Yibian
 Shen Yaozi 
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diseaseBullous Pemphigoid
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bubble_chart Overview

Bullous pemphigoid is an autoimmune disease characterized by the formation of subepidermal blisters. The exact cause and pathogenesis of the disease remain unclear. Patients have circulating antibodies against the epidermal basement membrane zone, mostly IgG antibodies. The disease predominantly affects the elderly and children, with no gender difference. The course is chronic; pediatric patients may experience spontaneous remission after several years, but adult cases often do not resolve naturally. Dapsone and corticosteroids are effective treatments. The prognosis is generally good for children and most adult patients, but poor for those with concurrent malignant tumors. Fatalities are mainly due to drug complications or associated malignancies.

bubble_chart Clinical Manifestations

  1. The basic damage manifests as tense, serous blisters and bullae on the skin, ranging in size from pea-sized to walnut-sized. The blister walls are thick and not easily ruptured, with a negative Nikolsky's sign. After rupture, the erosions exude little fluid and heal easily. The blisters may resolve spontaneously or dry up, but new blisters may recur in the same area or appear elsewhere. Post-healing hyperpigmentation may occur, but no scarring forms.
  2. The lesions are primarily distributed on the flexural surfaces of the limbs, groin, armpits, and waist, and in severe cases, they may become generalized.
  3. Most cases do not involve oral mucosal damage.
  4. There may be cutaneous pruritus or a burning sensation, but generally no systemic symptoms.

bubble_chart Diagnosis

  1. The disease occurs in the elderly or children;
  2. The skin lesions present as tense blisters or bullae, with a negative Nikolsky sign, and erosions that heal easily;
  3. The lesions are mainly distributed on the flexural surfaces of the limbs, groin, armpits, and waist;
  4. Histopathology shows subepidermal blisters. Direct immunofluorescence reveals IgG and/or complement C3 deposition at the epidermal basement membrane zone. Indirect immunofluorescence can detect circulating anti-basement membrane zone antibodies (primarily IgG) in the blood. Salt-split skin direct immunofluorescence shows immune complex deposition on the epidermal side of the blister.

bubble_chart Treatment Measures

Treatment Principles

  1. Elderly patients should be screened for malignant tumors;
  2. Dapsone or corticosteroids can be used to control the condition, and maintenance therapy is required after remission.
  3. Skin lesions should be treated symptomatically according to topical medication principles.
Medication Principles
  1. Pemphigoid is often treated with dapsone or corticosteroids, and mild cases may try preparations of Root Leaf or Flower of Common Threewingnut.
  2. Patients sensitive to corticosteroids may combine immunosuppressants.
  3. Poor treatment efficacy should raise suspicion of concurrent malignant tumors.

bubble_chart Cure Criteria

  1. Cure: Skin lesions subside, erosions heal, and no new rashes appear;
  2. Improvement: Partial absorption of blisters, reduced exudation from erosions, and no new blisters;
  3. No cure: New blisters continue to appear.

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