Ann Dermatol.  2017 Aug;29(4):476-478. 10.5021/ad.2017.29.4.476.

Pyoderma Gangrenosum in a Patient with X-Linked Agammaglobulinemia

• Tan Q
• Ren FL
• Wang H

• KMID: 2388947
• DOI: http://doi.org/10.5021/ad.2017.29.4.476

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.

Keyword

Agammaglobulinemia; Immunity; Pyoderma gangrenosum; X chromosome

MeSH Terms

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
Immunoglobulins
Prednisone
Protein-Tyrosine Kinases
Tacrolimus

Figure

• 

  Fig. 1 (A) The left shin ulcer with surrounding irregular, undermined, violaceous margins and induration. (B) Nearly healed leg ulcer 2 weeks after treatment with intravenous immunoglobulin in conjunction with prednisone and topical application of 0.03% tacrolimus ointment.

• 

  Fig. 2 A mixed neutrophilc and lymphocytic infiltrate and hemorrhage in the upper and lower dermis (H&E; A: ×40, B: ×200).

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