Pyoderma Gangrenosum in a Patient with X-Linked Agammaglobulinemia
Annals of Dermatology
; : 476-478, 2017.
Article
in En
| WPRIM
| ID: wpr-86511
Responsible library:
WPRO
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.
Key words
Full text:
1
Index:
WPRIM
Main subject:
Pyoderma
/
Skin Diseases
/
Ulcer
/
X Chromosome
/
Protein-Tyrosine Kinases
/
Immunoglobulins
/
Prednisone
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B-Lymphocytes
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Follow-Up Studies
/
Tacrolimus
Type of study:
Observational_studies
/
Prognostic_studies
Limits:
Child
/
Humans
Language:
En
Journal:
Annals of Dermatology
Year:
2017
Type:
Article