In a 60‑year‑old woman presenting with burning tongue, oral ulcerations, dry eyes, and new‑onset urticaria, what is the most likely diagnosis and the recommended evaluation and treatment?

Sjögren Syndrome: Most Likely Diagnosis

This 60-year-old woman presenting with burning tongue, oral ulcerations, dry eyes, and urticaria most likely has Sjögren syndrome, and she requires immediate serologic testing (anti-SSA/Ro, anti-SSB/La antibodies, ANA, RF), salivary gland assessment, and ophthalmologic evaluation with Schirmer testing. 1

Clinical Reasoning and Diagnostic Approach

The constellation of burning tongue (oral dysesthesia), dry eyes (keratoconjunctivitis sicca), and mouth sores forms the classic triad of Sjögren syndrome, a systemic autoimmune disease that predominantly affects middle-aged and elderly women. 1 The addition of urticaria suggests broader autoimmune activation, as chronic spontaneous urticaria can coexist with autoimmune conditions. 2

Key Distinguishing Features

• Burning tongue without visible lesions is characteristic of oral dysesthesia, which occurs in autoimmune conditions and is distinct from simple traumatic or infectious ulceration. 2, 3
• Dry eyes combined with oral symptoms strongly suggests Sjögren syndrome rather than isolated burning mouth syndrome or recurrent aphthous stomatitis. 1
• Age and sex demographics align perfectly—Sjögren syndrome affects women 7 times more frequently than men and typically presents in the 50-70 age range. 1, 3

Immediate Diagnostic Workup

Essential Laboratory Testing (Order Immediately)

• Serologic markers: Anti-SSA/Ro antibodies, anti-SSB/La antibodies, antinuclear antibodies (ANA), rheumatoid factor (RF), and complete blood count with differential. 1
• IgG anti-thyroid peroxidase (anti-TPO) and total IgE levels to assess for autoimmune urticaria, as elevated IgG-anti-TPO with low total IgE suggests autoimmune-mediated chronic spontaneous urticaria. 2
• Inflammatory markers: ESR and C-reactive protein to assess systemic inflammation. 2
• Metabolic screening: Fasting blood glucose to exclude diabetes (which predisposes to oral infections and can mimic some symptoms). 2

Specialized Testing

• Schirmer test for objective measurement of tear production (ophthalmology referral). 1
• Salivary flow rate measurement and consider minor salivary gland biopsy if serologies are negative but clinical suspicion remains high. 1
• Oral examination with biopsy if ulcers persist beyond 2 weeks despite symptomatic treatment, to exclude malignancy, pemphigus, or other autoimmune bullous diseases. 2, 4

Management Strategy

For Oral Symptoms (Burning Tongue and Ulcers)

• Topical high-potency corticosteroids (clobetasol 0.05% gel or dental paste) applied to ulcerated areas. 2
• Gabapentin starting at 300 mg daily, titrating up to 900-1800 mg/day in divided doses for burning sensation (oral dysesthesia). 2, 3
• Viscous lidocaine 2% for immediate pain relief before meals. 2
• Dietary modifications: Avoid crunchy, spicy, acidic, or hot foods. 2

For Dry Mouth (Sicca Syndrome)

• Immediate measures: Frequent water sips, saliva substitutes, sugar-free gum or lozenges. 2
• Systemic sialagogues: Cevimeline 30 mg three times daily or pilocarpine 5 mg four times daily if topical measures insufficient. 2
• Dental referral urgently—untreated severe dry mouth leads to rapid dental caries and tooth loss. 2

For Urticaria

• Second-generation H1-antihistamines at standard doses initially (e.g., cetirizine 10 mg daily, loratadine 10 mg daily). 2
• If inadequate response after 2-4 weeks: Updose antihistamines up to 4 times the standard dose. 2
• If still inadequate after 2-4 weeks of updosed antihistamines: Omalizumab 300 mg subcutaneously every 4 weeks (can increase to 600 mg every 2 weeks if needed). 2

Rheumatology Referral

• Urgent rheumatology consultation for comprehensive Sjögren syndrome management, consideration of systemic immunosuppression (hydroxychloroquine, methotrexate, or other DMARDs), and monitoring for systemic complications including lymphoma risk. 2, 1

Critical Pitfalls to Avoid

• Do not dismiss burning tongue as "just stress" without excluding autoimmune disease—this symptom in combination with dry eyes is pathognomonic for Sjögren syndrome until proven otherwise. 1, 3
• Do not delay dental referral—severe sicca syndrome causes irreversible dental damage within months. 2
• Do not perform oral biopsy before checking coagulation studies and complete blood count, as autoimmune conditions may have associated thrombocytopenia or coagulopathy. 2
• Do not assume all oral ulcers are benign—any ulcer persisting beyond 2 weeks requires biopsy to exclude squamous cell carcinoma, lymphoma, or pemphigus. 2, 4
• Do not overlook systemic complications—Sjögren syndrome increases risk of non-Hodgkin lymphoma, interstitial lung disease, and renal tubular acidosis. 1

Alternative Diagnoses to Consider

If serologic testing for Sjögren syndrome is negative:

• Primary burning mouth syndrome (idiopathic neuropathic pain)—but this would not explain dry eyes or urticaria. 3, 5
• Pemphigus vulgaris or mucous membrane pemphigoid—check serum antibodies (Dsg1, Dsg3, BP180, BP230) and consider direct immunofluorescence on biopsy. 2, 1
• Behçet disease—but typically presents with genital ulcers and pathergy, not dry eyes. 1, 6
• Systemic lupus erythematosus—can cause oral ulcers and urticaria, but dry eyes less prominent. 1

Monitoring and Follow-up

• Recheck in 2 weeks after initiating symptomatic treatment to assess response. 2
• If ulcers persist beyond 2 weeks total duration, proceed immediately to biopsy regardless of other test results. 2, 4
• Monitor urticaria control with Urticaria Control Test (UCT) at each visit—target complete disease control. 2
• Long-term ophthalmology follow-up for corneal complications of dry eye. 1