Diagnosis: Hailey Hailey disease
Description: The most prominent is acantholysis involving the epidermis. This is causing suprabasilar clefting with a 'tombstone' appearance of residual keratinocytes in the basal epidermis. No viral inclusions can be seen in these acantholytic cells or nearby epidermal cells.
Clinical Features: Lines of Blaschko
Pathology/Site Features: Acantholysis
Sex: F
Age: 56
Submitted By: Ian McColl
Differential DiagnosisHistory:
5155js Sudden onset of a burning periocular rash with spread to the neck. However also slight involvement of left periocular skin and right jaw. DD Plant contact, Herpes simplex or Zoster. Swabs and biopsy
Description: dermal eosinophils
Comments: Sections show a biopsy of skin in which a number of different changes are present. The most prominent is acantholysis involving the epidermis. This is causing suprabasilar clefting with a 'tombstone' appearance of residual keratinocytes in the basal epidermis. No viral inclusions can be seen in these acantholytic cells or nearby epidermal cells. Adjacent to the area of acantholysis there is prominent accumulation of neutrophils within the stratum corneum. The underlying dermis shows a superficial, mid and deep dermal infiltrate of neutrophils, eosinophils and some lymphocytes. The lymphocytes are mainly perivascular in location whilst the eosinophils are predominantly interstitial in location. The acantholytic changes present either pemphigus vulgaris or late onset Hailey-Hailey disease (would fit better with dermatomal distribution). The other changes are impetiginization (would certainly go with Hailey-Hailey disease) and a dermal infiltrate that looks more like an arthropod bite reaction. Herpes virus PCR studies are negative.