A Rare Case of Extensive Pemphigus Vegetans

Area of Science:

• Dermatology
• Autoimmune Blistering Diseases

Background:

• Pemphigus vegetans is a rare variant of pemphigus vulgaris.
• Characterized by pustules and papillomatous vegetations, typically in intertriginous areas.
• Known subtypes include Hallopeau and Neumann variants.

Observation:

• Presents an unusual case of pemphigus vegetans with extensive mucosal, trunk, and extremity involvement.
• Features included verrucous paronychia, fissured tongue, vesicles, erosions, and ulcers.
• Cerebriform tongue morphology, a known sign, was noted.

Findings:

• Clinical, histopathological, and immunofluorescence findings confirmed pemphigus vegetans.
• Demonstrated extensive cutaneous and mucosal lesions.
• Patient achieved excellent response to intravenous dexamethasone-cyclophosphamide pulse (DCP) therapy.

Implications:

• Highlights the diverse clinical presentation of pemphigus vegetans.
• Suggests DCP regimen as an effective treatment option for extensive cases.
• Emphasizes the importance of recognizing varied manifestations for timely diagnosis and management.