Diagnosis and Clinical Features of Pemphigus Foliaceus

James, Kirk A.; Culton, Donna A.; Díaz, Luis A.

doi:10.1016/j.det.2011.03.012

Cited by 111 publications

(158 citation statements)

References 93 publications

(76 reference statements)

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“…In FS, the disease usually begins on the head, neck, and seborrheic regions of the skin. The oral mucosa, palms of the hands, and plant of the feet are usually spared [59,66]. In both PF and FS, lesions may become confluent and can transform to exfoliative erythroderma [67].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…Sporadic form of PF is most common in Europe and USA [16].The average age of PF ranges between 40 and 60 years, while FS is very often in children, adolescents and young adults. It is usually seen equally in both females and males with a female preponderancy [59,60]. FS occurs in genetically related family members.…”

Section: Pemphigus Foliaceusmentioning

confidence: 99%

“…PF usually begins on the trunk, but may also be localized on the face and scalp. Sometimes yellowish crusted and scaly erythematous plaques on face and trunk predominate the clinical findings resembling the clinical picture of seborrheic dermatitis [2,5,16,59,66]. In FS, the disease usually begins on the head, neck, and seborrheic regions of the skin.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…PF is considered as a more benign form of the disease generally presenting with only cutaneous involvement [2,5,16,59]. However, transition from PV to PF or vice versa may be observed [63][64][65].…”

Section: Clinical Featuresmentioning

confidence: 99%

“…PF has a universal occurrence and occurs sporadically, while the endemic form of PF, called as fogo selvagem (FS) or wild fire (WF), is predominantly seen in the rural and tropical regions of Brazil [5,7,16,58,59]. Another variant of PF, a localized form, is called as pemphigus erythematosus (PE) [16,59]. Sporadic form of PF is most common in Europe and USA [16].The average age of PF ranges between 40 and 60 years, while FS is very often in children, adolescents and young adults.…”

Section: Pemphigus Foliaceusmentioning

confidence: 99%

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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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Section: Clinical Featuresmentioning

confidence: 99%

Section: Pemphigus Foliaceusmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

Section: Pemphigus Foliaceusmentioning

confidence: 99%

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Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Kılıç¹

2018

Autoimmune Bullous Diseases

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New‐onset pemphigus foliaceus following SARS‐CoV‐2 infection and unmasking multiple sclerosis: A case report

Mohaghegh

Hatami

Refaghat

et al. 2022

Clinical Case Reports

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Mendelian Disorders of Cornification Caused by Defects in Intracellular Calcium Pumps: Mutation Update and Database for Variants in ATP2A2 and ATP2C1 Associated with Darier Disease and Hailey-Hailey Disease

et al. 2017

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The two disorders of cornification associated with mutations in genes coding for intracellular calcium pumps are Darier disease (DD) and Hailey-Hailey disease (HHD). DD is caused by mutations in the ATP2A2 gene, whereas the ATP2C1 gene is associated with HHD. Both are inherited as autosomal-dominant traits. DD is mainly defined by warty papules in seborrheic and flexural areas, whereas the major symptoms of HHD are vesicles and erosions in flexural skin. Both phenotypes are highly variable. In 12%-40% of DD patients and 12%-55% of HHD patients, no mutations in ATP2A2 or ATP2C1 are found. We provide a comprehensive review of clinical variability in DD and HHD and a review of all reported mutations in ATP2A2 and ATP2C1. Having the entire spectrum of ATP2A2 and ATP2C1 variants allows us to address the question of a genotype-phenotype correlation, which has not been settled unequivocally in DD and HHD. We created a database for all mutations in ATP2A2 and ATP2C1 using the Leiden Open Variation Database (LOVD v3.0), for variants reported in the literature and future inclusions. This data may be of use as a reference tool in further research on treatment of DD and HHD.

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Diagnosis and Clinical Features of Pemphigus Foliaceus

Cited by 111 publications

References 93 publications

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

Pemphigus: Subtypes, Clinical Features, Diagnosis, and Treatment

New‐onset pemphigus foliaceus following SARS‐CoV‐2 infection and unmasking multiple sclerosis: A case report

Mendelian Disorders of Cornification Caused by Defects in Intracellular Calcium Pumps: Mutation Update and Database for Variants in ATP2A2 and ATP2C1 Associated with Darier Disease and Hailey-Hailey Disease

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