article · Cureus
Pemphigus vegetans is a rare variant of the autoimmune condition pemphigus vulgaris, typically marked by vegetating plaques in intertriginous skin folds. A clinical report documents a thirty-four-year-old woman presenting with persistent, painful oral lesions followed by progressive involvement across flexural areas, palms, soles, and all nails. Clinical examination showed severe erosive vegetating plaques in major folds alongside oral erosions and palmoplantar pustules. Diagnostic testing, including histopathology and immunofluorescence, identified characteristic features such as acantholysis, suprabasal cleavage, intercellular immunoglobulin G, and complement deposits, confirming Hallopeau-type pemphigus vegetans. Therapy combining systemic corticosteroids and rituximab led to the progressive healing of both mucosal and cutaneous lesions, as well as subsequent regrowth of the nails. The findings emphasise recognising extensive nail disease as a rare but distinctive feature of this disorder.
Rare autoimmune skin diseases can mimic other conditions and lead to severe tissue damage if not identified swiftly. Demonstrating that widespread nail involvement can accompany classical skin-fold lesions helps clinicians recognise atypical presentations of pemphigus vegetans. Furthermore, documenting successful resolution using established therapies like rituximab and corticosteroids provides valuable clinical evidence for managing challenging, multi-site manifestations of rare autoimmune disorders.
The abstract describes an individual clinical case treated with existing pharmaceutical therapies and does not indicate an application pathway or commercial development opportunity.
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Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by vegetating plaques predominantly involving intertriginous areas.A 34-year-old woman presented with persistent painful oral lesions followed by progressive flexural, palmoplantar, and pan-ungual involvement.Examination revealed exuberant erosive vegetating plaques of the major folds, associated with oral erosions, palmoplantar pustules, and diffuse nail involvement.Histopathology showed papillomatosis, acanthosis, suprabasal cleavage, and acantholysis.Direct immunofluorescence demonstrated intercellular immunoglobulin G and complement C3 deposits, while indirect immunofluorescence detected circulating anti-intercellular substance antibodies, confirming Hallopeau-type pemphigus vegetans.Treatment with systemic corticosteroids and rituximab resulted in progressive healing of the cutaneous and mucosal lesions, with subsequent nail regrowth.This case highlights the importance of considering pemphigus vegetans in patients with vegetating lesions of the folds associated with mucosal involvement and extensive pan-ungual involvement, a rare but clinically distinctive feature of this disease.
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DOI: 10.7759/cureus.115333
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