Autoimmune lymphoproliferative syndrome

A rare, inherited disorder characterized by non-malignant lymphoproliferation, multilineage cytopenias, and a lifelong increased risk of Hodgkin's and non-Hodgkin's lymphoma.

Angioedema

Rapid swelling (edema) of the dermis, subcutaneous tissue, mucosa and submucosal tissues of the skin of the face, normally around the mouth, and the mucosa of the mouth and/or throat, as well as the tongue during a period of minutes to several hours. The swelling can also occur elsewhere, typically in the hands. Angioedema is similar to urticaria, but the swelling is subcutaneous rather than on the epidermis.

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PMID (PMCID)
15942224	FEMALE
	Cutaneous manifestations as presenting sign of autoimmune lymphoproliferative syndrome in childhood.
	Auricchio L, Vitiello L, Adriani M, Ferri P, Chiocchetti A, Pettinato G, Racioppi L, Maiuri L, Dianzani U, Pignata C. Dermatology. 2005;210(4):336-40.
	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.