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.

• Cervical Lymphadenopathy

  The rest of the examination revealed cervical lymphadenopathy with Alteration of the general state. The skin biopsy showed papillomatous, verrucous epidermal hyperplasia with a suprabasal acantholysis. [scidoc.org]

  A positron emission tomography-computed tomography (PET-CT) scan was also carried out on suspicion of malignancy which revealed fluorodeoxyglucose (FDG)-avid lesions in the scalp, perioral region including lips, and nose, and bilateral cervical lymphadenopathy [ncbi.nlm.nih.gov]

• 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]

• 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]

  Pemphigus Vegetans is a blistering disease of the skin that I occasionally encounter in both my The Woodlands dermatology and Conroe dermatology offices. [perridermatology.com]

• Nikolsky's Sign

  Nikolsky’s sign Nikolsky`s sign was first described by a Russian dermatologist Pyotr Vasilievich Nikolskiy. [lecturio.com]

  […] and symptoms Flaccid blisters on face, scalp, upper body, and intertriginous areas Nikolsky sign – applying pressure on blister periphery extends lesion laterally Mucosal lesions and erosions Types of pemphigus Pemphigus vulgaris Initial presentation [arupconsult.com]

  They will look for an indicator of the condition called Nikolsky’s sign. A positive Nikolsky’s sign is when your skin shears off easily when the surface is wiped sideways with a cotton swab or a finger. [healthline.com]

  This is an indirect Nikolsky's sign. Healing is very slow, but no scars will remain. On the oral mucosa, bullae filled with fluid are also present but no inflammation develops. [contempclindent.org]

  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]

• 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]

• Hemorrhagic Bullae

  She reported no initial precipitating event as the lesion started with a hemorrhagic bulla on the soft palate [Figure 1] a. [njcponline.com]

• Chronic Dermatitis

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

• 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.

Possible active components of the treatment are discussed. [ncbi.nlm.nih.gov]

With long-term treatment, the condition can sometimes be controlled to the point that flare-ups no longer occur even after treatment is stopped. The treatments used to manage pemphigus vulgaris are similar to those used to treat severe burns. [news-medical.net]

The evolution was marked by subsidence of lesions in the first week of treatment with no new lesions. The treatment was continued for 3 months. Erythromycin side effects were minor, such as stomach pain managed by omeprazole drugs. [scidoc.org]

Treatment of pemphigus vegetans is similar to that for pemphigus vulgaris, and this is normally accomplished with systemic steroids [ 4 ]. [clinmedjournals.org]

Its prognosis is similar to pemphigus vulgaris. The controversy boils down to whether pemphigus vegetans of Hallopeau and pyodermite végétante (pyoderma vegetans) are separate diseases. [jamanetwork.com]

The prognosis for patients with this condition is much more positive than for patients with pemphigus vulgaris. [news-medical.net]

The Hallopeau type has a relatively benign course and responds to topical therapy, low dose steroids and occasionally to dapsone while the Neumann type has a poor prognosis and is resistant to treatment. [e-ijd.org]

PH is a rare form of pemphigus vegetans and it has a better prognosis with 1% to 2% of spontaneous remission [3]. It is also considered as an infectious disease [4] or as a disease associated with inflammatory enterocolitis [5]. [scirp.org]

To describe an unusual case of pemphigus vegetans occurring in a skin graft recipient site and to discuss the possible etiology. [ncbi.nlm.nih.gov]

The findings were typical of pemphigus, confirming the propriety of classifying this entity as a benign variant of pemphigus, rather than a disease of purely microbial etiology. [jamanetwork.com]

OBJECTIVE: In this retrospective study of P Veg, we aimed to analyse epidemiological, clinical, immunopathological and therapeutic data. Study of prognostic factors with accuracy of patient survival was also carried out. [ncbi.nlm.nih.gov]

You are ( * ) If you have selected the “Other” category, please specify which type of user you are: * Email address: * Topic of your comment * Epidemiology data Summary and related texts Related genes Clinical signs Nomenclature and/or coding Your message [orpha.net]

CIC Edizioni Internazionali, 28 oct. 2015 - 442 pagini Vulvar-vaginal disorders represent a field of growing interest in clinical, epidemiological and healthcare research. [books.google.ro]

Epidemiology : - Equal Incidence rate in both genders. - Most patient presenting during the 4th and 5th decades of life. - Genetic and ethnic factors: Ashkenazic Jews and Mediterranean patients. 4. [slideshare.net]

Epidemiologic analysis and clinical course of 84 consecutive cases of pemphigus in eastern Sicily. Int J Dermatol. 1998;37:197-200. 2. Mahé A, Flageul B, Cissé I, Kéita S, Bobin P. Pemphigus in Mali: a study of 30 cases. [scielo.br]

However, the pathophysiological significance of anti-periplakin reactivity in this pemphigus variant remains to be determined. [moh-it.pure.elsevier.com]

Pathogenesis Pathophysiology Postulated that it is a benign form of pemphigus vulgaris Skin: pemphigus vulgaris in dogs with some resistance to the disease. [vetstream.com]

[…] erythematosus (Senear-Usher syndrome) Pemphigus herpetiformis Paraneoplastic pemphigus Typically IgG antibodies IgA antibodies – rarely described IgA pemphigus – rare, with 2 main subsets Subcorneal pustular dermatoses Intraepidermal neutrophilic IgA dermatosis Pathophysiology [arupconsult.com]

The pathophysiological significance of nondesmoglein targets of pemphigus autoimmunity: development of antibodies against keratinocyte cholinergic receptors in patients with pemphigus vulgaris and pemphigus foliaceus Arch Dermatol 1998;134971- 980 PubMed [jamanetwork.com]

Treatment is aimed at reducing pain and symptoms and preventing complications like infection. It involves one or more drugs and other methods. [healthline.com]

Improved access to gynecological care and lengthening of life expectancy are the basis of increased incidence of chronic disorders requiring early diagnosis, prolonged treatment and adequate follow-up, in order to prevent development of neoplastic diseases [books.google.ro]

The main aim of treatment is to heal the blisters and prevent new ones forming. Steroid medication (corticosteroids) plus another immunosuppressant medication are usually recommended. These help stop the immune system damaging healthy tissue. [nhs.uk]

The aims of treatment are to prevent new blisters forming and to heal broken areas of skin. It usually takes about 2 to 3 weeks to stop new blisters developing, and 6 to 8 weeks for healing to occur. [britishskinfoundation.org.uk]

The primary aim of treatment of pemphigus vulgaris is to decrease blister formation, prevent infections and promote healing of blisters and erosions [4.5]. [dermnetnz.org]

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.