Hydroa vacciniforme-like lymphoma

Orpha code: 364039OMIM code:

Definition

A very rare Epstein-Barr virus-associated lymphoproliferative disorder characterized by a chronic, recurrent, vesiculopapular rash, which subsequently ulcerates and scars, located mainly on sun-exposed areas and which is associated with systemic manifestations, such as fever, weight loss, asthenia, facial edema, arthralgia, lymphadenopathy, hepatosplenomegaly and/or increased liver enzymes. Hypersensitivity to mosquito bites has been associated and an increased risk of developing systemic lymphoma has been reported.

Disease data
Classification

Disease

Synonyms
Angiocentric cutaneous T-cell lymphoma of childhood
HVLL
HVLL
Hydroa-like cutaneous T-cell lymphoma
ORPHA code
364039
OMIM code
-
ICD10 code
C84.5
ICD11 code
-

No additional description.

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