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Pemphigus Vegetans

Rare variant of pemphigus vulgaris with vegetating plaques in flexural areas

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Pemphigus Vegetans?

Pemphigus vegetans is a rare cutaneous variant of pemphigus vulgaris (PV), accounting for 1-2% of all pemphigus cases. It is divided into two subtypes: Neumann type (more severe, begins like classic PV with bullae that progress to vegetating plaques) and Hallopeau type (milder, presents as pustules that evolve into vegetating plaques without preceding bullae).

Pathogenesis: IgG autoantibodies target desmoglein 3 (Dsg3) in deep epidermis, causing acantholysis. The vegetating response is thought to be a hyperplastic reactive process to chronic intraepidermal blistering. Histopathology shows suprabasal acantholysis with intraepidermal abscesses containing eosinophils, papillomatosis, and pseudoepitheliomatous hyperplasia.

Diagnosis: clinical recognition + skin biopsy (lesional and perilesional for DIF showing intercellular IgG and C3), indirect immunofluorescence, ELISA for anti-Dsg3 antibodies. Treatment: systemic corticosteroids (prednisone 1-2 mg/kg/day) + steroid-sparing agents (azathioprine, mycophenolate mofetil, rituximab). Prognosis: chronic relapsing course, mortality reduced with modern therapy.

Symptoms

Vegetating, hypertrophic plaques in flexural areas (axillae, groin, inframammary, perianal)
Oral involvement: vegetating cheilitis, glossitis, painful erosions
Pustules and vesicles preceding plaques (Hallopeau type)
Bullae that rupture and form vegetations (Neumann type)
Malodorous lesions with serosanguineous exudate
Cerebriform tongue (corrugated, hypertrophic)
Nail dystrophy (chronic disease)
Pruritus, pain, secondary bacterial/fungal infection

Risk Factors

Genetic predisposition (HLA-DR4, HLA-DR14, HLA-DRB1*0402)
Mediterranean, Jewish, Indian ethnicity
Adult onset (40-60 years, but can occur at any age)
Drug-induced (penicillamine, captopril, NSAIDs - rare)
Underlying autoimmune disease (myasthenia gravis, thymoma)
Female slightly predominates (1.5:1)
Stress, infections may trigger flares

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent erosions or vegetating lesions in flexural areas
  • Oral lesions not healing >2 weeks (especially in adult)
  • New blisters or bullae with mucosal involvement
  • Worsening despite topical treatment
  • Painful oral lesions interfering with eating
  • Foul-smelling skin lesions in folds
  • Family history of pemphigus with similar lesions

Treatment Methods

01
Systemic corticosteroids (prednisone 1-2 mg/kg/day) - first-line induction
02
Rituximab (1g × 2 doses, 2 weeks apart) - first-line for moderate-severe disease (modern guidelines)
03
Azathioprine (1-3 mg/kg/day) - steroid-sparing
04
Mycophenolate mofetil (1-3 g/day) - alternative steroid-sparing
05
IVIG (2 g/kg/cycle) - refractory cases
06
Cyclophosphamide (severe, refractory)
07
Plasmapheresis (acute severe cases)
08
Topical corticosteroids and antimicrobials (adjunct)
09
Wound care, infection prevention
10
Anti-Dsg3 antibody monitoring (ELISA) for treatment response
11
Long-term follow-up (relapse common)

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.