513 Nonbullous cutaneous pemphigoid: A systematic review

Abstract

Cutaneous pemphigoid is an autoimmune bullous disease that typically presents with tense bullae and severe itch. However, vesicles or bullae may be lacking, called nonbullous cutaneous pemphigoid. The purpose of our study is summarize the reported characteristics of nonbullous cutaneous pemphigoid. We performed a systematic search in the EMBASE and MEDLINE databases using ‘nonbullous cutaneous pemphigoid’ and various synonyms. Case reports and series describing nonbullous cutaneous pemphigoid were included in this study. The search identified 133 articles. After selection 39 articles were included, presenting 132 cases. Erythematous, urticarial plaques (52.3%) were most frequently reported. Papules/nodules were found in 20.5% of the cases. Mucosal involvement was reported in one case. Histopathology was commonly nonspecific. Linear depositions of IgG/C3 along the basement membrane zone by direct immunofluorescence microscopy were found in 93.2%. Indirect immunofluorescence on salt split skin was positive in 90.2%. The mean duration of symptoms before diagnosis was 22.6 months. During follow-up blisters occurred in a minority of patients (9.8%) after a mean duration of 9.6 months. In conclusion, nonbullous cutaneous pemphigoid is not rare and mostly does not evolve to bullous cutaneous pemphigoid, but mimics other pruritic skin diseases. Awareness among doctors is needed, since the doctors delay in diagnosis may be long.