Cutaneous manifestations as presenting sign of autoimmune lymphoproliferative syndrome in childhood.
Journal Article (Journal Article)
Autoimmune lymphoproliferative syndrome is a disorder due to a defect of lymphocyte apoptosis, whose clinical manifestations consist of hyperplasia of lymphoid tissues and autoimmune diseases. We report on a 26-month-old child who presented with frequent eruptions of weals and angioedema without any apparent triggering factor, who subsequently developed an erythematopapular rash with a histological pattern of a lymphoplasmacellular infiltrate. Familial anamnesis revealed a history of lymphoadenomegaly and massive spleen and liver enlargement in her sister. Functional and molecular analysis led to a diagnosis of type 1a autoimmune lymphoproliferative syndrome. Immunophenotyping of the cutaneous lesion revealed the presence of an inflammatory infiltrate with a considerably high number of Langerhans cells. Cutaneous features such as urticaria, angioedema and vasculitis in children with a personal and familial history of hyperplasia of lymphoid tissues may be a presenting sign of a systemic disease, such as autoimmune lymphoproliferative syndrome.
Full Text
Duke Authors
Cited Authors
- Auricchio, L; Vitiello, L; Adriani, M; Ferri, P; Chiocchetti, A; Pettinato, G; Racioppi, L; Maiuri, L; Dianzani, U; Pignata, C
Published Date
- 2005
Published In
Volume / Issue
- 210 / 4
Start / End Page
- 336 - 340
PubMed ID
- 15942224
International Standard Serial Number (ISSN)
- 1018-8665
Digital Object Identifier (DOI)
- 10.1159/000084762
Language
- eng
Conference Location
- Switzerland