Pemphigus Vegetans Research Articles

Solitary verrucous plaque on a leg.

We present the case of a female in her 70s who presented with a solitary verrucous plaque on her left leg accompanied by painful oral erosions. Various differential diagnoses were considered, like lichen simplex chronicus, hypertrophic lichen planus, and chromoblastomycosis. We diagnosed pemphigus vegetans (PVeg) on a nonintertriginous site through comprehensive clinical examination and histopathological and immunopathological evaluations. This case highlights the importance of considering PVeg in the differential diagnosis of solitary verrucous plaques, even in atypical extra-flexural anatomical locations.

A peculiar post-vaccination eruption.

We report the case of a 47-year-old woman who presented with the appearance of oral mucositis and vegetative lesions localized on the major skin folds that appeared after Comirnaty (BNT162b2) BioNTech anti-SARS-CoV-2 vaccination. Clinical, histological and immunopathological findings led to a diagnosis of pemphigus vegetans (Neumann variant) and the Naranjo score suggested a probable association with the vaccination. To our knowledge, despite its rarity, one other case of pemphigus vegetans after Comirnaty (BNT162b2) BioNTech anti-SARS-CoV-2 vaccination has already been described in literature.

Acantholytic Dyskeratoses of the Vulva: Clinicopathologic Characterization of 16 Cases and Review of the Literature.

The vulva and perineum are rarely involved by acantholytic dyskeratoses, including Hailey-Hailey disease, Darier disease, papular acantholytic dyskeratosis of the genitocrural area, acantholytic dyskeratotic acanthoma, and warty dyskeratoma. These entities show broad histomorphologic overlap, generally requiring clinical correlation for definitive classification. This institutional series aims to better characterize vulvar acantholytic dyskeratoses and provide a practical literature review and diagnostic aid for gynecologic pathologists. Our institutional archives contained 16 vulvar acantholytic dyskeratoses diagnosed between 1990 and 2023. Affected patients were 36 to 79 (mean, 58) years old and presented with one or more asymptomatic (n = 9) or pruritic (n = 6) lesions involving the vulva (predominantly the labia majora), with additional perineal involvement in 2. Four patients have known Hailey-Hailey disease. Eleven cases comprised singular, raised, erythematous, or skin-colored papules, measuring 0.2 to 0.6 (mean, 0.3) cm. Two patients had oligofocal (both with known Hailey-Hailey disease) vulvar lesions, and 2 had multifocal vulvar lesions (one with known Hailey-Hailey disease). Histologically, all showed acantholysis and dyskeratoses (abundant in 8, focal in 8, with corps ronds generally more conspicuous than corps grains). Additional features included suprabasal clefting (n = 14), dermal papillomatosis (n = 12), and acanthosis (n = 8). Adnexal involvement was rare (n = 1). No histologic features reliably distinguished sporadic versus syndromic acantholytic dyskeratoses. Sporadic lesions were cured by local excision. Patients with Hailey-Hailey disease were variably responsive to corticosteroids. Neither our series nor the literature indicate a significant correlation between sporadic or syndromic acantholytic dyskeratosis and squamous cell carcinoma. Important differential diagnoses include pemphigus vulgaris and pemphigus vegetans, for which direct immunofluorescence may be performed, when indicated.

Immunohistopathological analyses of a case of pemphigus vegetans with antibodies against desmoglein 1 and desmocollins 1-3.

Pemphigus vegetans is a rare type of pemphigus characterized by vegetative lesions primarily localized to the intertriginous area. Despite its unique clinical presentation, the underlying pathomechanism remains unclear owing to the rarity of the disease. We report a case of pemphigus vegetans with antibodies against desmoglein 1 and desmocollins 1-3. Furthermore, immunohistochemical analyses were performed to address the pathogenesis of this disease. A 73-year-old man presented with multiple vegetative plaques with erythema on the trunk, groins, and extremities. Mucosal lesions were not observed. Laboratory examinations revealed mild leukocytosis with eosinophilia. A histopathological examination of the skin lesion showed epidermal hyperplasia and intraepidermal abscesses with marked infiltration of neutrophils and eosinophils, and infiltration of lymphocytes and eosinophils into the upper derms. Bacterial culture of the skin tissue was positive for Staphylococcus aureus. Direct immunofluorescence showed deposits of IgG and C3 on keratinocyte surfaces in the epidermis. Autoantibodies against desmoglein 1 and autoantibodies against desmocollin 1, desmocollin 2, and desmocollin 3 were detected by enzyme-linked immunosorbent assays. The diagnosis of pemphigus vegetans was made. Initiation of prednisolone (1.0 mg/kg/day) gradually improved his skin symptoms. We performed immunohistochemical analyses of the lesional skin, which revealed infiltration of CD3-positive, CD4-positive, and CD68-positive cells in the upper dermis, but CD20- or CD56-positive cells were negative. In addition, the present case showed more prominent infiltration of IL-17A- and IL-22-positive cells in the upper dermis than in pemphigus foliaceus, a type of pemphigus with autoantibodies against desmoglein 1. Furthermore, these cells co-expressed CD3 and CD68. We hypothesized that IL-22 and IL-17A produced by T cells and macrophages and their dysregulation might be involved in the pathogenesis of pemphigus vegetans. Additionally, skin colonization and/or infection with Staphylococcus aureus could potentially contribute to the pathogenesis of the disease.

P021 The diagnosis and management of pemphigus vegetans in pregnancy

P021 The diagnosis and management of pemphigus vegetans in pregnancy

Direct immunofluorescence (DIF) versus immunohistochemical (IHC) staining of complements and immunoglobulins (Ig) in pemphigus group.

Pemphigus is a group of bullous disorders of the skin characterized by the formation of autoantibodies present in the intercellular junction of the epidermis. Diagnosis is made by clinical, histopathological examination, and DIF. As DIF needs frozen sections, fluorescent tagged antibodies, UV light microscope for examination, and trained personnel, its non-availability makes a definitive diagnosis challenging. To evaluate the utility of IHC staining of complements and Ig in cases of Pemphigus. Twenty-six diagnosed cases of Pemphigus were stained by Peroxidase immunohistochemical method using monoclonal antibody to IgG, IgA, IgM, IgG4, C3, C4 d with DAB as chromogen. Pemphigus cases include twenty of pemphigus vulgaris (PV), four cases of pemphigus foliaceous (PF), and two of pemphigus vegetans (Pveg). Positivity was defined as the deposition of Ig and complements as distinct, continuous brown staining of keratinocytes at intercellular junctions. On IHC total of 20 PV 17 showed positivity (85%) for IgG, 11 (55%) C4d, 19 (95%) C3d, and 16 (80%) IgG4 deposits at the intercellular junction of the epidermis. All cases of PF showed a deposit of IgG, with three (75%) cases for IgG4, C3d, and C4d. Both cases of Pveg showed positivity for IgG and C4d while one case was negative for IgG4 and C3d. The overall IgG, C3, IgG4, and C4d expression for pemphigus was seen in 88%, 88%, 76.9%, and 61.5% of cases. The relation between these markers, combination of IgG and C3, was best related to each other ( P value = 0.80). The sensitivities for IgG, IgG4, and C3 were 77.8%%, 73%, and 73% resp. We conclude that IHC is a useful tool in the diagnosis of PV with the highest sensitivity of IgG and C3d. The combination of IgG and C3d could replace the DIF in almost all of our cases, so IHC on FFPE sections be used as an alternative method to DIF.

Ayurvedic Management of Pemphigus Vegetans

Pemphigus vegetans is a group of autoimmune disorder characterized by mucocutaneous bullae or pustules that erode to form hypertrophic papillated plaques that predominantly involve the scalp, and the face. It is the rarest variant of pemphigus vulgaris representing about 1-2% of all pemphigus. It may be correlated with Kardama visarpa in Ayurveda. The contemporary management of pemphigus is by systemic steroids. This case report of a 38 year old, male patient diagnosed with Pemphigus vegetans who presented with pustular lesions with foul smell, reddish spots with hypertrophic papillated lesions on scalp, itching and burning sensation over various body parts especially on upper parts since 20 years. He was treated with both Samana as well as Sodhana therapies for about 1 month. This condition was assessed using Pemphigus diseases area index. After treatment there was a significant reduction in the score. Moreover no new lesions were formed suggesting non progression of the diseases.

Pemphigus vulgaris

Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin.

Local neutrophil and eosinophil extracellular traps formation in pyodermatitis pyostomatitis vegetans.

Pyodermatitis pyostomatitis vegetans is a rare inflammatory condition, affecting the skin and/or mucous membrane. Some cases include both skin and mucous involvement, whereas others develop either skin or mucous lesions only. The typically affected areas are the scalp, face, trunk and extremities, including the flexural areas and umbilicus. Clinical features show erosive granulomatous plaques, keratotic plaques with overlying crusts and pustular lesions. Among mucous lesions, oral mucosa is most frequently involved, and gingival erythema, shallow erosions, cobblestone-like papules on the buccal mucosa or upper hard palate of the oral cavity are also observed. Some of the lesions assume a 'snail track' appearance. Although there are several similarities between pyodermatitis pyostomatitis vegetans and other diseases, that is pyoderma gangrenosum, pemphigus vegetans and pemphigoid vegetans, the histopathological features of pyodermatitis pyostomatitis vegetans are unique in that epidermal hyperplasia, focal acantholysis and dense inflammatory infiltrates with intraepidermal and subepidermal eosinophilic microabscesses are observed. Direct immunofluorescence findings are principally negative. Activated neutrophils are supposed to play an important role in the pathogenesis of pyodermatitis pyostomatitis vegetans. The expression of IL-36 and neutrophil extracellular traps (NETs) was observed in the lesional skin, and additionally, eosinophil extracellular traps (EETs) was detected in pyodermatitis pyostomatitis vegetans. A possible pathogenic role of NETs and EETs in the innate immunity and autoinflammatory aspects of pyodermatitis pyostomatitis vegetans was discussed.

A Case of Papillated Bowen's Disease Complicated with Pemphigus Vegetans

A Case of Papillated Bowen's Disease Complicated with Pemphigus Vegetans

PEMPHIGUS VEGETANS OF NEUMANN: A CASE REPORT OF A RARE VARIANT OF PEMPHIGUS VULGARIS

Pemphigus vegetans is a rare variant of pemphigus vulgaris characterized by periorificial papillomatous vegetations. We present the case of a 77-year-old female patient with a history of type 2 diabetes and hypertension who presented with painful verrucous lesions on her tongue. The examination revealed a cerebriform tongue with small, hard, confluent verrucous swellings, which is a well-known sign seen in pemphigus vegetans. A biopsy revealed acantholysis and absence of keratinocyte necrosis, leading to the diagnosis of Neumann-type pemphigus vegetans. The patient was prescribed prednisone and trichloroacetic acid, which resulted in successful treatment. Management of pemphigus vegetans should be individualized based on the patients clinical presentation and response to treatment.

IL-31 and IL-31 receptor alpha in pemphigus: Contributors to more than just itch?

Pemphigus is an autoimmune blistering disorder with four major subtypes: pemphigus vulgaris (PV), pemphigus vegetans (PVe), pemphigus foliaceus (PF), and pemphigus herpetiformis (PH). Among them, PF and PH present itching as a clinical feature; however, the mechanisms behind the pruritus are still unclear. In this report, we sought to investigate the expression of a type 2 inflammation-related pruritogenic cytokine IL-31 and its receptor subunit IL-31RA through immunofluorescence staining analysis. The number of eosinophils, basophils, and mast cells, and the expression levels of thymic stromal lymphopoietin (TSLP) and periostin were also investigated. Evaluation showed an increase in the number of dermal IL-31+ cells and IL-31RA+ cells in PH and PVe. Epidermal expression of IL-31RA increased in PV, PF, and PVe, but not in PH, compared to healthy individuals. The number of dermal eosinophils and basophils was also increased in PVe and PH. The number of dermal mast cells and expression levels of TSLP and periostin did not change among pemphigus subtypes and healthy controls. Collectively, enhanced IL-31/IL-31RA signaling and the increased numbers of dermal eosinophils and basophils may participate in itching in PH. On the other hand, IL-31/IL-31RA signaling seemed unable to provoke itching in PVe, a non-pruritic subtype of pemphigus, although it might contribute to epidermal thickening and dermal fibrosis.

A case of pemphigus vulgaris in a patient with abdominal tumor as collaborative disease

Background: Pemphigus is an autoimmune disease with clinical manifestations of chronic intraepidermal bullae on the skin, mucosa, and loose walls.Pemphigus can be categorized into four types, namely pemphigus vulgaris (PV), pemphigus vegetans, pemphigus foliaceus, and paraneoplastic pemphigus. Pemphigus vulgaris is the most common form of with the highest epidemiology. Pemphigus vulgaris with abdominal tumors cannot be associated where abdominal tumors do not trigger the occurrence of PV. However, abdominal tumors can increase morbidity and provide poor prognosis.
 Case Description: A male, 46 years old, Balinese, Indonesian, came to the Sanglah Hospital Emergency Room with the chief complaint of painful sores around the body, hands, and feet since 20 days ago. Initially the patient said that there were loose, watery blister filled with serous fluid, which after a few days burst and left sores. The blister start on the feet and then spread to the hands and body. The patient was diagnosed with pemphigus vulgaris and abdominal tumors by the digestive surgery doctor. The patient had never had a similar complaint before. History of applying oil, traditional ingredients and topical drugs was denied. The patient was treated mainly with methylprednisolone and showed clinical improvement.
 Conclusion: We report a case of PV in a 46-year-old man with abdominal tumors. This case report is expected to add insight on how to diagnose PV, how to determine therapy, the wound healing process, how to treat wounds, prevent disease expansion, and the relationship between abdominal tumors and PV.

Pemphigus vegentans Hallopeau tip sa promenama na noktima i bez zahvaćenosti oralne mukoze

Introduction: Pemphigus vegetans (PVeg) is the rarest form of autoimmune pemphigus. Lesions are primarily flexural and mucosal, although they may occur at any site. Oral involvement is common. Two subtypes are recognized - the Neumann type and the less common Hallopeau type. Patient review: We present a Hallopeau-type PVeg with no oral lesions and with uncommon nail changes. In the follow-up period of ten years, the patient experienced several flares but no changes in the oral cavity. Conclusion: This case is interesting due to the disease's rarity, atypic clinical presentation, and an association with multiple pregnancies as a possible precipitating factor.

Nail Involvement in Pemphigus: Brief Report

Background: The objective of our study is to describe the different nail involvement at the beginning of the disease according to the type of pemphigus and according to the severity of the pemphigus. Material and methods: We carried out a prospective study over a 3-year period from 2019 to 2022 that included 21 hospitalized patients out of 80 with polydactyly nail involvement 13 pemphigus vulgaris, 4 pemphigus vegetans, and 3 superficial pemphigus in our dermatology department Centre Hospitalier Universitaire (CHU) Hassan II Fez. Results: Paronychia was the most frequent finding overall, observed in 11 (52.38%) cases, followed by onychomadesis and Beau's lines in 8 cases (38%) and subungual hemorrhage in 6 cases (28.57%). Nail involvement was correlated with the severity of the pemphigus, in particular with severe mucosal involvement (17 patients or 80.9%). Conclusion: Nail involvement is a sign of pemphigus severity and may herald an exacerbation of the underlying disease.

Development of pemphigus vegetans and exacerbation of pemphigus foliaceus after secukinumab loading in a patient with complicated generalized pustular psoriasis and pyoderma gangrenosum.

In dermatology, biologics that block signaling pathways of TNF-α, IL-4/IL13, IL-17s, and IL-23 are widely used for the treatment of several inflammatory skin diseases, such as atopic dermatitis and psoriasis. They have shown excellent efficacy with an acceptable safety profile. However, these biologics targeting pathogenic cytokines and their receptors could modulate immunological balance, leading to the development of other inflammatory or autoimmune skin diseases in some cases. In this study, we present a patient who suffered pemphigus vegetans and showed an exacerbation of pemphigus foliaceus after secukinumab loading for the treatment of complicated generalized pustular psoriasis and pyoderma gangrenosum.

Pemphigus vegetans with antibodies against desmoglein 1 and desmocollin 1–3: a case report and literature review

Pemphigus vegetans with antibodies against desmoglein 1 and desmocollin 1–3: a case report and literature review

Immunopathology of acantholytic pemphigus (review)

The review presents problems of immunopathology of acantholytic pemphigus. Simultaneous increase of antibodies to desmoglein-1 and desmoglein-3 is revealed in pemphigus vulgaris and in pemphigus vegetans. In vulgar pemphigus with prevailing mucosal lesions the erosions appeared on the oral mucosa where desmoglein-3 was prevalent. The blister formation does not need complement activation as in other dermatoses, for the presence of framents of monoclonal antibody is sufficient. In pemphigus autoantibodies relate mainly to IgG4 subclass which does not activate complement, poorly activate effector cells through the Fc region and ineffectively crosslinks the antigen, which directly influences the desmosomal functions. In the case of atypical acantholytic pemphigus, non–desmoglein autoantigens may be revealed.

CLINICAL FEATURES OF PEMPHIGUS AT NATIONAL HOSPITAL OF DERMATOLOGY AND VENEREOLOGY FROM 2015 TO 2021.

Pemphigus is a group of autoimmune disease with several types different in clinical features and histopathology. Objectives: To investigate clinical features of pemphigus in out-patients at National Hospital of Dermatology and Venereology (NHDV). Method: We analyzed retrospectively 279 medical records of out-patients with pemphigus at NHDV from 2015 to 2020. Results: Female accounted for 62.7%. The mean age was 48.3 years old, the youngest was 14 years old and the oldest was 88 years old. The mean age of onset was 46.3 years old, the earliest onset was at 10 and the latest onset was at 88. The mean disease duration was 23.9 months, the shortest was one month and the longest was 20 years. The average hospitalization was 1.54, there was a case hospitalized 10 times. Pemphigus vulgaris accounted for the highest rate of 85.3%, the following was pemphigus erythema (10%). Pemphigus vegetans took the lowest rate of 0.4%. 83.4% of cases having lesions on both normal and seborrheic skin. 56.1% of cases were not affected at mucous membranes while only 4.4% of them free from lesions on the skin. Pemphigus vulgaris was highly developed lesions at mucosal areas more than other types. Moderate pemphigus accounted for the major cases of 68.4%. 14.1% was severe pemphigus. Mono-therapy with corticoid was the most common of (65.1%). A combination regime of corticoid and azathioprine took 12.9%. Notably, there was 3.3% having traditional treatments with herbs. Conclusions: Female accounted for 62.7%. The mean age was 48 years old; the mean age of onset was 46 years olds. Pemphigus vulgaris was the most common type and highly developed lesions at mucous membranes more than other types. Two-third suffered from moderate pemphigus. Mono-therapy with corticoid was seen the most.

De novo Pemphigus Vegetans

Pemphigus vegetans is an autoimmune disease characterized by flaccid bullae or pustules that erode to form hypertrophic plaques involving predominantly skin flexures and mucous membranes [1]. We are presenting an unusual case of de novo pemphigus vegetans with extensive involvement of genitals, perianal area, buttocks, nails, oral mucosa, and cerebriform tongue which was initially mistaken for malignancy due to its unusual presentation.