When can bullous pemphigoid present with subcutaneous hemorrhage or ecchymotic patches?

Bullous Pemphigoid Presentation with Subcutaneous Hemorrhage or Ecchymotic Patches

Bullous pemphigoid can present with subcutaneous hemorrhage or ecchymotic patches during atypical presentations, particularly in the early or prodromal phase of the disease before the development of classic tense bullae. 1

Clinical Presentations with Hemorrhagic Features

• Atypical presentations: Up to 20% of bullous pemphigoid cases may present without classic bullae, instead showing various atypical manifestations including ecchymotic patches 2

• Hemorrhagic variants: These can include:

  • Ecchymotic patches that later evolve into hemorrhagic bullae 1
  • Potentially hemorrhagic blisters in the dyshidrosiform variant (primarily affecting palms and soles) 3
  • Purpuric lesions that may precede the development of typical bullae 3

• Figurate erythema-like presentations: Some cases present with annular erythematous plaques resembling erythema annulare centrifugum, which may have hemorrhagic features 4

Diagnostic Considerations for Hemorrhagic Presentations

When bullous pemphigoid presents with subcutaneous hemorrhage or ecchymotic patches, diagnosis requires:

1. Histopathology: Biopsy from early lesions including ecchymotic areas should show:

   • Subepidermal clefting
   • Inflammatory infiltrate with eosinophils and/or neutrophils
   • Dermal infiltrate of eosinophils 2, 1

2. Direct Immunofluorescence (DIF): Essential for diagnosis, showing:

   • Linear deposits of IgG and/or C3 along the dermoepidermal junction
   • Biopsy should be taken from perilesional skin (1 cm away from lesion) 2

3. Serological tests:

   • Indirect immunofluorescence on salt-split skin
   • ELISA for anti-BP180 and anti-BP230 antibodies 2, 1

Clinical Contexts for Hemorrhagic Presentations

Hemorrhagic or ecchymotic presentations of bullous pemphigoid are more likely to occur in:

• Elderly patients: BP predominantly affects those over 70 years of age 2, 1

• Patients with neurological disorders: There's a significant association between BP and neurological conditions such as dementia, Parkinson's disease, and cerebrovascular disease 1, 3

• Medication-induced cases: Certain medications (diuretics, neuroleptics, gliptins) may trigger atypical presentations 1

• Dyshidrosiform variant: This rare variant can present with hemorrhagic blisters on palms and/or soles before spreading to other body sites 3

Differential Diagnosis for Hemorrhagic Lesions

When encountering hemorrhagic or ecchymotic lesions, consider:

• Erythema multiforme
• Urticarial vasculitis
• Subacute cutaneous lupus erythematosus
• Erythema annulare centrifugum
• Viral or bacterial skin infections 4, 3

Management Approach

For bullous pemphigoid presenting with ecchymotic or hemorrhagic features:

1. Initial treatment based on disease severity:

   • Topical high-potency corticosteroids for localized disease
   • Systemic corticosteroids (prednisolone) for widespread disease 2, 1

2. Adjuvant therapies:

   • Tetracyclines (doxycycline)
   • Azathioprine as a steroid-sparing agent 1

3. Monitoring:

   • BP180 ELISA titers may correlate with disease activity and can be used to monitor treatment response 1

Clinical Pitfalls to Avoid

• Delayed diagnosis: Atypical presentations with hemorrhagic features are often misdiagnosed, leading to delayed treatment 4, 5

• Failure to consider BP in elderly patients with unexplained ecchymoses: Maintain high index of suspicion for BP in elderly patients with unexplained ecchymotic lesions 5

• Overlooking associated conditions: Always assess for neurological disorders and review medications for potential triggers 1

• Inadequate biopsy technique: Ensure proper biopsy site selection (perilesional skin) for accurate DIF results 2