A patient with elderly-onset atypical hydroa vacciniforme with an indolent clinical course.
Br J Dermatol
; 173(3): 801-5, 2015 Sep.
Article
in En
| MEDLINE
| ID: mdl-25965563
ABSTRACT
Hydroa vacciniforme (HV) is a rare photodermatosis that mainly affects children and manifests as vesiculopapular eruptions in sun-exposed areas without systemic symptoms. HV-like lymphoma (HVLL) is one of the Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (LPD) of childhood. Its diagnosis is based on monoclonal T-cell proliferation; however, its degree of malignancy is controversial owing to its variable prognosis. Elderly-onset cases of these diseases are extremely rare, and the clinical features remain unknown. It has been shown that late onset is closely associated with a severe phenotype in EBV-associated LPD including atypical HV. Here we describe a case of elderly-onset atypical HV accompanied by T-cell monoclonality, but with a very indolent clinical course. Our patient indicates a possible case with elderly-onset atypical HV manifesting a favourable course, and that T-cell monoclonality and age of onset cannot always predict the disease severity, and highlights the difficulty of prognosis prediction in elderly-onset atypical HV.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Facial Dermatoses
/
Hydroa Vacciniforme
Type of study:
Prognostic_studies
Limits:
Aged
/
Female
/
Humans
Language:
En
Journal:
Br J Dermatol
Year:
2015
Document type:
Article
Affiliation country: