Pemphigus foliaceus with prominent neutrophilic pustules initially presenting as erythroderma

Miyakura, Takashi; Yamamoto, Toshiyuki; Okubo, Yukari; Ishii, Norito; Oyama, Bungo; Hashimoto, Takashi; Tsuboi, Ryoji

doi:10.1111/j.1365-2230.2008.03190.x

Cited by 10 publications

(13 citation statements)

References 8 publications

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“…In 2004, Gabbi et al reported the case of a woman in her fifth decade of life, similar to our case with recurrent manifestations but less skin involvement. The most recent report was issued by Miyakura et al, presenting a case of a man who complained of erythrodermia, and similar progression of clinical manifestations to our case. All cases (including ours) exhibited a greater percentage of skin involvement when compared with other variants of PF; but a different age of presentation was observed in our case.…”

Section: Discussionsupporting

confidence: 82%

Pemphigus foliaceus with circinated plaques and neutrophil pustules

et al. 2016

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Pemphigus is a group of autoimmune intraepidermal bullous diseases; being pemphigus foliaceus (PF) and pemphigus vulgaris (PV) the most common subtypes. Pustular variants are scarcely reported for both PV and PF. The purpose of this manuscript was to describe the clinical, microscopic and immunologic findings of an atypical case of PF presenting with pustules, including a review of the literature. PF is described as blisters and because this entity is rare, it is not known for the general medical community that they are other clinical features that can be seen as this one we present here with pustules.

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Section: Discussionsupporting

confidence: 82%

Pemphigus foliaceus with circinated plaques and neutrophil pustules

et al. 2016

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“…Unlike patients with pemphigus vulgaris (PV), patients with PF rarely develop mucosal lesions. The erosions may become generalized, and occasionally an exfoliative erythroderma may develop 5–10 . PF presenting as an exfoliative erythroderma has been reported more frequently in the endemic form of PF, fogo selvagem , 1 although a case reported by Nousari et al 6 describes a patient with non‐endemic erythrodermic PF, ultimately proving to be fatal.…”

Section: Discussionmentioning

confidence: 99%

Psoriasiform pemphigus foliaceus: a report of two cases

et al. 2012

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Pemphigus foliaceus (PF) represents an autoimmune blistering disease characterized by the disruption of epidermal intercellular adhesion proteins. Clinical findings include superficial crusted erosions in a seborrheic distribution; however, the disease can rarely present as an exfoliative erythroderma. Histopathologic findings include acantholysis with cleavage within the granular layer. Direct immunofluorescence studies show intercellular IgG and complement deposition. We present two patients, to our dermatology department, with a previous diagnosis of psoriasis, with an exfoliative erythroderma, which ultimately proved to be because of PF based on histopathological features, direct immunofluorescence results and levels of antibodies against desmoglein 1. Both patients responded well to oral prednisone and rituximab. This variant of PF should be entertained in both the clinical differential diagnosis of psoriasiform erythroderma and in the microscopic differential diagnosis of psoriasiform epidermal hyperplasia with focal acantholysis, particularly in patients for whom the clinical history is not classic for psoriasis or for whom conventional psoriasis therapies have not proven beneficial.

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“…Although rarely PF could be associated with GPP; interestingly we found case reports that PF has initially manifested as neutrophilic pustules but in those cases, pathologic changes and DIF studies of the pustules confirmed PF not GPP [1,5,6]. In our patient pustular lesions had no pathologic criteria of PF and were preceded by annular erythema, so she was not a case of pustular PF.…”

Section: Discussionmentioning

confidence: 75%

“…Pemphigus foliaceus (PF) is an autoimmune bullous disease (AIBD) which is presented by superficial flaccid blisters, erosions, scales and crusts on the seborrheic areas of the face and trunk without mucosal involvement [1]. Pathologically, PF is characterized by granular layer acantholysis with acantholytic cells and intraepidermal deposition of Immunoglobulin G (IgG) and complement component 3 (C3) on immunofluorescent studies as a result of antibody production against desmoglein 1, a component of desmosome [2].…”

Section: Introductionmentioning

confidence: 99%