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Patient handout

Bullous pemphigoid

PRODUCTION

1. Your condition

This handout is for bullous pemphigoid. Your care team identified this based on: tense bullae on erythematous/urticarial skin with intense pruritus in an elderly patient — the classic bullous phase (eadv s2k bp guideline, jeadv 2022; pmid 35766904).

Other reasons your team may use this plan: months of intractable pruritus ± urticarial/eczematous/prurigo-like lesions without blisters in the elderly — the non-bullous prodrome (commonly mis-labelled eczema/urticaria/scabies for months) (eadv 2022; pmid 35766904); new dpp-4 inhibitor (gliptin, esp. vildagliptin) / immune-checkpoint inhibitor / loop diuretic preceding a pruritic-bullous eruption → drug-induced bp (tasanen, front immunol 2019; pmid 31275298; sibaud, am j clin dermatol 2018; pmid 29256113); stroke / dementia / parkinson disease with a new blistering or chronically pruritic eruption (neurologic disease is an independent bp risk factor) (tasanen, front immunol 2019; pmid 31275298).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
withdraw_offending_drug_dpp4i_checkpoint_loop_diureticTasanen Front Immunol 2019 (PMID 31275298) + Sibaud Am J Clin Dermatol 2018 (PMID 29256113) — DPP-4 inhibitors (esp. vildagliptin), immune-checkpoint inhibitors and loop diuretics induce BP; withdrawing the culprit is disease-modifying and may obviate immunosuppression. Coordinate checkpoint-inhibitor hold with oncology.
gentle_wound_blister_care_and_infection_surveillanceEADV 2022 (PMID 35766904) — non-adherent dressings, blister decompression with roof preservation, emollients, and active surveillance for secondary infection (the leading cause of BP death).

Plan: Bullous pemphigoid — severity-tiered ladder, topical-clobetasol-first (EADV 2022 / Joly NEJM 2002 / BLISTER 2017)

3. When to call your provider

Contact your care team if any of the following happen:

  • Extensive BP with secondary infection / sepsis or erythroderma → admit + infection/sepsis pathway (EADV 2022 PMID 35766904)
  • Severe/scarring mucosal (esp. ocular) involvement → reassess for MMP + emergent ophthalmology/ENT (EADV MMP 2021 PMID 34309078)
  • Frail elderly unable to apply whole-body topical therapy + extensive disease → admit for supervised therapy

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • Extensive eroded/denuded BP with secondary skin infection, purulent crust/pustules, fever, or systemic toxicity / sepsis physiology(life-threatening)
  • Very extensive / near-erythrodermic BP with impaired thermoregulation, fluid/electrolyte loss, or haemodynamic stress
  • Predominant or scarring mucosal involvement, especially ocular (symblepharon/conjunctival scarring) or oral/laryngeal/oesophageal erosions
  • Rapidly progressive widespread blistering, or BP refractory to/intolerant of systemic corticosteroid + a steroid-sparing immunosuppressant
  • Systemic-corticosteroid complication in a frail elderly BP patient — hyperglycaemic crisis, serious infection, fracture, delirium, or GI bleed

5. Follow-up

Chronic relapsing-remitting maintenance: taper to lowest effective therapy (ideally topical/intermittent), relapse-recognition education (itch/new blisters), anti-BP180 titre trend for relapse prediction, periodic steroid/immunosuppressant safety surveillance, infection precautions, and — for drug-induced BP — a permanent culprit-avoidance flag (and an alternative-antidiabetic plan if gliptin-induced). Dermatology continuity for any systemic agent; reassess the diagnosis (repeat biopsy/serology) if the course is atypical or treatment-refractory.

6. Sources

Guideline: EADV updated S2k guideline for the management of bullous pemphigoid (Borradori et al, JEADV 2022; PMID 35766904) — high-potency topical corticosteroid is the mainstay first line; oral prednisone 0.5 mg/kg/day alternative; azathioprine/MMF/methotrexate steroid-sparing; doxycycline/dapsone when steroids contraindicated; rituximab/IVIG for resistant disease; omalizumab/dupilumab promising. Diagnosis per EADV autoimmune-bullous canon (perilesional DIF + lesional H&E + anti-BP180-NC16A/anti-BP230 ELISA + salt-split-skin IIF). Mucous-membrane-pemphigoid differential per EADV MMP S3 guideline Parts I+II (Rashid/Schmidt, JEADV 2021; PMID 34245180 / 34309078). Cornerstone trials: Joly NEJM 2002 topical-clobetasol superiority (PMID 11821508); Williams BLISTER Lancet 2017 doxycycline non-inferiority/safety (PMID 28279484).

  1. pubmed.ncbi.nlm.nih.gov/35766904
  2. pubmed.ncbi.nlm.nih.gov/11821508
  3. pubmed.ncbi.nlm.nih.gov/16038572