lupus erythematosus. Phototesing showed a normal minimal erythema/phototoxicity doses (MED, MPD) for UVB and UVA. Treatment with synthetic antimalarials and sun protection for several years was unsuccessful. Fish oil (Maxepa?, 8 capsules per day) was started in June 1998, and the lesions regressed for the duration of the summer fig.?2. Phototesting conducted after two months of treatment remained normal. At the patient's request, treatment was stopped in October 1998 because of fetid breath considered intolerable. In April 1999 the patient presented with a recurrence of hydroa vacciniforme. With the renewal of Maxepa? the lesions cleared within three weeks.

Discussion

Treatment of hydroa vacciniforme is difficult. Our case supports observations from Britain and advocates the utility of fish oil for this disorder [2]. The pathogenesis of this rare photodermatosis of childhood is uncertain. Many authors have pointed to a deficit in vitamin B6 (pyridoxine) with abnormal tryptophan tests, but supplementation did not modify the evolution of the disease [1]. Several authors have incriminated sensitivity to UVA, demonstrated by the reproduction of primary lesions of the disease during repeated UVA phototests [3, 4, 5]. The variola-type scarring that follows flares urges a search for an effective interim treatment, for use until the disease heals spontaneously at adolescence. There is no controlled therapeutic study in the literature, likely because of the exceptional nature of the disease. External sun protection is often insufficient. The multiplicity of systemic treatments proposed demonstrates the inconstancy of their effect. A few isolated results have been reported with carotenes, synthetic antimalarials, thalidomide, cyclosporine, and pyridoxine [6, 7, 8]. PUVA and UVB TL01 phototherapy appear relatively effective [9]. However, UVB must be used with care since it may provoke the development of lesions. A recent clinical study suggested the efficacy of fish oil in children suffering from hydroa vacciniforme [2]. Three children (mean age 7 years) were treated with Maxepa? for three months. One child cleared completely, another responded partially, and the third did not improve. Phototesting showed an improvement in the repeated phototests in one case and deterioration in the other two.

Dietary supplementation with fish oil increases levels of omega-3 polyunsaturated fatty acids and leads to decreased prostaglandin levels in the epidermis [10, 11]. The omega-3 fatty acids, present in high concentration in fish oil (Maxepa?), compete with arachidonic acid for binding to cyclooxygenases and lipooxygenases and thereby competitively reduce the synthesis of prostaglandins. Fish oil may therefore decrease inflammation provoked by sun exposure. Other reports have also shown that diets rich in omega-3 polyunsaturated fatty acids lead to decreased levels of other inflammatory mediators in the epidermis including interleukin-1, tumor necrosis factor, and leukotrienes. In addition to their UVB -protective effect, fish oils also increase the UVA photosensitivity threshold [11]. In our patient, the prior introduction of Maxepa? did not permit us to evaluate the efficacy of the product and the natural evolution of the disease as she approached adolescence. However, the recurrence of lesions 6 months after suspension of treatment and their resolution after renewal of the product suggests that the resolution was not due to spontaneous healing of the disease during adolescence but rather to the efficacy of fish oil. The efficacy of Maxepa? remains to be confirmed in a larger population of patients. However, the development of fetid breath or of burping, side effects well known in the literature, limit treatment because of social acceptability.

To cite the present paper, use exclusively following reference: Modeste AB, Cordel N, Balguerie X, Leroy D, Lauret Ph, Joly P. Hydroa vacciniforme: traitement par huiles de poisson (Maxepa?) (full text in english on e2med.com/ad). Ann Dermatol Venereol 2001; 128: 247-9.

Figure 1.
Vesicular and crusted lesions of the face.

Figure 2.
Resolution of the eruption after three weeks of treatment.

REFERENCES

[1] Rebondy JP, Sans B, Bazex J, Cathala-Laurent J. Hydroa vacciniforme. Ann Dermatol Venereol 1986;113:1093-5.

[2] Rhodes LE, White SI. Dietary fish oil as a photoprotective agent in hydroa vacciniforme. Br J Dermatol 1998;138:173-8.

[3] Halasz CL, Leach EE, Walther RR, Poh-Fitzpatrick MB. Hydroa vacciniforme: Induction of lesions with ultraviolet A. J Am Acad Dermatol 1983;8:171-6.

[4] Eramo LR, Garden JM, Esterly NB. Hydroa vacciniforme: Diagnosis by repetitive ultraviolet-A phototesting. Arch Dermatol 1986;122:1310-3.

[5] Hann SK, Im S, Park YK, Lee S. Hydroa vacciniforme with unusually severe scar formation: Diagnosis by repetitive UVA phototesting. J Am Acad Dermatol 1991;25:401-3.

[6] Sonnex TS, Hawk JLM. Hydroa vacciniforme: a review of ten cases. Br J Dermatol 1988;118:101-8.

[7] Rotteleur G, Thomas P, Desmons F. L'hydroa vacciniforme de Bazin. A propos de trois observations originales. Etude photobiologique et m?tabolique. Discussion ?tiopathog?nique. Ann Pediatr 1980;27:441-8.

[8] Blackwell V, Mcgregor JM, Hawk JLM. Hydroa vacciniforme presenting in an adulte successfully treated with cyclosporin A. Clin Exp Dermatol 1998;23:73-6.

[9] Collins P, Ferguson J. Narrow-band UVB (TL-01) phototherapy: an effective preventive treatment for the photodermatoses. Br J Dermatol 1995;132:956-63.

[10] Rhodes LE, O'Farrell S, Jackson MJ, Friedmann PS. Dietary fish-oil supplementation in humans reduces UVB-erythemal sensitivity but increases epidermal lipid peroxidation. J Invest Dermatol 1994;103:151-4.

[11] Rhodes LE, Durham BH, Fraser WD, Friedmann PS. Dietary fish oil reduces basal and ultraviolet B-generated PGE2 levels in skin and increases the threshold to provocation of polymorphic light eruption. J Invest Dermatol 1995;105:532-5.

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