Case Eleven – November 2017

  • Patient Information
    • 53 year old female presented with numerous flaccid blisters and erosions. Similar lesions were also present.
  • Clinical Diagnosis
    • Bullous pemphigoid
    • Pemphigus vulgaris
    • Erythema multiforme
  • Histology

    Sections show an acantholytic dermatitis affecting suprabasilar keratinocytes. Retention of basal keratinocytes within the floor of the blister resulting in a characteristic “tombstone” pattern is seen.

    Acantholysis involving the follicular epithelium is also present.

  • Final Diagnosis

    Pemphigus vulgaris

    • Pemphigus vulgaris (PV) is the most common type of pemphigus
      • Other subtypes of pemphigus include pemphigus foliaceus, drug-induced pemphigus, fogo selvagem, and paraneoplastic pemphigus
    • PV is an immunoblistering disease caused by autoantibodies to desmoglein 3, a component of desmosomes
    • PV clinically presents as flaccid blisters and erosions with frequent involvement of the mucous membranes, including the oral cavity and genitalia
    • Histopathology shows acantholysis of suprabasilar keratinocytes with adnexal involvement
      • Retention of the basal keratinocytes within the floor of the blister resulting in a “tombstone” pattern is frequently seen
      • Perivascular infiltration of eosinophils is also commonly present
    • Direct immunofluorescence study shows staining within the epidermis for IgG and/or C3 in a net-like pattern
    • The histologic differential diagnosis includes Darier’s disease and Hailey-Hailey disease
      • Darier’s disease shows more prominent acantholytic dyskeratosis with corp rounds and corp grains; DIF study is negative
      • Hailey-Hailey disease also shows acantholysis in a “dilapidated brick wall” pattern with the acantholysis sparing adnexal epithelium; DIF study is negative as well
    • PV is usually well controlled using corticosteroids and immunosuppressive drugs such as rituximab
  • Summary
    • PV is an immunoblistering disease caused by autoantibodies to desmoglein 3, a component of desmosomes
    • PV clinically presents as flaccid blisters and erosions with frequent involvement of the mucous membranes, including the oral cavity and genitalia
    • Histopathology shows acantholysis of suprabasilar keratinocytes with adnexal involvement and characteristic “tombstone” pattern at the base of the blister
    • Direct immunofluorescence study shows staining within the epidermis for IgG and/or C3 in a net-like pattern
    • The histologic differential diagnosis includes Darier’s disease and Hailey-Hailey disease