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This case report presents a 13-year-old boy referred to the Department of Paediatric Dentistry, Women's and Children's Hospital, Adelaide, South Australia, Australia, with a 5-week history of severe oral ulcerations and significant weight loss of unknown origin. The diagnosis of pemphigus vulgaris was made after histologic and immunofluorescent examination of an intraoral deep incisional biopsy, with eosinophilic esophagitis also diagnosed during the initial upper gastrointestinal endoscopy. The association between pemphigus vulgaris and eosinophilic esophagitis in this case, although previously unreported, is explicable on the basis of dysregulation of desmoglein 1 (DSG1). This case report identifies a new clinical association that could help clinicians identify further such cases and provides insight into the pathogenesis of both conditions.

The objective of this study was to describe the clinical features of Sweet syndrome in children. Our study population consisted of seven children diagnosed with Sweet syndrome over a 22-year period. Age, sex, appearance and location of lesions, associated signs and symptoms, past medical history, pathology, and subsequent disease course were documented for each patient. Fever and typical lesions were reported in most of patients in our study. The majority of patients presented with less-typical findings, such as pustules, vesicles, bullae, oral ulcerations, atrophic scars, and evidence of pathergy. Of the seven children in our study, four were found to have a preceding nonspecific upper respiratory or gastrointestinal infection, and two were diagnosed with an underlying hematologic malignancy. Our results suggest that atypical lesions are relatively common in children with Sweet syndrome and that underlying malignancy is associated with a minority of cases of pediatric Sweet syndrome.

Behcet disease is a complex, multisystem disease characterized by recurrent oral and genital ulcerations. It rarely occurs in infants or children. Neonatal Behcet disease has been reported in infants whose ulcers resolve at or before 9 weeks of age. Few cases of neonatal Behcet disease persisting into childhood have previously been reported. We report the case of a 1-month-old infant who presented with severe recurrent genital ulcerations and at 6 months developed recurrent oral ulcerations. Her orogenital ulcerations continue to recur. Human leukocyte antigen testing revealed HLA-B51 and B44 positivity. This is a case of pediatric Behcet disease in the neonatal period. Behcet disease should be considered in the differential diagnosis of recurrent genital and oral ulcerations in infants and children.

A young infant with fever and oral ulcerations is presented herein. Extensive infectious, nutritional, immunodeficiency and autoimmune work up was performed. Pertinent maternal history suggested the potential diagnosis of neonatal Behcet’s, and treatment for this condition resulted in rapid and sustained clinical improvement.