Over the years many amazing cases have been presented during the PEMPix presentation at the American Academy of Pediatrics’ annual national Conference and Exhibition during the Section on Emergency Medicine Sessions. PEMPix Classic is a new featured series on PEMBlog that will highlight some of those classic cases.

The Case

An 10 year old boy presents with mouth sores that have been present, and worsening for two to three weeks. They have become painful and he reports difficulty swallowing his saliva and now fluids. It started on his lips and now involves the tongue and throat.

He denies fevers, or other recent illnesses. He reports multiple recurrent episodes of mouth sores over the past one to two years, but none as extensive as today. He has been using an over the counter topical antibiotic to no avail. He denies any history of genital lesions or other skin lesions.

family history is negative for similar problems. There are no noteworthy relatives with autoimmune disease, Rheumatologic problems, or connective tissue disease.

What is the diagnosis?

A. Behçet Syndrome
B. Crohn’s Disease
C. Stevens-Johnson Syndrome
D. Systemic Lupus Erythematosus
E. Recurrent Herpes Labialis

A. Behçet Syndrome

The patient also reported prior scrotal ulcers and scarring. he was eventually found to have posterior uveitis as well. BehçetSyndrome is rare in children and most often presents with aphthous stomatitis. It is the only major vasculitis affecting arteries and veins. The diagnosis is supported in patients with recurrent aphthous stomatitis, genital ulcers, and posterior uveitis – the latter of which obviously needs an Ophthalmologist.
In lupus, oral lesions are on the hard palate and painless. Recurrent herpes simplex is confined to the lips, though the initial presentation can be the entire mouth. Stevens-Johnson patients are toxic, with rapid progression (unlike our patient). Crohn’s disease sees extra intestinal findings  almost always occurring in conjunction with colitis.


Yazici H, Fresko I, Yurdakul S. Behçet’s syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol 2007; 3:148.
Calamia KT, Wilson FC, Icen M, et al. Epidemiology and clinical characteristics of Behçet’s disease in the US: a population-based study. Arthritis Rheum 2009; 61:600.