Pemphigus vegetans is a subtype of pemphigus vulgaris, an autoimmune disease that leads to the persistent formation of intraepidermal bullae and lesions and can be potentially fatal [1] [2].

Pemphigus vegetans is a broader term that encompasses two disease types: the Hallopeau type and the Neumann type. The hallopeau disease type displays a less aggressive course and initially manifests with pustules, which, upon eruption lead to the formation of pink papillomatous lesions [3]. The Neumann type, on the other hand, starts to manifest with vesicles and bullae and follows a course with more outbreaks and a reduced response to therapeutic intervention. The lesions that develop during the disease are significantly more extensive than those of the Hallopeau type [4].

Both types of pemphigus vegetans are characterized by initial lesions, either pustules or bullae, which progressively assume vegetative characteristics and hypertrophy and are frequently misdiagnosed as warts. The vegetative lesions can expand considerably, with new bullae or pustules developing around their borders.

• Painter

  CASE REPORT A 45-year-old wood furniture painter was brought to the dermatology outpatient department with complaints of recurrent painful oral erosions for the last three years. [ncbi.nlm.nih.gov]

  A 45-year-old wood furniture painter was brought to the dermatology outpatient department with complaints of recurrent painful oral erosions for the last three years. [idoj.in]

• Increased Susceptibility to Infections

  This condition is associated with a decrease in Th1 cytokines interleukin-2 and interferon-γ, eventually resulting in an increased susceptibility to infection and protection against autoimmunity. [karger.com]

• Otalgia

  Complications such as deafness, otalgia, otorrhea and facial paralysis are secondarily described [4], though they are absent in our observations. [scirp.org]

• Blister

  A patient with painful erosions of the oral cavity and the labia minora developed multifocal blisters in inter-triginous areas. These blisters eroded and evolved into papillomatous erosive vegetations. [ncbi.nlm.nih.gov]

  Signs of acantholysis are evident within blister cells. Epithelial hyperplasia is a finding that is common amongst patients affected by pemphigus vulgaris. [symptoma.com]

  Sometimes, the blisters spread to the voice box, causing hoarseness. A few weeks or months later, blisters may start to form on the skin. [news-medical.net]

  blister), is also required for direct immunofluorescence (DIF). [pcds.org.uk]

• Nikolsky's Sign

  Different types of Nikolsky’s sign: Marginal Nikolsky’s sign : Erosion of perilesional skin on the application of pressure is called marginal Nikolsy s sign. This sign is 69 % sensitive in detecting PV. [lecturio.com]

  Lesions heal without scarring The Nikolsky sign is positive - this is where firm sliding pressure with a finger will cause an erosion Flexural involvement readily forms vegetating granulomas Nails Acute or chronic paronychia, subungual haematomas, and [pcds.org.uk]

  […] blisters and erosions on trunk, face, scalp, and proximal limbs, positive Nikolsky sign Pemphigus vegetans – intertriginous and oral with involvement of tongue (cerebriform tongue) Flaccid bullae and erosions (Neumann type) Pustules (Hallopeau type) [arupconsult.com]

  Nikolsky sign was negative. [njcponline.com]

  The Nikolsky sign is not specific for pemphigus vulgaris and may also be observed in other skin blistering diseases such as pemphigus foliaceus, staphylococcal scalded skin syndrome, and toxic epidermal necrolysis. [dermatologyadvisor.com]

• Macula

  Regression left a pigmented macula. [scirp.org]

• Chronic Dermatitis

  But plasma cells could be a histopathological feature of most of the cases with chronic dermatitis. [e-ijd.org]

• Pachyonychia

  She also had hyperkeratotic plaques on the borders of both feet ( Figure 2 ), as well as verrucous paronychia of the 20 nails ( Figure 3 ) with pachyonychia and onychorrhexis. Laboratory tests were normal. [scielo.br]

• Neglect

  He suffered from malnutrition due to parental neglect since he was 8 years old. Three and a half years later, he complained of extreme pain in the mouth, which resulted in poor ability of oral food intake. [karger.com]

For an accurate diagnosis of pemphigus vegetans to be made, the initial step involves diagnosing the wider pathology, which is essentially pemphigus vulgaris. A histopathologic analysis from the blister's margin, direct immunofluorescence (DIF) on the skin adjacent to the lesion that appears normal and indirect immunofluorescence (IDIF) in the serum after DIF has yielded positive results, are all useful tools towards the diagnosis of the disorder.

More specifically:

• Histopathology: a histologic analysis can delineate the background of blister formation; it clearly illustrates the detachment of the suprabasal epidermal cells from their basal counterparts in such a way that bullae are formed. Typically, basal cells remain connected to the basement membrane, even though they assume positions at a distance from one another. Signs of acantholysis are evident within blister cells [5]. Epithelial hyperplasia is a finding that is common amongst patients affected by pemphigus vulgaris [6].
• DIF: It usually illustrates immunoglobulin G (IgG) deposits on the superficial side of keratinocytes, with the primary Ig subclasses being IgG1 and IgG4. Deposits of other immune reactants can be detected occasionally, including complement components and IgM. DIF is carried out on skin that has been harvested from the area that surrounds the lesion and has a normal-looking appearance [7]. The results of a DIF, however, are non-specific to pemphigus vulgaris.
• IDIF: Due to the low specificity of the DIF test, in the light of positive results, an IDIF is required. This test can detect IgG autoantibodies in the serum, attached to the epidermal layer of the skin. Intercellular antibodies in the serum are detected in the vast majority of the patients affected by pemphigus vulgaris.

Findings, such as the ones described above, that correlate to pemphigus vulgaris, combined with a clinical presentation associated with pemphigus vegetans are the key to achieving a precise diagnosis of the latter.

1. Ahmed AR, Moy R. Death in pemphigus. J Am Acad Dermatol. 1982 Aug. 7(2):221-8.
2. Stanley JR. Pemphigus. In: Wolff K, Goldsmith LA, Katz SI, Gilch-rest BA, Paller AS, Leffell DJ, editors. Fitzpatrick's dermatology in general medicine. 7th ed. New York: McGraw-Hill; 2008. pp. 459–468.
3. Cozzani E, Christana K, Mastrogiacomo A, et al. Pemphigus vegetans Neumann type with anti-desmoglein and anti-periplakin autoantibodies. Eur J Dermatol. 2007;17:530–533.
4. Ahmed AR, Blose DA. Pemphigus vegetans. Neumann type and Hallopeau type. Int J Dermatol. 1984 Mar;23(2):135-41.
5. Abreu Velez AM, Calle-Isaza J, Howard MS. Autoimmune epidermal blistering diseases. Our Dermatol Online. 2013; 631-646.
6. Jansen T, Messer G, Meurer M, Plewig G. Pemphigus vegetans. A historical perspective. Hautarzt. 2001;52:504-9.
7. Helander SD, Rogers RS 3rd. The sensitivity and specificity of direct immunofluorescence testing in disorders of mucous membranes. J Am Acad Dermatol. 1994 Jan. 30(1):65-75.