What are the dermatologic manifestations of common variable immunodeficiency (CVID)?

Updated: Feb 23, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Alopecia areata and alopecia universalis may occur. [35] Any time a person presents with recurrent infections and alopecia, CVID should be considered in the differential diagnosis. In one case study from the Hacettepe University in Turkey, Kiliç et al [36] reported a 12-year-old boy who had recurrent respiratory tract infections and chronic diarrhea since age 2 years. At age 2.5 years, he had a bandlike localized loss of hair (alopecia areata), and 1 year later, he had lost all body hair. His sister had similar findings and died from a pulmonary infection at the age of 7 years. On further studies, the patient was found to have decreased serum levels of IgG, IgA, and IgM and an increased number of CD8 cells. In addition, a skin biopsy specimen showed a perifollicular infiltrate of mononuclear cells.

Cutaneous infections may be serious too, with necrotizing fasciitis described in one patient. [37]

In patients with CVID, both non-necrotizing granulomas (sarcoidlike) and necrotizing granulomas (tuberculoid) have been observed. [5, 6, 8, 7]

In one case report by Pujol et al, [38] histopathologic biopsy showed perivascular lymphoid infiltrates in the upper and mid dermis, with central necrosis and a palisading granuloma.

A syndrome similar to sarcoidosis can affect patients with CVID. [10] This syndrome is characterized by noninfectious cutaneous granulomas, with underlying visceral granulomas of the lungs, liver, spleen, or conjunctiva in most patients. These cutaneous granulomas are nonspecific in patients with CVID and can appear as a maculopapular rash; as infiltrated erythematous papules, plaques, excoriated papules, and ulcers; or as nodules with ulcerations. On histologic analysis, such granulomas are noncaseating and involve the dermis or subcutaneous fat. These nonsarcoidal, nontuberculoid asymptomatic cutaneous granulomas often appear as multiple, nontender subcutaneous nodules, predominantly juxta-articular, and the skin overlying the nodules was either normal or slightly erythematous. [11]

Lesions frequently appear on the face and extremities and are always sterile. They often resolve with treatment of the underlying disease.

Residual hyperpigmentation may be observed.

The increased incidence of malignancy in patients with CVID is well known. In 1992, Green and Moschella [39] reported the first known case of a patient with CVID who developed multiple squamous cell carcinomas. Many other cases have been published since then. Patients with CVID have an increased risk for actinic keratosis and squamous cell carcinoma. This risk is not surprising given the increased incidence of skin cancer with prolonged iatrogenic immunosuppression, as in transplant recipients.

Other dermatologic manifestations of CVID include atopic dermatitis, cutaneous vasculitis including polyarteritis nodosa, [9] and polymorphic light eruption. [40] However, these are not specific markers of CVID, and they are not indications for a workup for CVID unless other implicating factors (eg, recurrent infections) are present.

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