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.

• Malnutrition

  Conversely, malnutrition may also reduce vulnerability towards autoimmune diseases. [karger.com]

  Oral involvement is exceedingly common (60 %) often since the beginning of the disease (75%), leading sometimes to malnutrition [3, 7]. [scidoc.org]

  Complications Possible complications of pemphigus include: Infection of your skin Infection that spreads to your bloodstream (sepsis) Malnutrition, because painful mouth sores make it difficult to eat Medication side effects, such as high blood pressure [mayoclinic.org]

  The rare case of pemphigus vegetans in association with malnutrition children in the multidisciplinary management. Case Rep Dermatol. 2017;9(1):145-50. doi: 10.1159/000473875. [PubMed: 28559814]. [PubMed Central: PMC5437434]. 8. [brieflands.com]

  […] sports) if you have blisters on your skin Complications of pemphigus vulgaris Pemphigus vulgaris can lead to other problems, such as: blisters and sore patches becoming infected, which can cause life-threatening problems such as sepsis weight loss and malnutrition [nhs.uk]

• Swelling

  The cutaneous features of cryptococcosis include papules, pustules, nodules, subcutaneous swelling, abscesses, molluscum contagiosum-like or tumour-like lesions, cellulitis, blisters, ulcers and very rarely, necrotizing fasciitis (NF). [ncbi.nlm.nih.gov]

  Case Presentation A 62-year-old female, hailing from a rural area, presented with the chief complaints of swelling over upper and lower lips with fissures and raw areas from two months ago. [brieflands.com]

  Correspondence to Rachel Marchalik, rjb254{at}georgetown.edu Summary An elderly patient presented with a 4-month history of eroded hypertrophic condylomatous plaques with areas of vesiculation on the groin and lower extremities bilaterally, associated with swelling [casereports.bmj.com]

• Severe Pain

  The involvement of oral mucosa is usually associated with severe pain, eventually leading to weight loss and malnutrition. Malnutrition in general may reduce vulnerability towards autoimmune diseases [6]. [karger.com]

  Blistering may be accompanied by severe pain, itching, burning, and stinging. If extensive, blistering can lead to life-threatening fluid loss, infection, and disfigurement. [skinsight.com]

  Blisters may be painful. They may heal and leave dark patches on the skin for months. Most people with pemphigus vulgaris feel better with treatment. Without treatment, the condition can lead to severe pain and infection. [hopkinsmedicine.org]

• Fatigue

  Some subjects are asymptomatic, milder cases may present with fatigue, weakness, dizziness, nocturia and polydipsia whereas others can show severe musculoskeletal symptoms such as tetany and cramps.[2],[4] Exceptionally, Gitelman syndrome may be acquired [ijdvl.com]

  Fatigue. Muscle weakness. Vision problems and light sensitivity. Where does pemphigus form on my body? Pemphigus can form on your skin in different parts of your body. The most common places include your: Mouth and throat. Genitals. [my.clevelandclinic.org]

  […] abdominal pain, aphthae in oral mucosa, asthenia, weight loss, and anorexia Folic acid or Vitamin B12 deficiency – Oral pain, erythematous tongue, asthenia and anemia, paresthesias in limbs, and physical problems hypochromic iron deficiency pallor, fatigue [jdrr.org]

  […] by abdominal pain, aphthae in oral mucosa, asthenia, weight loss, and anorexia Folic acid or vitamin B12 deficiency Oral pain, erythematous tongue, asthenia and anemia, paresthesias in limbs, and physical problems Hypochromic iron deficiency Pallor, fatigue [omicsonline.org]

• 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]

• Intestinal Disease

  With increasing travel and increasing immunosuppression, parasitic intestinal diseases are now seen in every type of practice. The standard diagnostic procedures described herein reduce costs in therapy. [books.google.com]

• 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]

  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]

  Note how broad is the suprabasal blister. 10.77.3A: Vesicular dermatitis, intraepidermal, acantholytic + 10.77.3B: Acantholytic cells amid neutrophils in a blister in the lower part of the epidermis and in an eccrine duct = Pemphigus vulgaris, fully developed [derm101.com]

• Eruptions

  We report the case of a patient with a typical skin eruption of pemphigus vegetans, who was detected with colonic cancer. [ncbi.nlm.nih.gov]

  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. [symptoma.com]

  Pemphigus vegetans was first described in 1876 in a case of localized bullae that evolved into a verrucous plaque.1 This was followed by a similar widespread cutaneous and oral eruption. [jamanetwork.com]

  The eruption can develop and progress very quickly, over days. It is possible to have just the skin eruption or just the mouth eruption ; both sites are not always affected. [dermnetnz.org]

• Nikolsky's Sign

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

  […] of epidermal cells intraepidermal blister direct immunofluorescence intercellular deposition of IgG or C3 in net-like (reticular) pattern Differential Bullous pemphigoid less severe than PV does not affect mucous membranes negative Nikolsky sign Treatment [step2.medbullets.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]

  […] become widely known as “Nikolsky’s sign.” [hindawi.com]

• Hyperkeratosis

  All 3 biopsies showed histological features typical of pemphigus which included extensive suprabasal acantholysis with bullae formation, prominent villus-like processes at the base of the bullae, focal hyperkeratosis and papillomatosis, and the occasional [ncbi.nlm.nih.gov]

  On histopathologic exam, one finds hyperkeratosis, papillomatosis, and acanthosis in addition to suprabasilar clefts with acantholytic cells and eosinophils. [dermnet.com]

  Chronic vegetative lesions in both subtypes reveal hyperkeratosis, acanthosis, papillomatosis and a suprabasal cleft with eosinophils. [ijdvl.com]

  A skin biopsy revealed an epidermis with intense acanthosis and papillomatosis, hyperkeratosis and focal parakeratosis, with a subcorneal intraepidermal bulla that contained shed keratinocytes mixed with lymphocytes, eosinophils, and polymorphonuclear [actasdermo.org]

  Figure 5: Fissured tongue Subungual hyperkeratosis, discolored and thin nail plate with verrucous paronychia and flaccid bullae on third and fourth digits were also seen. General physical and systemic examination revealed nothing abnormal. [journals.lww.com]

• Keratosis

  In nonintertriginous locations, the vegetating buttons can dry out to change into warty, fissured, painful, seborrheic keratosis-like lesions (dry P Veg). [ncbi.nlm.nih.gov]

  Pemphigus foliaceous L10.3 Brazilian pemphigus [fogo selvagem] L10.4 Pemphigus erythematosus L10.5 Drug-induced pemphigus L10.8 Other pemphigus L10.81 Paraneoplastic pemphigus L10.9 Pemphigus, unspecified L11 Other acantholytic disorders L11.0 Acquired keratosis [icd10data.com]

  […] are also present at basement membrane Differential diagnosis Differential diagnosis of acantholysis includes: Darier's disease D-penicillamine related pemphigus like lesions Familial benign pemphigus Hailey-Hailey disease Viral vesicles Also: Actinic keratosis [pathologyoutlines.com]

  Cutis Current and Past Issues For Authors CME COVID-19 Updates Diseases & Conditions Acne Actinic Keratosis Aesthetic Dermatology Atopic Dermatitis Autoimmune Diseases Contact Dermatitis Hair & Nails Melanoma Nonmelanoma Skin Cancer Pigmentation Disorders [mdedge.com]

  In non-intertriginous locations, the vegetating buttons can dry out to change into warty, fissured, painful, seborrheic keratosis-like lesions (dry pemphigus vegetans).[3] We herein report an unusual case of pemphigus vegetans which needs documentation [journals.lww.com]

• 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]

Keywords: Diagnosis, pemphigus vegetans, treatment, unusual presentation How to cite this article: Dhamija A, D'souza P, Meherda A, Kothiwala RK. Pemphigus vegetans: An unusual presentation. [idoj.in]

The goal of the treatment is to induce complete remission while minimizing treatment-related adverse effects. [brieflands.com]

Treatment of severe PV often poses great problems. Systemic corticosteroid therapy is still the mainstay of treatment for pemphigus. However, the treatment is limited by various adverse effects. [jamanetwork.com]

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]

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]

Without treatment, prognosis is poor 75% mortality at 1 year With treatment, prognosis is improved 10% mortality at 1 year [step2.medbullets.com]

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]

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

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]

Disponible en: http://www.elsevier.es/en/revistas/actas-dermo-sifiliograficas-103/epidemiologic-study-of-20-cases-of-pemphigus-13154285-originales-2010 Zaraa I, Sellami A. [revmediciego.sld.cu]

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]

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]

is a potentially fatal autoimmune blistering disease characterized by painful mucocutaneous lesions Associated conditions drug use thiols penicillamine benzylpenicillin captopril cephalosporin thymoma myasthenia gravis systemic lupus erythematosus Epidemiology [step2.medbullets.com]

However, the pathophysiological significance of anti-periplakin reactivity in this pemphigus variant remains to be determined. [ncbi.nlm.nih.gov]

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]

Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology Histopathology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Consultations Deterrence and Patient Education [pubmed.ncbi.nlm.nih.gov]

[…] pemphigus vegetans treated with intralesional and systemic human papillomavirus vaccine. 62 Thomas R...Ho J 35071717 2022 13 Extensive pemphigus vegetans in a Filipino female. 62 Agon MKO...Ramirez-Quizon M 35028399 2021 14 Comprehensive review on the pathophysiology [malacards.org]

Dermatologist-approved lesson plans, activities you can use Free to everyone, these materials teach young people about common skin conditions, which can prevent misunderstanding and bullying. [aad.org]

Tetracycline antibiotics may also be prescribed to prevent secondary infections, while talcum powder can prevent bedsheets and clothing from sticking to the lesions. [verywellhealth.com]

It also aims to prevent complications, especially infections. [medlineplus.gov]

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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.