What is the likely diagnosis and emergent management for a patient with a painful, swollen nose that presses on the eye and has multiple ulcerated skin lesions?

Rhino-Orbital Mucormycosis Until Proven Otherwise

This presentation—painful, swollen nose with ulcerated lesions and orbital pressure—demands immediate consideration of invasive fungal infection (mucormycosis), particularly in diabetic or immunocompromised patients, and requires urgent ENT/ophthalmology consultation, tissue biopsy with fungal staining, and empiric antifungal therapy while awaiting confirmation. 1

Immediate Red Flag Assessment

This constellation of findings represents a medical emergency requiring same-day specialist evaluation. The combination of:

• Unilateral facial edema with nasal involvement 1
• Multiple ulcerated skin lesions 1
• Orbital pressure symptoms 1

These features are classic for rhino-orbito-cerebral mucormycosis, which typically originates in the paranasal sinuses with subsequent bone destruction and invasion of the orbit, eye, and potentially brain. 1 Mortality rates range from 40-80%, with the poorest prognosis in diabetic patients and those with disseminated disease. 1

Critical Differential Diagnoses to Consider

Primary Concern: Invasive Fungal Infection (Mucormycosis)

• Rhino-orbital-cerebral mucormycosis develops most commonly in diabetic patients and presents with unilateral facial edema, proptosis, and palatal or palpebral necrosis. 1
• Black necrotic eschars on nasal mucosa or palate are pathognomonic but not always present initially. 1
• The disease spreads contiguously through tissue planes and blood vessels, causing thrombosis and tissue necrosis. 1

Secondary Considerations

Bacterial sinusitis with orbital complications:

• Purulent pansinusitis can cause orbital cellulitis with periorbital edema, erythema, and proptosis. 2
• However, multiple skin ulcerations are atypical for uncomplicated bacterial sinusitis. 1, 2

Pyoderma gangrenosum:

• Can present with deep excavating ulcerations containing purulent material (sterile on culture). 1
• May involve orbital inflammation and scleritis. 3
• However, nasal involvement with orbital pressure is uncommon. 1

Granulomatosis with polyangiitis (Wegener's):

• Approximately 30% have ocular involvement with orbital disease being most common. 4
• Can cause nasal ulceration and orbital inflammation. 4
• Typically more indolent than the acute presentation described. 4

Emergent Diagnostic Workup

Immediate Laboratory Studies

• Complete blood count with differential to assess for neutropenia or leukocytosis 1
• Comprehensive metabolic panel including glucose and renal function 1
• Hemoglobin A1c to assess diabetic control 1
• Inflammatory markers (ESR, CRP) 4

Urgent Imaging

• CT scan of paranaosinus sinuses with contrast to evaluate bone destruction, soft tissue invasion, and orbital involvement 1
• MRI with gadolinium if intracranial extension is suspected (provides superior soft tissue detail) 1

Tissue Diagnosis (Cannot Be Delayed)

• Urgent ENT consultation for endoscopic examination and biopsy of necrotic tissue 1, 5
• Tissue must be sent for: 1, 5
  • Routine histopathology with special stains for fungi
  • Fungal culture
  • Bacterial culture
• Do not crush tissue during collection—use appropriate forceps or scissors 5

Emergent Management Algorithm

Step 1: Immediate Stabilization (Within Hours)

• Admit to hospital for close monitoring 1
• Ophthalmology consultation to assess visual acuity, extraocular movements, and perform slit-lamp examination 1
• Check for warning signs of cavernous sinus thrombosis: diplopia, ophthalmoplegia, reduced visual acuity, severe headache, altered consciousness 1

Step 2: Empiric Antifungal Therapy (Do Not Wait for Culture Results)

If mucormycosis is suspected based on clinical presentation and patient risk factors:

• Initiate liposomal amphotericin B immediately (first-line agent for mucormycosis) 1
• Delay in antifungal therapy significantly increases mortality 1
• Earlier diagnosis and treatment are directly correlated with improved survival 1

Step 3: Aggressive Surgical Debridement

• Multidisciplinary approach involving ENT surgery, ophthalmology, and potentially maxillofacial surgery 1, 2
• Surgical debridement is essential and must be aggressive to remove all necrotic tissue 1
• Improved survival is directly related to early, aggressive surgical intervention 1

Step 4: Address Underlying Risk Factors

• Optimize diabetic control with insulin therapy if hyperglycemic 1
• Reduce immunosuppression if feasible 1
• Correct metabolic acidosis if present 1

Critical Pitfalls to Avoid

Do Not Delay for "Complete Workup"

• Mucormycosis progresses rapidly with mortality exceeding 80% when diagnosis is delayed 1
• Initiate empiric therapy based on clinical suspicion while awaiting tissue confirmation 1

Do Not Assume Bacterial Infection

• Purulent-appearing material in mucormycosis is often sterile or contains secondary bacterial colonization 1
• Antibiotics alone will not treat invasive fungal infection 1

Do Not Manipulate Nasal Lesions Aggressively

• The nasal vestibule's venous drainage to the cavernous sinus creates risk for intracranial spread 6
• Avoid squeezing or aggressive manipulation of nasal furuncles or ulcers 6

Do Not Miss Orbital Complications

• Periorbital edema/erythema, displaced globe, double vision, ophthalmoplegia, or reduced visual acuity require emergency ophthalmology evaluation 1
• These signs indicate orbital or intracranial extension requiring immediate intervention 1, 2

Alternative Diagnoses If Mucormycosis Excluded

If tissue biopsy and fungal stains are negative:

For bacterial etiology:

• Broad-spectrum IV antibiotics covering MRSA and gram-negative organisms 1, 2
• Surgical drainage of any abscesses 1, 2

For pyoderma gangrenosum:

• Systemic corticosteroids as first-line therapy 1
• Immunomodulation with azathioprine or anti-TNF agents for resistant cases 1

For granulomatous disease:

• Tissue biopsy showing non-caseating granulomas supports vasculitis 5
• Systemic immunosuppression guided by rheumatology 4

Follow-Up Monitoring

• Daily ophthalmologic examination during acute illness to monitor for progression 1
• Serial imaging to assess response to therapy and extent of disease 1
• Repeat surgical debridement as needed until all necrotic tissue is removed 1