Nodular Skin Lesions in a Patient With X-Linked Agammaglobulinemia.

Abstract

A 28-year-old man with X-linked agammaglobulinemia (XLA) on subcutaneous immunoglobulin replacement therapy presented with a 2-year history of scattered nodular skin lesions. Lesions were occasionally pruritic, mildly painful, and were worse during the summer when he was more active with his carpentry. He had no fevers, weight loss, or fatigue. Physical examination was notable for some scattered pink dermal papules and larger ulceronecrotic nodules with hemorrhagic crust on the bilateral arms, forearms, and lower back (Figure 1). His serum immunoglobulin (Ig) G level was 990 mg/dL (on IgG replacement). Serum IgA and IgM levels were undetectable. Analysis of lymphocyte subsets revealed absent CD19+ B cells and a normal CD3+ T-cell count. Shave biopsy of an ulceronecrotic plaque of the left forearm revealed irregular epidermal acanthosis with central ulceration (Figure 2A). In the dermis, there were multiple clusters of basophilic granules surrounded by a thin rim of eosinophilic material (Figure 2B). Additionally, there was a dermal mixed inflammatory infiltrate, primarily comprised of neutrophils and eosinophils.