Brunsting-Perry Type Pemphigoid Causing Secondary Cicatricial Alopecia in 2 Patients

Area of Science:

• Dermatology
• Immunodermatology
• Pathology

Background:

• Brunsting-Perry type pemphigoid (BPP) is a rare subepidermal blistering disease.
• It is recognized as a cause of secondary cicatricial alopecia, primarily affecting the scalp.
• BPP was initially described as a variant of cicatricial pemphigoid with localized bullous lesions.

Purpose of the Study:

• To report two cases of BPP with secondary cicatricial alopecia.
• To investigate the diverse clinical presentations and immunofluorescence findings in BPP.
• To explore the potential association between BPP and other autoimmune blistering diseases like bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA).

Main Methods:

• Histopathological examination of skin biopsies to confirm subepidermal blister formation.
• Clinical assessment of lesion distribution and mucosal involvement.
• Salt-split indirect immunofluorescence testing to evaluate antibody targets.

Main Results:

• Two distinct cases of BPP-associated cicatricial alopecia were presented.
• One patient exhibited features of bullous pemphigoid with significant oral mucosal involvement.
• The second patient presented with characteristics of epidermolysis bullosa acquisita.

Conclusions:

• Secondary cicatricial alopecia in BPP may result from different antigens associated with BP or EBA.
• Epitope spreading is a potential mechanism explaining the co-occurrence of distinct autoimmune blistering diseases.
• The specific target antigen in Brunsting-Perry type pemphigoid remains to be definitively identified.