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For further information, see CMDT Part 6-44: Pemphigus

Key Features

Essentials of Diagnosis

  • Relapsing crops of skin bullae

  • Often preceded by mucous membrane bullae, erosions, and ulcerations

  • Superficial detachment of the skin after pressure or trauma variably present (Nikolsky sign)

  • Acantholysis on biopsy

  • Immunofluorescence studies and serum ELISA for pathogenic antibodies are confirmatory

General Considerations

  • Pemphigus is an uncommon intraepidermal blistering disease occurring on skin and mucous membranes

  • Caused by autoantibodies to adhesion molecules expressed in the skin and mucous membranes

  • There are several forms of pemphigus

    • Pemphigus vulgaris and its variant, pemphigus vegetans

    • Pemphigus foliaceus, which is more superficially blistering, and its variant, pemphigus erythematosus

    • Paraneoplastic pemphigus

  • The vulgaris form begins in the mouth in over 50% of cases

  • The foliaceus form is especially apt to be associated with other autoimmune diseases, or it may be drug-induced

  • Paraneoplastic pemphigus, a unique form of the disorder, is associated with numerous types of benign and malignant neoplasms but most frequently chronic lymphocytic leukemia, Castleman disease, B cell lymphoma, plasmacytoma, and thymoma

Demographics

  • All forms may present at any age but most commonly in middle age

Clinical Findings

Symptoms and Signs

  • An insidious onset of flaccid bullae, crusts, and erosions in crops or waves

  • The bullae appear spontaneously and are tender and painful when they rupture

  • In pemphigus vulgaris, lesions often appear first on the oral mucous membranes, and these rapidly become erosive

  • The scalp is another site of early involvement

  • Rubbing a cotton swab or finger laterally on the surface of uninvolved skin may cause easy separation of the epidermis (Nikolsky sign)

  • Pemphigus vegetans presents as erosive vegetating plaques, most often in intertriginous areas

  • Pemphigus foliaceus

    • A superficial form of pemphigus where cutaneous lesions often present as flaccid bullae that quickly evolve into superficial erosions and thin pink plaques with overlying scale

    • Mucosal lesions are rare

  • Pemphigus erythematosus

    • Has overlapping features of pemphigus foliaceus and lupus erythematosus

    • Presents with flaccid bullae that develop overlying scale and crust in a photodistributed area

    • Mucosal lesions are rare

  • Paraneoplastic pemphigus

    • Histologically and immunologically distinct from other forms

    • Oral lesions predominate

    • Cutaneous erythematous plaques resembling erythema multiforme are characteristic

    • Survival rates are low because of the underlying malignancy

Differential Diagnosis

  • Erythema multiforme, Stevens-Johnson syndrome, toxic epidermal necrolysis

  • Drug eruptions

  • Bullous impetigo

  • Contact dermatitis

  • Dermatitis herpetiformis

  • Bullous pemphigoid

  • Subacute cutaneous lupus erythematosus

Diagnosis

Laboratory Tests

  • Light microscopy and direct and indirect immunofluorescence microscopy confirm the diagnosis

  • ELISA assay can detect autoantibodies to intercellular adhesion molecules

Treatment

Medications

  • See Table 6–2

  • General measures

    • Bed rest and intravenous antibiotics ...

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