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This is an EBV-positive cutaneous T-cell lymphoma (sometimes can be NK-cells) occurring in children and associated with sensitivity to insect bites and sun. Like other EBV-positive T-cell and NK-cell lymphomas, predisposition for this condition may be related to a defective cytotoxicimmune response to EBV.
(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010 see code 9702/3).
Hydroa vacciniforme-like lymphoma is one of two major types of Epstein-Barr (EBV) associated T-cell lymphoproliferative disorders that have been reported in the pediatric age group. (See also 9724/3: Systemic EBV-positive T-cell lymphoproliferative disease of childhood)
The neoplastic cells are generally small to medium in size without significant atypia. The infiltrates show extension from the epidermis to the subcutis, showing necrosis, angiocentricity and angioinvasion. The overlying epidermis is frequently ulcerated.
Systemic spread and mosquito bite allergy/hypersensitivity result in a much more aggressive clinical course.
This disease is treated with UV (ultra-violet light) or PUVA (psoralen plus ultraviolet light). UV and PUVA are both coded as "Other Treatment."