Cutaneous manifestations as presenting sign of autoimmune lymphoproliferative syndrome in childhood

Luigi Auricchio, Laura Vitiello, Marsilio Adriani, Pasqualina Ferri, Annalisa Chiocchetti, Guido Pettinato, Luigi Racioppi, Luigi Maiuri, Umberto Dianzani, Claudio Pignata

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)336-340
Number of pages5
Issue number4
Publication statusPublished - 2005


  • Autoimmune lymphoproliferative syndrome
  • Erythematopapular rash
  • Lymphoplasmacellular infiltrate

ASJC Scopus subject areas

  • Dermatology


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