Pyostomatitis vegetans and Crohn's disease. A specific association of 2 diseases

HISTORY AND CLINICAL FINDINGS: A 27-year-old man was referred to the dermatological out-patient clinic because of inflammatory changes in the oral mucosa of unknown cause. 5 months earlier he had been diagnosed as having Crohn's disease of the terminal ileum. On both sides of the buccal mucosa there were rough erythematous vegetations and disseminated miliary abscesses, which extended to the labial gingiva and the soft palate. Further physical examination was unremarkable. INVESTIGATIONS: Several inflammatory parameters were increased: C-reactive protein 100 mg/l, erythrocyte sedimentation rate 55/88 mm, eosinophilic cationic protein 35.8 ng/ml (normal range 2.3-16 ng/ml). White cell count was normal (7,25/nl), with a lymphocytopenia of 11.9%. There was no eosinophilia. Haemoglobin was reduced to 11.6 g/dl and the platelets raised to 526/nl. Smears of the oral mucosa showed no fungal, viral or bacterial infection. Biopsy revealed leucocytic microabscesses in the epithelium, granulation tissue and flat ulcerations with adjoining superficial necrotic zones. DIAGNOSIS, TREATMENT AND COURSE: The clinical and histological picture as well as the association with Crohn's disease (CD) suggested pyostomatitis vegetans (PV). The PV was treated with disinfectant mouth washes which improved the subjective findings. Budesonide was given for CD. CONCLUSION: PV is a rare and usually isolated condition, but it can also occur in association with a chronic gastrointestinal disease such as ulcerative colitis and Crohn's disease. The diagnosis of PV indicates a thorough gastroenterological investigation.