Pyoderma Gangrenosum in a Patient with X-Linked Agammaglobulinemia.

Author: Qi TAN ¹ ; Fa liang REN ; Hua WANG
Author Information

1. Ministry of Education Key Laboratory of Child Development and Disorders, Key Laboratory of Pediatrics in Chongqing, Chongqing, China.
Publication Type:Case Report
Keywords: Agammaglobulinemia; Immunity; Pyoderma gangrenosum; X chromosome
MeSH: Agammaglobulinemia*; B-Lymphocytes; Child; Follow-Up Studies; Germ-Line Mutation; Humans; Immunity, Humoral; Immunoglobulins; Neutrophils; Prednisone; Protein-Tyrosine Kinases; Pyoderma Gangrenosum*; Pyoderma*; Skin Diseases; Tacrolimus; Ulcer; X Chromosome
From:Annals of Dermatology 2017;29(4):476-478
CountryRepublic of Korea
Language:English
Abstract: 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.