Diagnostic Information

History of Present Illness Len is a 54-year-old white male who reports to your office in great distress. For the past 4 days he has been suffering from painful, widespread ulcerations affecting his mouth. He states is unable to eat and has been drinking nutritional protein shakes to get by. He woke up with mouth sores 4 days ago. He denies any constitutional signs and symptoms such as fever, malaise or fatigue. He denies having any rashes or ulcers affecting his skin or any other mucosal sites such as his eyes and genitalia. He does note a recent medication change for his osteoarthritis management from ibuprofen to naproxen about 2 weeks ago. Medical History - Adverse drug effects: none

- Medications: aripiprazole 5 mg qd, duloxetine 60 g qd, naproxen 500 mg bid, hydrochlorathiazide 25 mg qd

- Pertinent medical history: depression x 10 years, hypertension x 15 years, osteoarthritis x 20 years

- Pertinent family history: paternal - depression, non-fatal MI age 61; maternal - DM type 2; siblings - 2 healthy younger sisters

- Social history: denies tobacco use, 2-3 mixed drinks on weekends x 35 years, marijuana use while in college Clinical Findings Extraoral examination reveals normal TMJ function, no facial muscle tenderness, and no cervical lymphadenopathy. The labial mucosa exhibits a serohemorrhagic weep with crusting (Figures 1-2). Intraoral examination reveals wide-spread areas of bullae with mucosal sloughing affecting virtually all nonkeratinized tissues and palate (Figures 2-6). An incisional biopsy was performed on the right commissural area and the specimen was submitted for histologic assessment.

Figure 1. Weeping and crusting on the lip commissures.

Figure 2. Bullae ventral aspect of the tongue and weeping and crusting on the upper and lower lip vermilion.

Figure 3. Bullae on the dorsal aspect of the tongue.

Figure 4. Multiple bullae affecting the hard palate.

Figure 5. Bullous eruption on the right side commissure and buccal mucosa.

Figure 6. Bullous eruption on the left side commissure and buccal mucosa.

Histopathologic Findings The biopsy shows inflamed oral mucosa consisting of reactive stratified squamous surface epithelium with subjacent fibrovascular connective tissue. The interface to perivascular inflammatory infiltrate consists predominantly of lymphocytes with admixed neutrophils and eosinophils. There is superficial stromal edema with areas of subepithelial and intraepithelial vesiculation. The basal portion of the epithelium displays scattered necrotic keratinocytes.

Figure 7. Low-power histologic image showing a dense superficial mixed interface inflammatory infiltrate with associated sub and intraepithelial vesicle formation.

Figure 8. High-power histologic image showing an interface lymphocytic inflammatory infiltrate with basal epithelial disruption, exocytosis, and focal necrotic keratinocytes.