Brunsting-Perry cicatricial pemphigoid pathology

Introduction

Brunsting-Perry cicatricial pemphigoid is a rare form of localised cicatritial pemphigoid, commonly occurring on head and neck region. Interestingly, it usually does not involve the mucosal membranes as seen in typical cicatricial pemphigoid. Clinical differential is localised bullous pemphigoid, in which there is hardly any scarring in comparison to Brunsting Perry cicatritial pemphigoid.

Histology of Brunsting-Perry cicatricial pemphigoid

Microscopy reveals subepidermal blistering with various admixture of inflammatory cell infiltrate. Early lesions may show small papillary microabscesses

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Images provided by Dr Duncan Lamont, Waikato Hospital

Special studies in Brunsting-Perry cicatricial pemphigoid

Immunofluorescence shows basement membrane zone IgG and/or C3.

Electron microscopy reveals the split in the sublamina densa with preserved basal lamina and anchoring fibrils on the roof of the blister.

Differential diagnosis of Brunsting-Perry cicatricial pemphigoid

Localised bullous pemphigoid