Cervicovaginal involvement in pemphigus vulgaris: a clinical study of 77 cases

Akhyani, Maryam; Chams‐Davatchi, Cheyda; Naraghi, Z Safaei; Daneshpazhooh, Maryam; Toosi, Siavash; Asgari, Masoud; Malekhami, F.

doi:10.1111/j.1365-2133.2007.08356.x

Cited by 38 publications

(42 citation statements)

References 24 publications

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“…7,9,10 Yapı-lan az sayıdaki çalışmada farklı tutulum oranları bildirilmiş-tir. 3,11,12 Marren ve arkadaşları dokuz pemfigus vulgarisli hastanın dördünde vulva tutulumu olduğunu belirtirken 11 Malik ve Ahmed sadece genital bölge tutulumlu pemfiguslu hastalarla yaptıkları çalışmada pemfigusta genital tutulumun nadir olduğunu ve vajinal lezyonlardan ziyade vulva tutulumunun daha fazla olduğunu bildirmişlerdir. Hastaların %61'inde labial tutulum, %30'unda vajinal tutulum, %3'ünde ise sadece vajinal tutulum saptanmış-tır.3 Genital bölge tutulumu nadir olmakla birlikte yapı-lan bir diğer çalışmada 77 pemfigus vulgarisli hastanın 39'unda (%51) genital bölge tutulumu tespit edilerek oral mukozadan sonra ikinci en sık tutulan mukozal bölge olduğu öne sürülmüştür.…”

Section: Discussionunclassified

A Delayed Diagnosis: Pemphigus Vulgaris With Mucosal Involvement

Bilgili¹,

Kurdoğlu²,

Karadağ³

et al. 2012

Sakarya Med J

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Pemfigus vulgaris deri ve mukozaları tutan, vezikül ve büllerle karakterize, kronik, otoimmün bir hastalıktır. Sıklıkla oral mukozada başlayıp deriye ve diğer mukozal bölgelere yayılım gösterir. Pemfigus vulgariste genital mukoza tutulumu daha nadir görülmektedir. Deri tutulumunun olmadığı durumlarda tanı koymak güç olabilmektedir. Yirmi yıldır oral mukozada, 10 yıldır genital bölgede erozyon ve ülserasyonları olan 46 yaşındaki bayan hastaya, klinik ve histopatolojik değerlendirme sonucu pemfigus vulgaris tanısı konuldu. Bu olgu, histopatolojik inceleme ile tanısı kolayca doğrulanabilen pemfigus vulgarisin, tedaviye yanıt vermeyen oral ve genital ülserlerin ayırıcı tanısında akılda tutulması gerektiğini vurgulamak amacı ile sunulmaktadır.

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Section: Discussionunclassified

A Delayed Diagnosis: Pemphigus Vulgaris With Mucosal Involvement

Bilgili¹,

Kurdoğlu²,

Karadağ³

et al. 2012

Sakarya Med J

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“…Genital tract lesions have also been described in PV, but almost invariably in women. Akhyani et al (12) observed genital involvement in 51%, almost invariably showing extensive mucocutaneous involvement. After the oral mucosa, genital mucosa might thus represent the second most commonly affected mucosal site in PV, and its involvement is surely under-diagnosed (12).…”

Section: Included In the Theme Issue: Venereology -Not To Be Forgottenmentioning

confidence: 99%

“…Akhyani et al (12) observed genital involvement in 51%, almost invariably showing extensive mucocutaneous involvement. After the oral mucosa, genital mucosa might thus represent the second most commonly affected mucosal site in PV, and its involvement is surely under-diagnosed (12). Finally, the presence of genital lesions was associated with treatment-resistance and regarded as a poor prognostic factor and marker of disease severity (9).…”

Section: Included In the Theme Issue: Venereology -Not To Be Forgottenmentioning

confidence: 99%

Penile Erosions as First Manifestation of Pemphigus Vulgaris: A Misleading Presentation

Stieger¹,

Pelivani²,

Ramelet³

et al. 2013

Acta Derm Venerol

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Pemphigus vulgaris (PV) is a potentially devastating autoimmune bullous disease that affects the mucosa and skin. It is characteristically associated with intraepidermal blistering and cell-cell dissociation due to the presence of autoantibodies targeting desmoglein (dsg) 3 and dsg 1, i.e. transmembrane adhesion molecules of the desmosomal cadherin protein family (1-3). In most patients, PV characteristically starts with and affects the oral mucosa, although involvement of other mucosae may also occur (4-10). We have recently observed 2 patients, in whom the first clinical manifestation of PV consisted of chronic painful penile erosions, which represented a diagnostic challenge. CASE REPORTSCase 1. A 59-year-old man presented with painful penile erosions of 3-months duration. Microbiological cultures and serology for Treponema pallidum were negative. Under treatment with local antibiotics and disinfectants the erosive lesions further progressed and affected the entire glans penis and part of the shaft. Only after the patient developed painful aphthoid lesions in the oral mucosa, was a biopsy specimen from the oral mucosa obtained, which showed suprabasal blistering with acantholysis. Direct immunofluorescence studies of perilesional oral mucosa demonstrated intercellular epithelial deposits of immunoglobulin G (IgG) and complement (C3). Enzyme-linked immunoassay-desmoglein 3 (ELISA-dsg 3) (136 U/ml, normal < 7) and ELISA-dsg 1 (62 U/ ml, normal < 14) were both positive. The patient was first given prednisone 0.5 mg/kg body weight. However, under this regimen he further developed multiple vesicles and bullae on the neck, trunk and extremities. Prednisone was hence increased to 1 mg/ kg body weight/day and associated with azathioprine 2.5 mg/ kg/day. Genital lesions were concomitantly treated with topical betamethasone associated with topical antibiotics and disinfectants. Thereafter, both mucous and cutaneous lesions started healing. Four weeks later, at the end of the consolidation phase, prednisone was tapered gradually. After 12 months, the patient remained in complete remission under therapy (Fig. 1). Case 2. A 35-year-old man was referred for evaluation of a 3-month history of painful penile erosions. Topical treatments with disinfectants and topical antibiotics had no effect. Two months after appearance of the genital lesions the patient also developed painful oral lesions. On examination, the patient had multiple erythematous erosions on the glans penis and sulcus coronarius (Fig. 2). In the oral cavity, the patient exhibited asymptomatic erosions in the region of the palatal arch and the lower lip. Histology from the glans penis showed an intraepidermal bulla with acantholytic cells. Direct immunofluorescence microscopy studies demonstrated deposits of IgG and C3 on the cytoplasmic membrane of the keratinocytes. ELISA-dsg 3 was strongly positive (146 U/ml, normal <7), whereas ELISA-dsg 1 was negative. The patient was treated with prednisone 1 mg/ kg body weight and azathioprine 150 mg/day. Furthermore, he...

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“…The other mucosal areas, nasal cavity, larynx (epiglottis, vocal cords), oropharynx, esophagus, vagina, vulva, penis, and anus may also be affected [1,13]. Epistaxis and hoarseness are present owing to the involvement of the nose, pharynx, and larynx [1,13,[35][36][37]. Genital mucosa is one of the frequent sites involved in PV after the oral mucosa [37].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Epistaxis and hoarseness are present owing to the involvement of the nose, pharynx, and larynx [1,13,[35][36][37]. Genital mucosa is one of the frequent sites involved in PV after the oral mucosa [37].…”

Section: Clinical Featuresmentioning

confidence: 99%

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Kılıç¹

2018

Autoimmune Bullous Diseases

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Pemphigus is a group of autoimmune blistering disorders associated with autoantibodies against the keratinocyte cell surface. Pemphigus has three major variants: pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) which all have further subtypes. The variants of pemphigus are classified depending upon the clinical and histological features, immunofluorescence staining pattern, and autoantibody profile of the disease. The onset and course of pemphigus appear on the basis of interaction between genetic predisposition and various triggering factors. Pemphigus vulgaris is the most commonly seen and representative clinical form of pemphigus. Together with clinical manifestations, the histopathological and immunopathological data support the diagnosis. As though some pemphigus variants, particularly pemphigus vulgaris and paraneoplastic pemphigus, have a mortality risk, early diagnosis is necessary and onset of treatment should be promptly initiated. In this chapter, firstly, classification of pemphigus is described. After then, clinical features, histopathological and immunopathological findings, target antigens, etiopathogenesis and comorbidities of each pemphigus variant are discussed briefly.Keywords: pemphigus, pemphigus variants, clinical manifestations, pathogenesis, histopathology, immunopathology IntroductionPemphigus is a distinct organ-specific autoimmune blistering disorder involving skin and mucous membranes associated with autoantibodies directed against desmosomes-intercellular adhesive molecule complex localized on the keratinocyte cell surface [1][2][3][4].© 2018 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Pemphigus has three major variants, which are classified depending on the basis of the clinical, histological features, immunofluorescence staining pattern and autoantibody profile of the disease including pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP), which all have further subtypes. Less frequently seen and newer variants of pemphigus include IgA pemphigus (IGAP) and pemphigus herpetiformis (PH) [1-6].The term "pemphigus" origins from the Greek word "pemphix", which has a meaning of "blister" [1]. It is a chronic potentially life-threating bullous disorder if not treated on time [4,7,8]. The phenotypes of pemphigus represent a complex spectrum with multiple genetic and environmental factors playing a role in disease pathogenesis [9,10].Together with clinical manifestations, the histopathological and immunopathological data support the diagnosis of the disease. The best site for the cutaneous biopsy for the appropriate histopathological examination is a fresh (< 24 h) small vesicle or 1/3 of the peripheral portion of the blister including the perilesional normal appearing s...

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Cervicovaginal involvement in pemphigus vulgaris: a clinical study of 77 cases

Abstract: Involvement of the female genital tract with PV might not be as infrequent as was previously thought; it is probably the second most common mucosal site of PV after the oral mucosa. Genital lesions may be missed and the need for thorough pelvic examination should not be overlooked.

Cited by 38 publications

References 24 publications

A Delayed Diagnosis: Pemphigus Vulgaris With Mucosal Involvement

A Delayed Diagnosis: Pemphigus Vulgaris With Mucosal Involvement

Penile Erosions as First Manifestation of Pemphigus Vulgaris: A Misleading Presentation

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

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