Case Eleven – November 2017
- 53 year old female presented with numerous flaccid blisters and erosions. Similar lesions were also present.
- Bullous pemphigoid
- Pemphigus vulgaris
- Erythema multiforme
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.
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
- 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