“…It was also clear that it was possible to reduce other treatments when the lesions had healed and to discontinue hdIGIV. Improvement of PG in the setting of hypogammaglobulinemia has also been described with replacement IGIV [44,45]. The mechanism of action is not clear; however, it is possible that effects of hdIGIV on the local cytokine environment (e.g.…”
“…PG is a neutrophilic dermatosis that occurs both as a primary disorder as well as secondary to an underlying disease. To date, only five previous reports have documented the association of PG with XLA56789. We report herein a case of PG in a patient with XLA.…”
X-linked agammaglobulinemia (XLA) is a primary immunodeficiency disorder caused by germline mutations of B-cell tyrosine kinase (BTK) gene. It is characterized by decreased serum immunoglobulins levels and circulating mature B cells. This defect in humoral immunity leads to increased susceptibility to infection. Pyoderma gangrenosum (PG) is an uncommon, ulcerating, neutrophilic dermatosis. Here we report PG in an 8-year-old patient with XLA. The patient received intravenous immunoglobulin treatment in conjunction with prednisone and topical application of 0.03% tacrolimus ointment and the ulcer was almost completely healed in the 2 weeks of follow-up. The coexistence has been rarely reported. XLA may be a possible cofactor in the pathogenesis of PG.
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