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September 1998

Chronic Plaques in a Patient With Ataxia Telangiectasia

Arch Dermatol. 1998;134(9):1145-1150. doi:

The cheek biopsy specimen displayed a nearly diffuse infiltrate composed of mononuclear cells; nodular aggregations of macrophages, including multinucleate forms, were also observed. In the background of the granulomas, there were lymphocytes of variable size.

A second biopsy was performed (findings not shown), and histological examination of the specimen again revealed a diffuse infiltrate of mononuclear cells. In contrast to the granulomatous pattern observed in the first biopsy specimen, lymphocytes vastly predominated in the second biopsy specimen, although small clusters of macrophages were still identifiable. The lymphocytes were polymorphous, including both small cleaved and immunoblastic forms. CD3 immunostaining confirmed that most of the lymphocytes were of T-cell lineage. The patient refused to undergo a third biopsy, to evaluate for T-cell receptor gene rearrangements. The lesions were treated with various potent topical steroids and topical and systemic antibiotics, without significant benefit. They have persisted and have only minimally enlarged.