A young woman with diffuse skin lesions.

Figure 1. Computed tomography of the chest demonstrates long, dilated bronchi (short arrow), as well as the cystic " signet ring " lesions (long arrow), which are consistent with bronchiectasis. Diagnosis: common variable immunodeficiency (CVID) with epidermodysplasia verruciformis-like lesions. Epidermodysplasia verruciformis was classically described as a genetic condition involving diffuse warty skin lesions due to human pampillomavirus (HPV), such as those seen in Figure 1. However, it has also been associated with both acquired immune defects, such as human immunodeficiency virus infection and organ transplantation, and primary immunodefi-ciencies, such as CVID, X-linked hyper-immunoglobulin (Ig) M immunodeficiency syndrome, and Wilskott-Aldrich syndrome , among others [1–4]. Interestingly, it has been described in both immunodeficiencies that involve T cells and those that involve B cells. The presence of epidermodysplasia verrucifor-mis–like lesions should initiate a search for an underlying immune defect. In this patient, thoracic high-resolution computed tomog-raphy (HRCT) revealed diffuse lymphadenopathy, consolidation in the right middle lobe, and lingula with bilateral bron-chiectasis (Figure 1). An abdominal CT demonstrated mild hepatomegaly and retroperitoneal lymphadenopathy, and a sinus CT revealed pansinusitis. Nitro blue tetrazolium test and complement levels (CH50, C3, and C4) were normal. Immu-noglobulin quantitative study revealed the following results: Flow cy-tometric analysis of peripheral blood mononuclear cells demonstrated 80% CD3 + cells, 23% CD4 + cells, 58% CD8 + cells, and 6% CD19 + cells, with a CD4 + /CD8 + cell ratio of 0.4. The blood group was O + , and blood isoagglutinins (anti-A and anti-B) were not detectable. Polymerase chain reaction of the warty lesions had results that were positive for HPV type 33. A diagnosis of CVID was confirmed by the patient's clinical history, demonstration of low levels of all immunoglobulin classes, absence of isoagglutinins, decreased CD4 + /CD8 + cell ratio, generalized lymphadenopathy, hepatomegaly, thrombo-cytopenia, bilateral bronchiectasis, and pansinusitis. CVID is a heterogeneous immune disorder with a prevalence of 1 case per 25,000 individuals that is distributed in a bimodal pattern of age (1–5 years of age and 18–25 years of age). Patients have markedly reduced levels of IgG and IgA or IgM with impaired antibody responses despite the presence of B cells, which is attributable to the defective B cell differentiation and immunoglobulin secretion. As a result, patients experience recurrent bacterial pneumonia and bronchiectasis, which are seen in 30%–50% of patients and are an important cause of morbidity and mortality [5–7]. Sinusitis—acute and chronic—is very common [8]. …