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KMID : 0032220170290040476
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Annals of Dermatology
2017 Volume.29 No. 4 p.476 ~ p.478
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Pyoderma Gangrenosum in a Patient with X-Linked Agammaglobulinemia
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Tan Qi
Ren Fa Liang
Wang Hua
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Abstract
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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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KEYWORD
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Agammaglobulinemia, Immunity, Pyoderma gangrenosum, X chromosome
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