A Rare Association of Pyoderma Gangrenosum and Hyper-IgE Syndrome (Job-Buckley) in a Child: A case report

Background: Hyper-IgE syndrome (Job-Buckley syndrome) is a rare primary immunodeficiency caused by mutations in the STAT3 gene. It is characterized by recurrent severe infections, chronic eczema-like skin lesion, and markedly elevated serum IgE levels. Pyoderma gangrenosum (PG) is an inflammatory neutrophilic dermatosis that is rare in children.

Case presentation: We report the case of a 9-year-old boy, followed for dilated cardiomyopathy and diagnosed with Hyper-IgE syndrome since 2021, treated with sulfamethoxazole-trimethoprim. He presented with painful chronic ulcers on both lower limbs, evolving over the course of one year. Clinical examination revealed multiple deep, painful ulcers over the knees and ankles with violaceous raised borders and granulating bases, associated with erythematous, scaly papules resembling atopic dermatitis. Microbiological and laboratory investigations were negative. Histopathological analysis revealed a neutrophilic dermatosis. A diagnosis of pyoderma gangrenosum was made, and treatment with systemic corticosteroids (1 mg/kg/day) was initiated, resulting in favorable clinical evolution without relapse.

Conclusion: To our knowledge, this is the first reported case of pyoderma gangrenosum associated with Job-Buckley syndrome. This case highlights the importance of considering PG in immunocompromised children presenting with chronic ulcers.