Hydroa vacciniforme L56.8

Bazin 1862

Rare light dermatosis with pathologically increased photosensitivity and intermittent formation of pruritic and burning erythema, papules, urticarial plaques, blisters and consecutive scars in the exposed areas. Disturbances of the porphyrin metabolism do not exist!

Some authors define hydroa vacciniforme as a cutaneous subset of Epstein-Barr virus (EBV)-associated lymphoproliferative T / NK diseases (LPDs) (Iwatsuki K et al. 2019).

A close relationship to polymorphic light dermatosis is assumed and thus a delayed-type hypersensitivity reaction to a hitherto unknown photo-induced antigen is suspected.

However, viral infections probably play a comorbid role. Thus, associations with Epstein-Barr virus infection have been described (serological EBV detection and PCR detection of EBV antigen in lesional tissue), as well as (much less frequently) with herpes simplex virus infection.

Genetic factors are suspected in familial clustering.

Sudden erythema, usually occurring a few hours after sun exposure, at the light-exposed areas, circumscribed erythema with the formation of up to 2.0 cm large, partially umbilical blisters with serous or haemorrhagic content.

Drying up, formation of a blackish scab, after which bowl-shaped, varioliform, often depigmented scars appear. Simultaneous occurrence of all stages, resulting in a polymorphic appearance. Disfiguring mutilations of nose and auricles are possible. Furthermore, ophthalmological complications in the form of recurrent keratoconjunctivitis have been described (Mortazavi H et al. 2015).

In severe cases, fever reactions and disturbances of the general condition may occur.

Erythropoietic porphyria (detection of porphyrins in blood and urine; erythrocyte fluorescence)

Hepatic porphyria (detection of porphyrins in blood and urine)

Erythema (exsudativum) multiforme (exanthematous course; cocard form of efflorescences)

Actinic prurigo (rare light dermatosis, lichenified foci)

Bullous lupus erythematosus (rare, mainly on non-light-exposed areas, histo)

Bullous impetigo (large, flaccid blisters on a reddened background, with initially clear, then whitish-grey, creamy purulent contents)

Hydroa vacciniformia-like lymphoma (histological clarification with immature cell infiltrate)

In the acute stage, short-term glucocorticoids such as prednicarbate (e.g. Dermatop cream). Clarify eye involvement and treat if necessary.

It is important to protect the skin and eyes from light.

In severe cases systemic glucocorticoids (50-75 mg prednisone p.o.). Therapeutic approaches with chloroquine are described, but not convincing; also therapeutic approaches with pyridoxine (600mg/day).

An experiment with beta-carotene seems reasonable.

Avoid direct exposure to sunlight, UV-protective goggles. Consistent light protection for UVA and UVB.

Light-hardening with narrow-band UVB or PUVA before the sunny season begins makes sense.

A highly superfluous discussion about Bazin's term "Hydroa vacciniforme" has lasted since its first designation in 1862 (see synonyms below). The term "Hydroa" cannot be found in any linguistic lexicon, thus it is an erroneous neologism, to which already Joseph Jakob Plenck and the French Baron Jean Louis Alibert fell victim. Bazin originally called the disease "l'hydroa boulleux" (more consistent, if already axiologically wrong, would be "boulleuse" in the feminine spelling) and derived the word "hydroa" from water, which according to Rille is terminologically complete "nonsense". Hidroa comes from "hidros" - sweat. Thus the correct designation would be "Hidroa vacciniformia". But nothing lasts in medicine as surely as misnomers. So we sighingly follow the compulsion of the international majority and remain with a linguistic misstep (to be read in Johann Heinrich Rille 1938), which also leads straight to the dead end with regard to its etiopathogenetic interpretation.

1. Bazin E (1862) Lecons theoriques et cliniques sur les affections generiques de la peau. Paris Delabrage 1: 133-134
2. Di Lernia V et al (2013) Epstein-Barr virus and skin manifestations in childhood. Int J Dermatol 52:1177-1184

3. Guo N et al. (2019) Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases. Diagn Pathol 14:82.

4. Gupta G et al (2000) Hydroa vacciniforme: A clinical and follow-up study of 17 cases. J Am Acad Dermatol 42: 208-213
5. Haddad JM et al (2014) Hydroa vacciniforme: a rare photodermatosis. Dermatol Online J PubMed PMID: 25148284.
6. Hann SK et al (1991) Hydroa vacciniforme with unusually severe scar formation: diagnosis by repetitve UV-A phototesting. J Am Acad Dermatol 25: 401-403.
7. Höllhumer R (2018) Ocular manifestations of hydroa vacciniforme in a Black child. Eur J Ophthalmol 1120672118820518.
8. Iwatsuki K et al (1999) The association of latent Epstein-Barr virus infection with hydroa vacciniforme. Br J Dermatol 140: 715-721
9. Iwatsuki Ket al (2019) Hydroa vacciniforme: a distinctive form of Epstein-Barr virus-associated T-cell lymphoproliferative disorders. Eur J Dermatol 29: 21-28.
10. Jaschke E et al (1981) Hydroa vacciniforme - spectrum of action. UV tolerance after photochemotherapy. Dermatol 32: 350-353
11. Miranda MFR et al (2018) Hydroa vacciniforme-Like T-cell lymphoma: A Further Brazilian Case. Am J Dermatopathol 40:201-204
12. Mortazavi H et al (2015) Hydroa vacciniforme with eye involvement: report of two cases. Pediatr Dermatol 32:e39-41.
13. Pahlow Mose A et al (2014) Antiviral treatment of a boy with EBV-associated hydroa vacciniforme. BMJ Case Rep doi: 10.1136/bcr-2014-206488.
14. Rille JH (1938) Hydroa vacciniformis or vacciniforme? Archives of Dermatology and Syphilis 177: 122-124.
15. Stratigos AJ et al (2003) Spectrum of idiopathic photodermatoses in a Mediterranean country. Int J Dermatol 42: 449-454
16. Uchiyama M et al (2015) A case of recurrent facial herpes simplex mimicking hydroa vacciniforme. Int J Dermatol 54(3):e84-85