[Shifting cellulitis in a patient with X-linked hypogammaglobulinemia]

Abstract

Background: In cases of immunodeficiency, a systemic infection may be revealed by atypical symptoms, particularly those involving the skin.

Patients and methods: The present case describes a 19-year-old male with X-linked hypogammaglobulinemia, or Bruton agammaglobulinemia, treated with intravenous immunoglobulin G antibodies. Over a 6-week period, the patient developed recurrent plaques in both legs, first on one and then on the other, without fever. Blood cultures were repeated and the fifth pair proved positive for Campylobacter jejuni. An abdominal scan showed appendicitis without intestinal signs. The patient was treated with azithromycin for 2 weeks, which resulted in full recovery from the skin lesions.

Discussion: Campylobacter bacteremia infections are severe and carry a 15% mortality rate at 30 days. The majority of affected patients present humoral immunodeficiency. The literature contains reports of 10 patients with C. jejuni-associated cellulitis, of whom 6 presented hypogammaglobulinemia. We postulate that the cutaneous manifestations were caused by septic metastases. The immunoglobulin replacement therapy mainly comprised IgG antibodies; IgA and IgM antibodies appear to play a key role in the response to C. jejuni infection, which could explain the susceptibility observed. The American guidelines recommend blood and skin cultures in patients with cellular immune defects. We suggest that this recommendation be extended to patients with humoral immunodeficiency.

Keywords: Bacteremia; Bactériémie; Bruton; Campylobacter; Cellulitis; Dermo-hypodermite bactérienne; Erysipela; Hypogammaglobulinemia; Hypogammaglobulinémie; Érysipèle.