Vulvar involvement in autoimmune bullous diseases

Area of Science:

• Dermatology
• Immunology
• Gynecology

Background:

• Autoimmune blistering diseases manifest on skin and mucous membranes, with blisters often eroding into lesions due to friction.
• Vulval involvement is a notable, yet sometimes overlooked, presentation in these conditions.

Purpose of the Study:

• To investigate the prevalence and patterns of vulval involvement across a spectrum of autoimmune blistering diseases in female patients.
• To identify potential diagnostic challenges associated with mucosal and vulval presentations.

Main Methods:

• Retrospective analysis of 140 female patients diagnosed with various autoimmune blistering diseases.
• Review of clinical data focusing on the presence and severity of vulval and perineal involvement.

Main Results:

• Vulval involvement was observed in a significant proportion of patients across multiple diseases, including cicatricial pemphigoid (14/26), chronic bullous disease of childhood (16/20), linear IgA disease (9/22), epidermolysis bullosa acquisita (2/3 girls), and pemphigus vulgaris (4/9).
• Mucosal and perineal involvement led to initial misdiagnoses, including herpes simplex and sexual abuse, particularly in pediatric cases.
• Severe scarring was a common outcome in cicatricial pemphigoid with vulval involvement.

Conclusions:

• Vulval involvement is a common feature in autoimmune blistering diseases, irrespective of the specific diagnosis.
• The presentation of blistering diseases on mucosal surfaces, especially the vulva, can mimic other conditions, necessitating careful diagnostic evaluation.
• Increased awareness is crucial for timely diagnosis and management of these rare but potentially debilitating conditions.