Clinical Case Detail

A 60 year old male presents with a chief complaint of "mouth lesions and sores." The patient experienced 6 months of painful mouth sores on the palate, tongue, lips, gums and inner cheeks.  He lost 20 lbs. in 2 months due to the mouth pain/sores. After otolaryngology evaluation and management for GERD and for a "virus," the patient had a biopsy which read, "non-specific acute and chronic inflammation."  One week ago, he reports having had "tiny, line-like ulcers on the soft palate" which are now healed, but appeared identical to the current lesion on the maxillary labial mucosa (see clinical photo).

The past medical history includes psoriasis vs. eczema (elbows), seasonal/environmental allergies, panic disorder, hypercholesterolemia, DVT with pulmonary embolus, s/p tonsillectomy (age 10), s/p mononucleosis/hepatitis (during college) and recurrent aphthous stomatitis episodes at times of increased stress.  Medications include Paxil and Lipitor.  There are no known drug allergies.  The social history includes casual intake of alcohol (sake & wine, 2-4 units per week), marathon runner (30-50 miles/week), and increased personal stress over the past 6 months. A thorough review of systems is significant for mouth sores/pain and hoarseness.  He denies nausea, emesis and diarrhea. 

• Pyostomatitis Vegetans
• Crohn's Disease
• Orofacial Granulomatosis
  

•  Etiology � unknown
• Cultures for pathogenic bacteria, viruses & fungi are negative
• IBD/ulcerative colitis usually precedes oral findings; referral to gastroenterology
• Dx: clinical findings, association with IBD, peripheral eosinophilia, negative culture results, histology
• Differential: pemphigus vegetans (variant of pemphigus vulgaris)

• Topical corticosteroids
• Systemic steroids
• Dapsone
• Azathioprine
  

• Large eosinophilic abscesses � suggestive of PSV
• 90% of PSV cases show elevated eosinophil count**
• Direct & indirect immunofluorescence (DIF & IIF) �usually� show negative results with PSV, some cases show nonspecific, weak deposition of C3, IgG & IgA
• Not all cases of PSV in literature had DIF/IIF
• DIF/IIF can be helpful, but possible false + & false