Pyoderma Gangrenosum in a Patient With Dubowitz Syndrome: a New Comorbidity?

Abstract

Introduction. Dubowitz syndrome is a rare genetic disease with only a few cases reported in the literature. It is characterized by growth retardation, microcephaly, facial dysmorphism and higher risk of developing cancer and cardiomyopathies. PG is an autoinflammatory disorder that causes painful ulcers to develop on the skin and has not been previously associated with Dubowitz syndrome. Case Presentation. The authors report the case of a 50-year-old female with Dubowitz syndrome who developed painful ulcerative lesions. An incisional biopsy was performed to rule out other diagnoses, and a subsequent clinical diagnosis of PG was made. The patient was treated with specialized wound dressings and oral glucocorticoids. The clinical picture improved consistently after 7 weeks of therapy. Conclusions. This case report, to the authors’ knowledge, is the first to suggest a possible association between Dubowitz syndrome and PG and also to indicate an effective treatment.