PLAID

By Gary M. White, MD


A new syndrome, PLAID (PLCG2-associated antibody deficiency and immune dysregulation) is characterized by cold urticaria, autoimmunity, atopy and humoral immune deficiency that causes recurrent sinopulmonary infections [JAMA Derm 2015;151;627].

Clinical

The cold urticaria associated with PLAID develops within the first year of life--even the first few days. Evaporative cooling or immersion in cold water leads to blotchy pruritic erythematous lesions. Syncopal episodes may occur with prolonged immersion in cold water. Granulomatous lesions associated with immunodeficiency are associated.

Nasal tip lesions are common. Spontaneous ulceration approximately three days after birth is typical. Some have progressive nasal involvement leading to progressive localized nasal destruction.

Firm, red/brown plaques may occur widely and represent granulomatous lesions. For pictures of cutaneous granulomatous lesions associated with immunodeficiency, see here.

References

JAMA Derm 2015;151;627.

JAMA Derm 2015;151;627.

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