Atypical familial benign chronic pemphigus

Area of Science:

• Dermatology
• Genetics
• Autoimmune Diseases

Background:

• Familial benign chronic pemphigus (FBCP), also known as Hailey-Hailey disease, is a rare autosomal dominant disorder.
• It is characterized by recurrent blistering erosions, typically in intertriginous areas.

Observation:

• This report details two patients with FBCP exhibiting atypical clinical presentations.
• One patient presented with lesions confined to the anterior chest.
• The second patient had lesions localized exclusively to the lateral arms.

Findings:

• Neither patient displayed the characteristic prominent intertriginous involvement commonly seen in FBCP.
• The observed lesion distributions (anterior chest, lateral arms) are unusual for this condition.
• These cases underscore the variable and unpredictable nature of FBCP lesion localization.

Implications:

• The findings suggest that FBCP should be considered in the differential diagnosis of skin conditions even when classic intertriginous lesions are absent.
• Recognizing these protean manifestations is crucial for accurate and timely diagnosis.
• Further research into the genetic and environmental factors influencing FBCP phenotype variability may be warranted.