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


  • eng

Conference Location

  • Switzerland