Pyoderma Vegetans: A Case Report in a Child Suspected to Primary Immunodeficiency and Review of the Literature

Document Type: Case Report(s)

Authors

1 Department of Immunology and Allergy, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

2 Departement of Pathology, Shohada-e-Tajrish Hospital, Shahid Beheshti University of Medical Science, Tehran, Iran

3 Skin Research Center, Shohada-e-Tajrish Hospital, Shahid Beheshti University of Medical Science, Tehran, Iran

4 Department of Pediatric Infectious Diseases, Mofid Children Hospital, Pediatric Infections Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran

5 Pediatric Congenital Hematologic Disorders Research Center, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

Abstract

Pyoderma vegetans (PV) is a rare inflammatory disorder characterized by vegetating pustules and plaques affecting the skin and mucosal membranes. It is believed that this entity is mostly associated with inflammatory bowel disease (IBD), chronic malnutrition, human immunodeficiency virus (HIV), malignancies, and other immunocompromised states. Pyoderma vegetans occurs more commonly in young and middle-aged adults. There is no sex predilection for this entity. The lesions could heal spontaneously, but usually recur and become chronic.Our patient was an 11-year-old girl suspected to have primary combined immunodeficiency complicated by chronic recurrent vegetating pustular lesions on the face and postauricular area since one year of age. The histological features of the lesions were consistent with pyoderma vegetans. This is the first case of PV beginning from early infancy in the setting of primary immunodeficiency and in an unusual location.