Evolution of IgG Subclass Deficiency into Common Variable Immunodeficiency in a Child with Pulmonary Interstitial Glycogenosis

Abstract

We present a 5-year-old boy who initially presented during infancy with pulmonary interstitial glycogenosis (PIG) and recurrent sinopulmonary infections. At 3.5 years old, IgG-2 subclass defi ciency associated with low IgA level and selective antibody defi ciency was diagnosed. Subsequently, the immune defi ciency evolved into common variable immunodefi ciency (CVID). This is the fi rst case reported of the association of PIG and CVID. In children with PIG who have recurrent infections, associated immunodefi ciency should be suspected.

Treatment of selective antibody deficiency with IVIG resulting in decreased frequency of streptococcal infection and improvement of guttate psoriasis

“…The implicated Dermatology Online Journal || Case Presentation mechanisms of immune modulation include Fcepsilon R blockade (ITP), antibody inhibition (myasthenia gravis), inhibition of immune activation (Kawasaki syndrome), complement inhibition (dermatomyositis), super-antigen inhibitor (toxic shock syndrome), and modulation of T cell activation (graft versus host disease), [26]. Replacement doses of IVIG were indicated for our patient because his age predicted a low likelihood that SAD would spontaneously resolve [29,30]. There are a few case reports supporting the efficacy of low and higherdose IVIG for psoriasis and psoriatic arthritis in adults [31][32][33][34].…”