dyshidrosiform pemphigoid

Epidemiology

• rare, elderly

Pathology

• skin biopsy
  • subepidermal blisters with eosinophilic infiltration
  • direct immunofluorescence reveals strong linear fluorescence with continuous deposition along the basement membrane zone, C3 positive, IgA & IgM negative
  • IgE-activated mast cells, basophils, & eosinophils may play a role in inducing pruritus, blister formation & reduced response to systemic glucocorticoids

* images

Clinical manifestations

• recurrent itching & tense blisters on both hands
• recurrent or persistent vesicular eruptions resembling pompholyx
  • hemorrhagic or purpuric features, particularly in elderly
• blisters most commonly found on both the palms & soles (67%), soles only (30%), or palms only (3%), with 72% progressing elsewhere.

* image Labroatory;

• epidermal basement membrane IgG Ab in serum
  • ELISA using antibodies to bullous pemphigoid autoantigen-2 (COL171A, BP180)
  • anti BP230 (gene on 6p) [homology with desmoplakin]
• IgE in serum

Diagnostic procedures

• skin biopsy

Management

• oral methylprednisolone 8 mg QD for 7 days
• oral minocycline 50 mg BID +
• nicotinamide 200 mg TID +
• dupilumab 300 mg SQ biweekly for 3 months

More general terms

• pemphigoid