Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Abstract

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...

Clinico-Epidemiologic Profile And Treatment Outcomes of Pemphigus Vulgaris: A Study in a Tertiary Care Center

“…He also added epistaxis and hoarseness of voice while mucosal erosion present. 15 Trunk was the commonest site of cutaneous involvement and oral mucosa was the next common site. In PV patients 71% were mucocutaneous type and 29% was cutaneous type but no one found pure mucous type.…”

Efek Samping Steroid Sistemik pada Terapi Pemgus Vulgaris

“…4 Pencetus PV berasal dari berbagai faktor seperti obat-obatan (kaptopril, penisilamin), infeksi (virus herpes simpleks, virus Epstein-Barr, dan lain-lain), pestisida, radiasi ultraviolet, luka bakar, stres, dan makanan yang mengandung allium, fenol, tiol, atau urushiol. 10 Secara klinis, lesi kulit pada PV jarang dirasa gatal, lebih sering terasa nyeri. Lesi awal PV adalah bula kendur di permukaan kulit mana saja.…”

Concordance of clinical, histopathologic and direct Immunofluorescence findings in patients with intraepidermal immunobullous disorders

“…The other intraepidermal immunobllous disorders are pemphigus foliaceus (PF), paraneoplastic pemphigus, intercellular immunoglobulin A (IgA) pemphigus, and pemphigus erythematous (PE). [5][6][7][8] The disease can be life-threatening in 5-10% of cases. An accurate diagnosis of disease requires a correlation of clinical findings and microscopy with findings on direct immunofluorescence (DIF).…”