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Key Features

Essentials of Diagnosis

  • Large, tense blisters that rupture, leaving denuded areas that heal without scarring

  • Caused by autoantibodies to specific components of the hemidesmosome

General Considerations

  • Relatively benign pruritic disease characterized by tense blisters in flexural areas, usually remitting in 5 or 6 years, with a course characterized by exacerbations and remissions

  • Oral lesions are present in about one-third of affected persons

Demographics

  • Most patients are over the age of 60 (often in their 70s and 80s)

  • Men are affected twice as frequently as women

Clinical Findings

Symptoms and Signs

  • Characterized by tense blisters in flexural areas

  • Predilection for groin, axillae, flexor forearms, thighs and shins, though may occur anywhere; some have oral involvement

  • Appearance of blisters may be preceded by urticarial or edematous lesions for months

  • May occur in various forms, including

    • Localized

    • Vesicular

    • Vegetating

    • Erythematous

    • Erythrodermic

    • Nodular

  • Course characterized by exacerbations and remissions

Differential Diagnosis

  • Pemphigus

  • Drug eruptions

  • Erythema multiforme major or toxic epidermal necrolysis

  • Bullous impetigo

  • Contact dermatitis

  • Dermatitis herpetiformis

  • Cicatricial pemphigoid

  • Paraneoplastic pemphigus

  • Linear IgA dermatosis

  • Pemphigus foliaceus

  • Porphyria cutanea tarda

  • Epidermolysis bullosa

  • Staphylococcal scalded skin syndrome

  • Herpes gestationis

  • Graft-versus-host disease

Diagnosis

Laboratory Tests

  • Circulating anti-basement membrane antibodies can be found in the sera of patients in about 70% of cases

Diagnostic Procedures

  • The diagnosis is made by biopsy and direct immunofluorescence examination

  • Light microscopy shows a subepidermal blister

  • With direct immunofluorescence, IgG and C3 are found at the dermal–epidermal junction

Treatment

Medications

  • If the patient has mild disease, ultrapotent topical corticosteroids may be adequate (Table 6–2)

  • Oral prednisone at dosages of 0.75 mg/kg/day is often used to achieve rapid control of more widespread disease

  • Oral tetracycline, 500 mg three times daily, alone or combined with nicotinamide—not nicotinic acid or niacin—(up to 1.5 g/day)

    • Slower in onset of action than prednisone

    • May control the disease in patients who cannot use corticosteroids

    • May allow decreasing or eliminating corticosteroids after control is achieved

  • Dapsone is particularly effective in mucous membrane pemphigoid

  • If these drugs are not effective, one of the following may be used as steroid-sparing agent:

    • Methotrexate, 5–25 mg orally weekly

    • Azathioprine, 50 mg once to three times orally daily

    • Mycophenolate mofetil, 1 g orally twice daily

  • Intravenous immunoglobulin, intravenous rituximab, and subcutaneous omalizumab have been used successfully in refractory cases

Table 6–2.Useful topical dermatologic therapeutic agents.

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