Pyoderma gangrenosum arising at the site of BCG immunization in a nine-month-old girl
Yuka Okura1, Yasuyoshi Hiramatsu1, Masaki Shimomura1
1Department of Pediatrics, KKR Sapporo Medical Center, Sapporo, Japan.
Insights
Pyoderma gangrenosum (PG) in infants is rare. This case highlights atypical skin reactions post-BCG immunization as a potential early sign, with similar cytokine profiles to adult PG.
Area of Science:
- Pediatrics
- Dermatology
- Immunology
Background:
- Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis.
- Infantile PG is exceptionally rare, with limited reported cases.
- Atypical skin reactions following Bacillus Calmette-Guérin (BCG) immunization can occur.
Purpose of the Study:
- To report a rare case of infantile pyoderma gangrenosum.
- To investigate the clinical presentation, cytokine profile, and genetic factors in infantile PG.
- To highlight the potential link between BCG immunization and infantile PG.
Main Methods:
- Case report of a nine-month-old girl with PG.
- Clinical observation of ulcer progression post-BCG immunization.
- Cytokine profiling and genetic analysis (MEFV gene).
- Treatment with intravenous methylprednisolone and oral prednisolone.
Main Results:
- The patient developed rapidly progressive ulcers at the BCG inoculation site and pustules on her extremities and neck.
- Elevated serum levels of IL-1β, IL-10, IL-17A, IL-6, and IL-18 were observed.
- A heterozygous R202Q variant in the MEFV gene was identified.
- Symptoms resolved with corticosteroid therapy and showed no relapse after discontinuation.
Conclusions:
- Atypical skin reactions after BCG immunization may herald infantile PG.
- The cytokine profile in infantile PG resembles that of adult PG, suggesting a common pathophysiology.
- Early recognition and treatment of infantile PG are crucial, even after BCG vaccination.
Abstract:
Pyoderma gangrenosum (PG) is an extremely rare disorder in children. We report a nine-month-old girl with PG who presented with high-grade fever and rapidly progressive ulcers at the site of a Bacillus Calmette-Guérin (BCG) inoculation 2 months after the immunization. Additional small pustules developed on her hand and posterior neck three months after the immunization and rapidly progressed. Cytokine profiling demonstrated elevated serum levels of interleukin (IL)-1β, IL-10, IL-17A, IL-6 and IL-18, which is similar to adult cases. Genetic analysis identified heterozygous R202Q variant of the MEFV gene. All of her systemic and local symptoms responded to intravenous methylprednisolone pulse therapy followed by prednisolone 2 mg/kg/day. There is no relapse of PG, to date, even after discontinuation of prednisolone. Atypical skin reactions after a BCG immunization could be an initial manifestation of infantile PG and need attention. Similarity of cytokine profile suggests common pathophysiology of infantile and adult PG.
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