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hydroa vacciniforme

Etiology: - photodermatosis of unknown origin - Ultraviolet radiation & Epstein Barr virus are 2 potential triggers Epidemiology: - rare - bimodal incidence in early childhood (1-7 years) & adolescence (12-16 years) Pathology: - intraepidermal reticular degeneration & cellular necrosis - spongiosis, focal epidermal necrosis, & intraepidermal lymphocytes - skin biopsy: - immunohistochemistry - EBV latent membrane protein-1 - EB nuclear antigen - Eptein Barr virus DNA Clinical manifestations: - recurrent vesicles on sun-exposed skin that heal with vacciniform or varioliform scarring - vesicles occur within minutes to hours of sun exposure - resolution of the acute phase & progression to scarring occur over ~6-8 weeks [3] - chronic remitting & relapsing course - characteristic seasonal flare (early summer) - cases remit spontaneously by late adolescence with persistent scarring [3] * image: [3] Complications: - transformation lymphoma & secondary hemophagocytic lymphohistiocytosis - extracutaneous involvement is infrequent - cartilage destruction - ocular involvement: photophobia & keratoconjunctivitis Differential diagnosis: - erythropoietic protoporphyria - polymorphic light eruptions - bullous lupus erythematosus - porphyrea cutanea tarda - lymphoma - may present with tissue necrosis, involvement of photoprotected sites, presence of facial edema, extracutaneous involvement (liver, lung) [3] Management: - avoid sun exposure - use of high SPF suncreen with UV-A blocking - topical corticosteroids [3] - phototherapy - low-dose, narrow-band UV-B (TL-01) phototherapy, either daily or 3 times a week in the spring - psoralen photochemotherapy (PUVA) prophylactically in older patients may be of benefit - antimalarials have been used [3]

General

skin disease (dermatologic disorder, dermatopathy, dermatosis)

References

  1. Sebastain QL and Del Rosario R eMedicine: Hydroa Vacciniforme http://emedicine.medscape.com/article/1119445-overview
  2. Balestri R and Neri I Hydroa vacciniforme CMAJ. 2010 Nov 23;182(17):E796. Epub 2010 Sep 13. PMID: 20837686
  3. Saraswat N, Tripathy DM, Kumar S. Hydroa Vacciniforme. JAMA Dermatol. 2023;159(9):997 PMID: 37436742 https://jamanetwork.com/journals/jamadermatology/fullarticle/2807317