Abstract
We report a case of atopic dermatitis-like rash in a patient with IgA deficiency accompanied by IgG2/IgG4 deficiency and low IgE levels. The patient was a 32-year-old female with primary immunodeficiency disease (deficiency of IgG2, IgG4, as well as IgA) from early childhood. Due to frequent recurrences of bacterial pneumonia, she began to receive Gammagard®, a low IgA immune globulin intravenous (human) (IGIV) treatment on a regular basis at 19 years of age. At 31 years of age, an itchy rash erupted all over her body, and she was referred to our clinic after her local dermatologist was unable to alleviate her symptoms. The itchy rash covering her whole body was accompanied by scaling erythematous macule, erythematous papule, and pigment deposition. The rash on her back and elbows was lichenified, and her four limbs had encrustations as evidence of rubbing. We suspected atopic dermatitis, but her IgE levels were low. Because no improvement was made following external steroid application and internal allergy drug treatment, we also had her take internal prednisolone (PSL), but her rash was refractory. She mentioned that the rash was worse during and immediately after IGIV treatment, so we lengthened the time between IGIV treatments. However, this only caused the dermatitis symptoms to worsen prior to her IGIV treatments. We surmised that PSL administration and extending the length of time between IGIV treatments cultivated a more compromised immune system, which may have yielded the worsening dermatitis symptoms. Intradermal test for IGIV treatment was negative, so we reestablished the original 3-week interval for IGIV administration, and reduced PSL to 5mg/day before terminating PSL administration. With the hypothesis that the increase in itching during and immediately after IGIV treatment was caused by the psychological stress accompanying intravenous treatment, she reduced her stress level by taking a leave of absence from work, which led to further alleviation of her rash. In this case, the 31-year-old patient had started a new job several months prior to the eruption of the rash. She noted that she had a great deal of stress at work, so adjustment of her living environment to reduce stress levels may have led to improvement of her symptoms. Skin Research, 10: 41-45, 2011
