Pyoderma Gangrenosum in a Patient with X-Linked Agammaglobulinemia = Pyoderma Gangrenosum in a Patient with X-Linked Agammaglobulinemia

다국어 초록 (Multilingual Abstract)

X-linked agammaglobulinemia (XLA) is a primary im-munodeficiency disorder caused by germline mutations of B-cell tyrosine kinase (BTK) gene. It is characterized by de-creased serum immunoglobulins levels and circulating ma-ture B cells. This defect in humoral immunity leads to in-creased 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 pa-tient received intravenous immunoglobulin treatment in conjunction with prednisone and topical application of 0.03% tacrolimus ointment and the ulcer was almost com-pletely 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. (Ann Dermatol 29(4) 476∼478, 2017)